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“An 18-year-old male presented with a short history of 8 days of weakness of both lower limbs. There was no history of trauma or tuberculosis or skin lesions in form of papules or macules. No other complaint was associated and the past history was of no significance. The cranial neurological examination was normal. His Bulk, tone, and power in both upper limbs were normal. On examination of both lower limb, his bulk was normal, had bilateral spasticity with muscle power of grade 4 in all groups of muscles. His deep tendon reflexes were exaggerated with bilateral extensor plantar. No sphincter involvement and all routine investigations including lipid profile were normal. Magnetic Resonance Imaging of Spine showed mass lesion in epidural space compressing cord from behind without any bony involvement atD7 to D10 vertebral segment. Mass lesion was isointense on T1 and hyper-intense on T2 with no contrast enhancement [Figure , ].\nD7 to D10 Laminectomy with complete excision of firm epidural mass, which could be separated from dura with difficulty, was carried out. Histopathology revealed fibro-collagenous tissue with dense infiltration of lymphocytes, histiocytes, plasma cells, eosinophils, and multinucleated Touton giant cells. Histiocytic granulomas were seen. Presence of arteritis and panniculitis noted. Overall morphology was suggestive of JXG. Immune marker study was proposed and revealed Viamentin positive, Cluster Differentiation-68 positive in Touton giant cell and negative for S-100, CD1a, and Actin smooth muscle [Figure –].\nPost-operative, patient improved neurologically. His power improved to grade 5/5 with decreased spasticity. At 9 month follow-up patient had mild spasticity with extensor plantar. His MRI carried out at 3 months shows post-Laminectomy status with no residual tumor [].” | The second child (Fig. ) of a 29-year-old woman was spontaneously born at 39 + 4 weeks of gestation after an unremarkable pregnancy. Birth weight was 3510 g (50th percentile), length 55 cm (75th percentile) and head circumference 33.5 cm (20th percentile). Apgar scores were 9/10/10. At the age of 3 months the girl was seen by a general pediatrician and consecutively referred to our hospital because of a recently developed mass on the left temple. The subcutaneous swelling was about 2 cm in diameter, non-moveable, not reddish or overheated and not painful. Furthermore, the mother reported recurrent fever spikes up to 38.5 °C without signs of inflammation for about 4 weeks. Defecation and drinking habits were adequate, vomiting was denied. However, a weight loss of 200 g within 3 weeks was obvious. In addition to a pale skin color and three pinhead-large livid subcutaneous lesions located on the trunk and the lower extremities, there was a left-sided rib hump situated at the level of Th6 to Th10; a secondary finding was oral candidiasis. Laboratory values on admission showed: hemoglobin 85 g/l, hematocrit 0.24 L/l, thrombocytes 380 G/l, lactate dehydrogenase 308 U/l, alpha-1-fetoprotein 225.6 ng/ml, beta-human chorionic gonadotropin < 1 mU/ml, c-reactive protein 10.13 mg/dl, interleukin-6 45.8 pg/ml and procalcitonin 0.31 ng/ml. To define the extent of disease, whole-body magnetic resonance imaging (MRI) (Fig. ) was performed. An intraosseous soft tissue lesion in the left sphenoid bone (diameter 18 × 20 mm), a big paravertebral thoracic tumor conglomerate (diameter 85 × 59 mm), multiple papules to nodules in the liver (7 mm), in both kidneys (6 mm) and lungs (3 × 4.3 mm) and in the pancreatic head (3.5 mm), as well as cutaneous (5 mm) and intraosseous lesions were found. A vertebra plana of Th9, together with infiltration of the adjacent Th8 and Th10, resulting in a kinking of the spinal column compromising the spinal canal and obliteration of nerve roots by soft tissue tumor mass was seen. Due to the lesion in the skull and the vertebra plana, Langerhans cell histiocytosis was one of the primary differential diagnoses. But the histology of one cutaneous lesion of the trunk did not confirm this diagnosis. Rapid deterioration with paraplegia prompted us to administer immunosuppressive treatment immediately. Based on the presumed diagnosis of a neoplasia of the Ewing / PNET group the patient was initially treated according to the Euro-Ewing protocol. After the third biopsy and histological examination two independent pathology centers confirmed the diagnosis of xanthosiderohistiocytosis, which is not well-defined and is regarded as a morphologic variant of xanthoma disseminatum – a type that most often occurs in adult patients with monoclonal gammopathy (Fig. ). In keeping with the established diagnosis, Langerhans cell histiocytosis-based chemotherapy treatment was administered. Following the arm for the high-risk group, the chemotherapy agents included prednisone, vinblastine, 6-mercaptopurine and methotrexate. With this therapy the primary tumor mass decreased. Clinical and radiologic examinations at the age of 3 years show partial remission after 1 year maintenance chemotherapy with puri-nethol and methotrexate. | 0 | Write a detailed clinical case vignette based on the following key phrases: Histiocytic lesions, Spinal cord compression, Xanthoma/xanthogranuloma |
A 50-year-old male patient was referred to our hospital, with a 4-month history of neck pain and gradually progressive weakness of all four limbs. Over the past 2 weeks, the patient noticed significant deterioration and became bed bound. The patient denies any previous history of trauma, malignancy, or medical history of note. Clinical examination revealed spastic quadriparesis of 3/5 with 80% decreased sensation of all modalities below C5. Sphincters were found to be intact.\nPlain X-ray cervical spine on anterior-posterior (AP) view showed a large lucent lesion replacing the C5 vertebra with loss of the left pedicle []. The magnetic resonance imaging (MRI) of the cervical spine demonstrated a T1 and T2 isointense, expansile mass lesion involving the C5 vertebral body extending into the left pedicle but sparing neural foramina. On T2, small hyperintense intralesional cavity was noted in the anterior part. The lesion extended across the C5/6 intervertebral disc and into the anteroinferior part of the C4 vertebra. Significant cord compression with absent thecal sac against C5 was noted. The lesion enhanced homogeneously with gadolinium and appeared completely extradural [Figure -]. Based on imaging, metastasis, myeloma, and tuberculous granuloma were suspected.\nThe patient underwent standard anterior cervical decompression and fixation. Peroperatively, tumor appeared as a well-encapsulated, firm, multinodular mass, yellow tan in color, 3 cm × 3 cm × 2 cm in size. There was a small necrotic part corresponding to the T2 hyperintensity. The lesion was excised in piecemeal fashion [] to completely decompress the cord. No clue of bone was found within the mass. At this point, differential was further narrowed to granulomatous lesion like tuberculosis. Histology revealed sheets of histiocytes with foamy cytoplasm and bland nuclei. Focal cholesterol clefts and multinucleated giant cells were also noticed. There was no cellular atypia. CD68 was positive and S100 was found to be negative [Figure and ].\nThe serum calcium was normal, and lipid profile showed normolipemia. Clinical examination did not reveal any soft-tissue xanthomas. The patient was able to return to his normal activities 4 weeks after the operation. Postoperative imaging as per routine following anterior decompression and fixation consisted of plain X-ray cervical spine AP and lateral views [Figure and ]. Postoperative computed tomography (CT) of the same area was planned at the 6-month follow-up both to see the integrity of fixation as well as to rule out the tumor recurrence, but the patient was not able to reach out for imaging because of pandemic restrictions. Currently, he is 1 year out of his surgery, with no reported complaint as per telephonic follow-up. | “An 18-year-old male presented with a short history of 8 days of weakness of both lower limbs. There was no history of trauma or tuberculosis or skin lesions in form of papules or macules. No other complaint was associated and the past history was of no significance. The cranial neurological examination was normal. His Bulk, tone, and power in both upper limbs were normal. On examination of both lower limb, his bulk was normal, had bilateral spasticity with muscle power of grade 4 in all groups of muscles. His deep tendon reflexes were exaggerated with bilateral extensor plantar. No sphincter involvement and all routine investigations including lipid profile were normal. Magnetic Resonance Imaging of Spine showed mass lesion in epidural space compressing cord from behind without any bony involvement atD7 to D10 vertebral segment. Mass lesion was isointense on T1 and hyper-intense on T2 with no contrast enhancement [Figure , ].\nD7 to D10 Laminectomy with complete excision of firm epidural mass, which could be separated from dura with difficulty, was carried out. Histopathology revealed fibro-collagenous tissue with dense infiltration of lymphocytes, histiocytes, plasma cells, eosinophils, and multinucleated Touton giant cells. Histiocytic granulomas were seen. Presence of arteritis and panniculitis noted. Overall morphology was suggestive of JXG. Immune marker study was proposed and revealed Viamentin positive, Cluster Differentiation-68 positive in Touton giant cell and negative for S-100, CD1a, and Actin smooth muscle [Figure –].\nPost-operative, patient improved neurologically. His power improved to grade 5/5 with decreased spasticity. At 9 month follow-up patient had mild spasticity with extensor plantar. His MRI carried out at 3 months shows post-Laminectomy status with no residual tumor [].” | 0 | Write a detailed clinical case vignette based on the following key phrases: Histiocytic lesions, Spinal cord compression, Xanthoma/xanthogranuloma |
The mother of a 1-year-old girl initially noticed reduced activity of the lower limbs which worsened over 8 days. On examination paraplegia, exaggerated deep tendon reflexes and positive Babinski sign were noted. No other neurological deficit found. Cutaneous lesions were absent. Magnetic resonance imaging (MRI) of the spine showed intradural extramedullary (IDEM) lesion at D6–D8 level suggestive of a benign nerve sheath tumor [].\nTotal excision of the tumor was done. Gross specimen included multiple bits yellowish-white, soft to firm. Histopathology showed tumor composed of sheets of spherical, ovoid, and spindle cells with vesicular bland nuclei. Many Touton giant cells were seen [].\nOn immunohistochemistry, the tumor cells were strongly positive for CD68 (histiocytic marker) and weakly for CD163 []. Cells were negative for s100 protein, CD1a, glial fibrillary acidic protein, epithelial membrane antigen. Special stain such as Zeil Neelson, Gomori methenamine silver, and periodic acid-Schiff stain were negative, thus ruling out mycobacteria and fungi as etiologic agents. Thus, a diagnosis of JXG was made. The infant showed full clinical recovery. The patient was managed with regular follow-up only and is doing well until date. | “An 18-year-old male presented with a short history of 8 days of weakness of both lower limbs. There was no history of trauma or tuberculosis or skin lesions in form of papules or macules. No other complaint was associated and the past history was of no significance. The cranial neurological examination was normal. His Bulk, tone, and power in both upper limbs were normal. On examination of both lower limb, his bulk was normal, had bilateral spasticity with muscle power of grade 4 in all groups of muscles. His deep tendon reflexes were exaggerated with bilateral extensor plantar. No sphincter involvement and all routine investigations including lipid profile were normal. Magnetic Resonance Imaging of Spine showed mass lesion in epidural space compressing cord from behind without any bony involvement atD7 to D10 vertebral segment. Mass lesion was isointense on T1 and hyper-intense on T2 with no contrast enhancement [Figure , ].\nD7 to D10 Laminectomy with complete excision of firm epidural mass, which could be separated from dura with difficulty, was carried out. Histopathology revealed fibro-collagenous tissue with dense infiltration of lymphocytes, histiocytes, plasma cells, eosinophils, and multinucleated Touton giant cells. Histiocytic granulomas were seen. Presence of arteritis and panniculitis noted. Overall morphology was suggestive of JXG. Immune marker study was proposed and revealed Viamentin positive, Cluster Differentiation-68 positive in Touton giant cell and negative for S-100, CD1a, and Actin smooth muscle [Figure –].\nPost-operative, patient improved neurologically. His power improved to grade 5/5 with decreased spasticity. At 9 month follow-up patient had mild spasticity with extensor plantar. His MRI carried out at 3 months shows post-Laminectomy status with no residual tumor [].” | 0 | Write a detailed clinical case vignette based on the following key phrases: Histiocytic lesions, Spinal cord compression, Xanthoma/xanthogranuloma |
“An 18-year-old male presented with a short history of 8 days of weakness of both lower limbs. There was no history of trauma or tuberculosis or skin lesions in form of papules or macules. No other complaint was associated and the past history was of no significance. The cranial neurological examination was normal. His Bulk, tone, and power in both upper limbs were normal. On examination of both lower limb, his bulk was normal, had bilateral spasticity with muscle power of grade 4 in all groups of muscles. His deep tendon reflexes were exaggerated with bilateral extensor plantar. No sphincter involvement and all routine investigations including lipid profile were normal. Magnetic Resonance Imaging of Spine showed mass lesion in epidural space compressing cord from behind without any bony involvement atD7 to D10 vertebral segment. Mass lesion was isointense on T1 and hyper-intense on T2 with no contrast enhancement [Figure , ].\nD7 to D10 Laminectomy with complete excision of firm epidural mass, which could be separated from dura with difficulty, was carried out. Histopathology revealed fibro-collagenous tissue with dense infiltration of lymphocytes, histiocytes, plasma cells, eosinophils, and multinucleated Touton giant cells. Histiocytic granulomas were seen. Presence of arteritis and panniculitis noted. Overall morphology was suggestive of JXG. Immune marker study was proposed and revealed Viamentin positive, Cluster Differentiation-68 positive in Touton giant cell and negative for S-100, CD1a, and Actin smooth muscle [Figure –].\nPost-operative, patient improved neurologically. His power improved to grade 5/5 with decreased spasticity. At 9 month follow-up patient had mild spasticity with extensor plantar. His MRI carried out at 3 months shows post-Laminectomy status with no residual tumor [].” | A 15-year-old male presented with pain in the nape of the neck and gradually progressive ascending weakness of all four limbs along with bladder and bowel involvement for 3 years. On examination, tone was increased in all four limbs with 4/5 power in bilateral upper limbs and 2/5 power in both lower limbs, along with exaggerated deep tendon reflexes in all four limbs. Superficial abdominal reflexes were absent and, bilateral planters were extensor. He had 70–80% sensory loss below T4 level. His routine laboratory studies including hematological and biochemical parameters were within normal limits.\nComputerised tomography scan of the cervical spine revealed a large osseo-destructive lesion involving body of C5and C6 vertebra which was causing spinal canal compromise [Figure and ]. The lesion was involving surrounding structures with extension into the submuscular plane anteriorly. On magnetic resonance imaging (MRI) evaluation, the mass was isointense on T1 and T2 weighted sequences with minimal contrast enhancement. It was causing distrucation of C5 and C6 vertebra body with compression of thecal sac [Figure –].\nUsing anterior cervical approach, the tumor resection was performed. Intra-operatively, the tumor involved whole of the C5 and C6 vertebral body. There was a soft tissue component of the tumor, which was extending into the submuscular plane. Inside the capsule, the tumor consisted of cheesy material that was minimally vascular. Total tumor excision was done with tricorticate iliac crest bone grafting and plate and screw fixation between C4 and C7 vertebral bodies.\nMicroscopic examination showed spindle cells disposed of in intersecting fascicles intermingled with mononuclear xanthomatous cells displaying round nuclei with dense chromatin inconspicuous nucleoli and pale eosinophilic finely vacuolated chromatin. Spindle cells display bland appearing nuclear chromatin and eosinophilic cytoplasm. Occasional cells displayed nuclear atypia and enlargement. Scattered neutrophils were seen admixed with these cells. Focal areas of calcification and occasional osteoclasts like giant cells were also seen. Interspersed areas showed degenerated bony trabeculae and cartilage [Figure and ].\nPostoperatively, patient had significant improvement in neck pain and spasticity in all four limbs. At follow-up of 6 months, he was doing well. | 0 | Write a detailed clinical case vignette based on the following key phrases: Histiocytic lesions, Spinal cord compression, Xanthoma/xanthogranuloma |
A 29-year-old male patient presented with right side weakness and left side numbness that occurred just after a car accident. Upon physical examination, there was no abnormal finding, except for tenderness in the upper cervical area. Routine laboratory tests demonstrated normal results. Motor weakness of right side extremity was disclosed as 1/5 for arm and 2/5 for leg with slightly hyperactivity of deep tendon reflex. Sensory examination showed significantly decreased pain, temperature, vibration, and discriminatory touch sensations below the left C2 dermatome. Babinski's reflex and Hoffman's sign were present on the left side. Based on the symptoms and neurological findings, the assumptive diagnosis of Brown-Sequard syndrome was made. The patient had no symptoms or past medical history related to a cervical spine lesion before the trauma. Cervical plain films revealed straightening of the cervical spine with loss of normal lordosis and widening between the posterior arch of the atlas and spinous process of the axis. A computed tomography (CT) scan of the upper cervical spine revealed a large round mass which measured 25×18×24 mm with relatively homogeneous strong enhancement and was located between the C1 and C2 interspinous space. In addition, the CT scan showed cortical osseous erosions at the lower C1 posterior arc and upper C2 spinous process. Cervical magnetic resonance images (MRI) revealed an epidural dumbbell-shaped mass traversing the C1 to C2 interspinous space, compressing the surrounding structures (). The spinal cord was compressed by the lesion which was isointense on T1-weighted image (WI) and mixed hypointense on T2WI with homogenous strong enhanced after gadolinium administration. Radiologic analysis indicated possible lymphoma, meningioma, schwannoma or other neuronal tumors.\nThe patient underwent a surgery immediately for decompression of the spinal cord. A total tumor resection was performed with only a partial laminectomy from the lower half of the C1 to upper half of C2 without any instrumentation and access to the tumor. After partial lamicectomy, an extradural and yellowish mass was exposed with well-demarcated from the surrounding structures but adhesive at the epidural area. Under microscopy, the tumor was delicately dissected, and en bloc removal was achieved (). The mass was ovoid, soft like gelatin, avascular, and well-encapsulated, measuring 25 mm at the largest diameter, and was adherented to the outer layer of the dura mater. And, there was no root invasion. On the basis of the clinical and operative features, we thought that the lesion originated from the outer aspect of the dura mater. Upon sectioning, the mass was particularly yellow and white with cystic components ().\nAfter surgery, the patient's right hemiparesis fully recovered in 2 weeks. However, a slightly tingling numbness on his left arm remained and was controlled through medication in the outpatient clinic for 4 months. Currently, the patient has no symptoms without instability of cervical spine. Cervical MRI with gadolinium enhancement at 2 years after surgery demonstrated no recurrent lesion ().\nHistological examination of the tumor uncovered focal aggregation cells with ample, clear, and foamy cytoplasm in a background of loose, fibrous stroma of xanthic appearance. Large, round cells had irregular vesicular nuclei which were regular and small with condensed chromatin (). The specimen was underwent to immunohistochemical testing, yielding strongly positive for CD 68 and lysozyme stain, which confirmed that the large foam cells were histiocytes. The test with CD-1a was negative, thus ruling out Langerhans' cell histiocytosis. GFAP stain and S-100 protein were negative. Thus, a diagnosis of xanthogranuloma was established. | “An 18-year-old male presented with a short history of 8 days of weakness of both lower limbs. There was no history of trauma or tuberculosis or skin lesions in form of papules or macules. No other complaint was associated and the past history was of no significance. The cranial neurological examination was normal. His Bulk, tone, and power in both upper limbs were normal. On examination of both lower limb, his bulk was normal, had bilateral spasticity with muscle power of grade 4 in all groups of muscles. His deep tendon reflexes were exaggerated with bilateral extensor plantar. No sphincter involvement and all routine investigations including lipid profile were normal. Magnetic Resonance Imaging of Spine showed mass lesion in epidural space compressing cord from behind without any bony involvement atD7 to D10 vertebral segment. Mass lesion was isointense on T1 and hyper-intense on T2 with no contrast enhancement [Figure , ].\nD7 to D10 Laminectomy with complete excision of firm epidural mass, which could be separated from dura with difficulty, was carried out. Histopathology revealed fibro-collagenous tissue with dense infiltration of lymphocytes, histiocytes, plasma cells, eosinophils, and multinucleated Touton giant cells. Histiocytic granulomas were seen. Presence of arteritis and panniculitis noted. Overall morphology was suggestive of JXG. Immune marker study was proposed and revealed Viamentin positive, Cluster Differentiation-68 positive in Touton giant cell and negative for S-100, CD1a, and Actin smooth muscle [Figure –].\nPost-operative, patient improved neurologically. His power improved to grade 5/5 with decreased spasticity. At 9 month follow-up patient had mild spasticity with extensor plantar. His MRI carried out at 3 months shows post-Laminectomy status with no residual tumor [].” | 0 | Write a detailed clinical case vignette based on the following key phrases: Histiocytic lesions, Spinal cord compression, Xanthoma/xanthogranuloma |
A 50-year-old male patient was referred to our hospital, with a 4-month history of neck pain and gradually progressive weakness of all four limbs. Over the past 2 weeks, the patient noticed significant deterioration and became bed bound. The patient denies any previous history of trauma, malignancy, or medical history of note. Clinical examination revealed spastic quadriparesis of 3/5 with 80% decreased sensation of all modalities below C5. Sphincters were found to be intact.\nPlain X-ray cervical spine on anterior-posterior (AP) view showed a large lucent lesion replacing the C5 vertebra with loss of the left pedicle []. The magnetic resonance imaging (MRI) of the cervical spine demonstrated a T1 and T2 isointense, expansile mass lesion involving the C5 vertebral body extending into the left pedicle but sparing neural foramina. On T2, small hyperintense intralesional cavity was noted in the anterior part. The lesion extended across the C5/6 intervertebral disc and into the anteroinferior part of the C4 vertebra. Significant cord compression with absent thecal sac against C5 was noted. The lesion enhanced homogeneously with gadolinium and appeared completely extradural [Figure -]. Based on imaging, metastasis, myeloma, and tuberculous granuloma were suspected.\nThe patient underwent standard anterior cervical decompression and fixation. Peroperatively, tumor appeared as a well-encapsulated, firm, multinodular mass, yellow tan in color, 3 cm × 3 cm × 2 cm in size. There was a small necrotic part corresponding to the T2 hyperintensity. The lesion was excised in piecemeal fashion [] to completely decompress the cord. No clue of bone was found within the mass. At this point, differential was further narrowed to granulomatous lesion like tuberculosis. Histology revealed sheets of histiocytes with foamy cytoplasm and bland nuclei. Focal cholesterol clefts and multinucleated giant cells were also noticed. There was no cellular atypia. CD68 was positive and S100 was found to be negative [Figure and ].\nThe serum calcium was normal, and lipid profile showed normolipemia. Clinical examination did not reveal any soft-tissue xanthomas. The patient was able to return to his normal activities 4 weeks after the operation. Postoperative imaging as per routine following anterior decompression and fixation consisted of plain X-ray cervical spine AP and lateral views [Figure and ]. Postoperative computed tomography (CT) of the same area was planned at the 6-month follow-up both to see the integrity of fixation as well as to rule out the tumor recurrence, but the patient was not able to reach out for imaging because of pandemic restrictions. Currently, he is 1 year out of his surgery, with no reported complaint as per telephonic follow-up. | The second child (Fig. ) of a 29-year-old woman was spontaneously born at 39 + 4 weeks of gestation after an unremarkable pregnancy. Birth weight was 3510 g (50th percentile), length 55 cm (75th percentile) and head circumference 33.5 cm (20th percentile). Apgar scores were 9/10/10. At the age of 3 months the girl was seen by a general pediatrician and consecutively referred to our hospital because of a recently developed mass on the left temple. The subcutaneous swelling was about 2 cm in diameter, non-moveable, not reddish or overheated and not painful. Furthermore, the mother reported recurrent fever spikes up to 38.5 °C without signs of inflammation for about 4 weeks. Defecation and drinking habits were adequate, vomiting was denied. However, a weight loss of 200 g within 3 weeks was obvious. In addition to a pale skin color and three pinhead-large livid subcutaneous lesions located on the trunk and the lower extremities, there was a left-sided rib hump situated at the level of Th6 to Th10; a secondary finding was oral candidiasis. Laboratory values on admission showed: hemoglobin 85 g/l, hematocrit 0.24 L/l, thrombocytes 380 G/l, lactate dehydrogenase 308 U/l, alpha-1-fetoprotein 225.6 ng/ml, beta-human chorionic gonadotropin < 1 mU/ml, c-reactive protein 10.13 mg/dl, interleukin-6 45.8 pg/ml and procalcitonin 0.31 ng/ml. To define the extent of disease, whole-body magnetic resonance imaging (MRI) (Fig. ) was performed. An intraosseous soft tissue lesion in the left sphenoid bone (diameter 18 × 20 mm), a big paravertebral thoracic tumor conglomerate (diameter 85 × 59 mm), multiple papules to nodules in the liver (7 mm), in both kidneys (6 mm) and lungs (3 × 4.3 mm) and in the pancreatic head (3.5 mm), as well as cutaneous (5 mm) and intraosseous lesions were found. A vertebra plana of Th9, together with infiltration of the adjacent Th8 and Th10, resulting in a kinking of the spinal column compromising the spinal canal and obliteration of nerve roots by soft tissue tumor mass was seen. Due to the lesion in the skull and the vertebra plana, Langerhans cell histiocytosis was one of the primary differential diagnoses. But the histology of one cutaneous lesion of the trunk did not confirm this diagnosis. Rapid deterioration with paraplegia prompted us to administer immunosuppressive treatment immediately. Based on the presumed diagnosis of a neoplasia of the Ewing / PNET group the patient was initially treated according to the Euro-Ewing protocol. After the third biopsy and histological examination two independent pathology centers confirmed the diagnosis of xanthosiderohistiocytosis, which is not well-defined and is regarded as a morphologic variant of xanthoma disseminatum – a type that most often occurs in adult patients with monoclonal gammopathy (Fig. ). In keeping with the established diagnosis, Langerhans cell histiocytosis-based chemotherapy treatment was administered. Following the arm for the high-risk group, the chemotherapy agents included prednisone, vinblastine, 6-mercaptopurine and methotrexate. With this therapy the primary tumor mass decreased. Clinical and radiologic examinations at the age of 3 years show partial remission after 1 year maintenance chemotherapy with puri-nethol and methotrexate. | 0 | Write a detailed clinical case vignette based on the following key phrases: Histiocytic lesions, Spinal cord compression, Xanthoma/xanthogranuloma |
The second child (Fig. ) of a 29-year-old woman was spontaneously born at 39 + 4 weeks of gestation after an unremarkable pregnancy. Birth weight was 3510 g (50th percentile), length 55 cm (75th percentile) and head circumference 33.5 cm (20th percentile). Apgar scores were 9/10/10. At the age of 3 months the girl was seen by a general pediatrician and consecutively referred to our hospital because of a recently developed mass on the left temple. The subcutaneous swelling was about 2 cm in diameter, non-moveable, not reddish or overheated and not painful. Furthermore, the mother reported recurrent fever spikes up to 38.5 °C without signs of inflammation for about 4 weeks. Defecation and drinking habits were adequate, vomiting was denied. However, a weight loss of 200 g within 3 weeks was obvious. In addition to a pale skin color and three pinhead-large livid subcutaneous lesions located on the trunk and the lower extremities, there was a left-sided rib hump situated at the level of Th6 to Th10; a secondary finding was oral candidiasis. Laboratory values on admission showed: hemoglobin 85 g/l, hematocrit 0.24 L/l, thrombocytes 380 G/l, lactate dehydrogenase 308 U/l, alpha-1-fetoprotein 225.6 ng/ml, beta-human chorionic gonadotropin < 1 mU/ml, c-reactive protein 10.13 mg/dl, interleukin-6 45.8 pg/ml and procalcitonin 0.31 ng/ml. To define the extent of disease, whole-body magnetic resonance imaging (MRI) (Fig. ) was performed. An intraosseous soft tissue lesion in the left sphenoid bone (diameter 18 × 20 mm), a big paravertebral thoracic tumor conglomerate (diameter 85 × 59 mm), multiple papules to nodules in the liver (7 mm), in both kidneys (6 mm) and lungs (3 × 4.3 mm) and in the pancreatic head (3.5 mm), as well as cutaneous (5 mm) and intraosseous lesions were found. A vertebra plana of Th9, together with infiltration of the adjacent Th8 and Th10, resulting in a kinking of the spinal column compromising the spinal canal and obliteration of nerve roots by soft tissue tumor mass was seen. Due to the lesion in the skull and the vertebra plana, Langerhans cell histiocytosis was one of the primary differential diagnoses. But the histology of one cutaneous lesion of the trunk did not confirm this diagnosis. Rapid deterioration with paraplegia prompted us to administer immunosuppressive treatment immediately. Based on the presumed diagnosis of a neoplasia of the Ewing / PNET group the patient was initially treated according to the Euro-Ewing protocol. After the third biopsy and histological examination two independent pathology centers confirmed the diagnosis of xanthosiderohistiocytosis, which is not well-defined and is regarded as a morphologic variant of xanthoma disseminatum – a type that most often occurs in adult patients with monoclonal gammopathy (Fig. ). In keeping with the established diagnosis, Langerhans cell histiocytosis-based chemotherapy treatment was administered. Following the arm for the high-risk group, the chemotherapy agents included prednisone, vinblastine, 6-mercaptopurine and methotrexate. With this therapy the primary tumor mass decreased. Clinical and radiologic examinations at the age of 3 years show partial remission after 1 year maintenance chemotherapy with puri-nethol and methotrexate. | The mother of a 1-year-old girl initially noticed reduced activity of the lower limbs which worsened over 8 days. On examination paraplegia, exaggerated deep tendon reflexes and positive Babinski sign were noted. No other neurological deficit found. Cutaneous lesions were absent. Magnetic resonance imaging (MRI) of the spine showed intradural extramedullary (IDEM) lesion at D6–D8 level suggestive of a benign nerve sheath tumor [].\nTotal excision of the tumor was done. Gross specimen included multiple bits yellowish-white, soft to firm. Histopathology showed tumor composed of sheets of spherical, ovoid, and spindle cells with vesicular bland nuclei. Many Touton giant cells were seen [].\nOn immunohistochemistry, the tumor cells were strongly positive for CD68 (histiocytic marker) and weakly for CD163 []. Cells were negative for s100 protein, CD1a, glial fibrillary acidic protein, epithelial membrane antigen. Special stain such as Zeil Neelson, Gomori methenamine silver, and periodic acid-Schiff stain were negative, thus ruling out mycobacteria and fungi as etiologic agents. Thus, a diagnosis of JXG was made. The infant showed full clinical recovery. The patient was managed with regular follow-up only and is doing well until date. | 0 | Write a detailed clinical case vignette based on the following key phrases: Histiocytic lesions, Spinal cord compression, Xanthoma/xanthogranuloma |
The second child (Fig. ) of a 29-year-old woman was spontaneously born at 39 + 4 weeks of gestation after an unremarkable pregnancy. Birth weight was 3510 g (50th percentile), length 55 cm (75th percentile) and head circumference 33.5 cm (20th percentile). Apgar scores were 9/10/10. At the age of 3 months the girl was seen by a general pediatrician and consecutively referred to our hospital because of a recently developed mass on the left temple. The subcutaneous swelling was about 2 cm in diameter, non-moveable, not reddish or overheated and not painful. Furthermore, the mother reported recurrent fever spikes up to 38.5 °C without signs of inflammation for about 4 weeks. Defecation and drinking habits were adequate, vomiting was denied. However, a weight loss of 200 g within 3 weeks was obvious. In addition to a pale skin color and three pinhead-large livid subcutaneous lesions located on the trunk and the lower extremities, there was a left-sided rib hump situated at the level of Th6 to Th10; a secondary finding was oral candidiasis. Laboratory values on admission showed: hemoglobin 85 g/l, hematocrit 0.24 L/l, thrombocytes 380 G/l, lactate dehydrogenase 308 U/l, alpha-1-fetoprotein 225.6 ng/ml, beta-human chorionic gonadotropin < 1 mU/ml, c-reactive protein 10.13 mg/dl, interleukin-6 45.8 pg/ml and procalcitonin 0.31 ng/ml. To define the extent of disease, whole-body magnetic resonance imaging (MRI) (Fig. ) was performed. An intraosseous soft tissue lesion in the left sphenoid bone (diameter 18 × 20 mm), a big paravertebral thoracic tumor conglomerate (diameter 85 × 59 mm), multiple papules to nodules in the liver (7 mm), in both kidneys (6 mm) and lungs (3 × 4.3 mm) and in the pancreatic head (3.5 mm), as well as cutaneous (5 mm) and intraosseous lesions were found. A vertebra plana of Th9, together with infiltration of the adjacent Th8 and Th10, resulting in a kinking of the spinal column compromising the spinal canal and obliteration of nerve roots by soft tissue tumor mass was seen. Due to the lesion in the skull and the vertebra plana, Langerhans cell histiocytosis was one of the primary differential diagnoses. But the histology of one cutaneous lesion of the trunk did not confirm this diagnosis. Rapid deterioration with paraplegia prompted us to administer immunosuppressive treatment immediately. Based on the presumed diagnosis of a neoplasia of the Ewing / PNET group the patient was initially treated according to the Euro-Ewing protocol. After the third biopsy and histological examination two independent pathology centers confirmed the diagnosis of xanthosiderohistiocytosis, which is not well-defined and is regarded as a morphologic variant of xanthoma disseminatum – a type that most often occurs in adult patients with monoclonal gammopathy (Fig. ). In keeping with the established diagnosis, Langerhans cell histiocytosis-based chemotherapy treatment was administered. Following the arm for the high-risk group, the chemotherapy agents included prednisone, vinblastine, 6-mercaptopurine and methotrexate. With this therapy the primary tumor mass decreased. Clinical and radiologic examinations at the age of 3 years show partial remission after 1 year maintenance chemotherapy with puri-nethol and methotrexate. | A 15-year-old male presented with pain in the nape of the neck and gradually progressive ascending weakness of all four limbs along with bladder and bowel involvement for 3 years. On examination, tone was increased in all four limbs with 4/5 power in bilateral upper limbs and 2/5 power in both lower limbs, along with exaggerated deep tendon reflexes in all four limbs. Superficial abdominal reflexes were absent and, bilateral planters were extensor. He had 70–80% sensory loss below T4 level. His routine laboratory studies including hematological and biochemical parameters were within normal limits.\nComputerised tomography scan of the cervical spine revealed a large osseo-destructive lesion involving body of C5and C6 vertebra which was causing spinal canal compromise [Figure and ]. The lesion was involving surrounding structures with extension into the submuscular plane anteriorly. On magnetic resonance imaging (MRI) evaluation, the mass was isointense on T1 and T2 weighted sequences with minimal contrast enhancement. It was causing distrucation of C5 and C6 vertebra body with compression of thecal sac [Figure –].\nUsing anterior cervical approach, the tumor resection was performed. Intra-operatively, the tumor involved whole of the C5 and C6 vertebral body. There was a soft tissue component of the tumor, which was extending into the submuscular plane. Inside the capsule, the tumor consisted of cheesy material that was minimally vascular. Total tumor excision was done with tricorticate iliac crest bone grafting and plate and screw fixation between C4 and C7 vertebral bodies.\nMicroscopic examination showed spindle cells disposed of in intersecting fascicles intermingled with mononuclear xanthomatous cells displaying round nuclei with dense chromatin inconspicuous nucleoli and pale eosinophilic finely vacuolated chromatin. Spindle cells display bland appearing nuclear chromatin and eosinophilic cytoplasm. Occasional cells displayed nuclear atypia and enlargement. Scattered neutrophils were seen admixed with these cells. Focal areas of calcification and occasional osteoclasts like giant cells were also seen. Interspersed areas showed degenerated bony trabeculae and cartilage [Figure and ].\nPostoperatively, patient had significant improvement in neck pain and spasticity in all four limbs. At follow-up of 6 months, he was doing well. | 0 | Write a detailed clinical case vignette based on the following key phrases: Histiocytic lesions, Spinal cord compression, Xanthoma/xanthogranuloma |
A 29-year-old male patient presented with right side weakness and left side numbness that occurred just after a car accident. Upon physical examination, there was no abnormal finding, except for tenderness in the upper cervical area. Routine laboratory tests demonstrated normal results. Motor weakness of right side extremity was disclosed as 1/5 for arm and 2/5 for leg with slightly hyperactivity of deep tendon reflex. Sensory examination showed significantly decreased pain, temperature, vibration, and discriminatory touch sensations below the left C2 dermatome. Babinski's reflex and Hoffman's sign were present on the left side. Based on the symptoms and neurological findings, the assumptive diagnosis of Brown-Sequard syndrome was made. The patient had no symptoms or past medical history related to a cervical spine lesion before the trauma. Cervical plain films revealed straightening of the cervical spine with loss of normal lordosis and widening between the posterior arch of the atlas and spinous process of the axis. A computed tomography (CT) scan of the upper cervical spine revealed a large round mass which measured 25×18×24 mm with relatively homogeneous strong enhancement and was located between the C1 and C2 interspinous space. In addition, the CT scan showed cortical osseous erosions at the lower C1 posterior arc and upper C2 spinous process. Cervical magnetic resonance images (MRI) revealed an epidural dumbbell-shaped mass traversing the C1 to C2 interspinous space, compressing the surrounding structures (). The spinal cord was compressed by the lesion which was isointense on T1-weighted image (WI) and mixed hypointense on T2WI with homogenous strong enhanced after gadolinium administration. Radiologic analysis indicated possible lymphoma, meningioma, schwannoma or other neuronal tumors.\nThe patient underwent a surgery immediately for decompression of the spinal cord. A total tumor resection was performed with only a partial laminectomy from the lower half of the C1 to upper half of C2 without any instrumentation and access to the tumor. After partial lamicectomy, an extradural and yellowish mass was exposed with well-demarcated from the surrounding structures but adhesive at the epidural area. Under microscopy, the tumor was delicately dissected, and en bloc removal was achieved (). The mass was ovoid, soft like gelatin, avascular, and well-encapsulated, measuring 25 mm at the largest diameter, and was adherented to the outer layer of the dura mater. And, there was no root invasion. On the basis of the clinical and operative features, we thought that the lesion originated from the outer aspect of the dura mater. Upon sectioning, the mass was particularly yellow and white with cystic components ().\nAfter surgery, the patient's right hemiparesis fully recovered in 2 weeks. However, a slightly tingling numbness on his left arm remained and was controlled through medication in the outpatient clinic for 4 months. Currently, the patient has no symptoms without instability of cervical spine. Cervical MRI with gadolinium enhancement at 2 years after surgery demonstrated no recurrent lesion ().\nHistological examination of the tumor uncovered focal aggregation cells with ample, clear, and foamy cytoplasm in a background of loose, fibrous stroma of xanthic appearance. Large, round cells had irregular vesicular nuclei which were regular and small with condensed chromatin (). The specimen was underwent to immunohistochemical testing, yielding strongly positive for CD 68 and lysozyme stain, which confirmed that the large foam cells were histiocytes. The test with CD-1a was negative, thus ruling out Langerhans' cell histiocytosis. GFAP stain and S-100 protein were negative. Thus, a diagnosis of xanthogranuloma was established. | The second child (Fig. ) of a 29-year-old woman was spontaneously born at 39 + 4 weeks of gestation after an unremarkable pregnancy. Birth weight was 3510 g (50th percentile), length 55 cm (75th percentile) and head circumference 33.5 cm (20th percentile). Apgar scores were 9/10/10. At the age of 3 months the girl was seen by a general pediatrician and consecutively referred to our hospital because of a recently developed mass on the left temple. The subcutaneous swelling was about 2 cm in diameter, non-moveable, not reddish or overheated and not painful. Furthermore, the mother reported recurrent fever spikes up to 38.5 °C without signs of inflammation for about 4 weeks. Defecation and drinking habits were adequate, vomiting was denied. However, a weight loss of 200 g within 3 weeks was obvious. In addition to a pale skin color and three pinhead-large livid subcutaneous lesions located on the trunk and the lower extremities, there was a left-sided rib hump situated at the level of Th6 to Th10; a secondary finding was oral candidiasis. Laboratory values on admission showed: hemoglobin 85 g/l, hematocrit 0.24 L/l, thrombocytes 380 G/l, lactate dehydrogenase 308 U/l, alpha-1-fetoprotein 225.6 ng/ml, beta-human chorionic gonadotropin < 1 mU/ml, c-reactive protein 10.13 mg/dl, interleukin-6 45.8 pg/ml and procalcitonin 0.31 ng/ml. To define the extent of disease, whole-body magnetic resonance imaging (MRI) (Fig. ) was performed. An intraosseous soft tissue lesion in the left sphenoid bone (diameter 18 × 20 mm), a big paravertebral thoracic tumor conglomerate (diameter 85 × 59 mm), multiple papules to nodules in the liver (7 mm), in both kidneys (6 mm) and lungs (3 × 4.3 mm) and in the pancreatic head (3.5 mm), as well as cutaneous (5 mm) and intraosseous lesions were found. A vertebra plana of Th9, together with infiltration of the adjacent Th8 and Th10, resulting in a kinking of the spinal column compromising the spinal canal and obliteration of nerve roots by soft tissue tumor mass was seen. Due to the lesion in the skull and the vertebra plana, Langerhans cell histiocytosis was one of the primary differential diagnoses. But the histology of one cutaneous lesion of the trunk did not confirm this diagnosis. Rapid deterioration with paraplegia prompted us to administer immunosuppressive treatment immediately. Based on the presumed diagnosis of a neoplasia of the Ewing / PNET group the patient was initially treated according to the Euro-Ewing protocol. After the third biopsy and histological examination two independent pathology centers confirmed the diagnosis of xanthosiderohistiocytosis, which is not well-defined and is regarded as a morphologic variant of xanthoma disseminatum – a type that most often occurs in adult patients with monoclonal gammopathy (Fig. ). In keeping with the established diagnosis, Langerhans cell histiocytosis-based chemotherapy treatment was administered. Following the arm for the high-risk group, the chemotherapy agents included prednisone, vinblastine, 6-mercaptopurine and methotrexate. With this therapy the primary tumor mass decreased. Clinical and radiologic examinations at the age of 3 years show partial remission after 1 year maintenance chemotherapy with puri-nethol and methotrexate. | 0 | Write a detailed clinical case vignette based on the following key phrases: Histiocytic lesions, Spinal cord compression, Xanthoma/xanthogranuloma |
A 50-year-old male patient was referred to our hospital, with a 4-month history of neck pain and gradually progressive weakness of all four limbs. Over the past 2 weeks, the patient noticed significant deterioration and became bed bound. The patient denies any previous history of trauma, malignancy, or medical history of note. Clinical examination revealed spastic quadriparesis of 3/5 with 80% decreased sensation of all modalities below C5. Sphincters were found to be intact.\nPlain X-ray cervical spine on anterior-posterior (AP) view showed a large lucent lesion replacing the C5 vertebra with loss of the left pedicle []. The magnetic resonance imaging (MRI) of the cervical spine demonstrated a T1 and T2 isointense, expansile mass lesion involving the C5 vertebral body extending into the left pedicle but sparing neural foramina. On T2, small hyperintense intralesional cavity was noted in the anterior part. The lesion extended across the C5/6 intervertebral disc and into the anteroinferior part of the C4 vertebra. Significant cord compression with absent thecal sac against C5 was noted. The lesion enhanced homogeneously with gadolinium and appeared completely extradural [Figure -]. Based on imaging, metastasis, myeloma, and tuberculous granuloma were suspected.\nThe patient underwent standard anterior cervical decompression and fixation. Peroperatively, tumor appeared as a well-encapsulated, firm, multinodular mass, yellow tan in color, 3 cm × 3 cm × 2 cm in size. There was a small necrotic part corresponding to the T2 hyperintensity. The lesion was excised in piecemeal fashion [] to completely decompress the cord. No clue of bone was found within the mass. At this point, differential was further narrowed to granulomatous lesion like tuberculosis. Histology revealed sheets of histiocytes with foamy cytoplasm and bland nuclei. Focal cholesterol clefts and multinucleated giant cells were also noticed. There was no cellular atypia. CD68 was positive and S100 was found to be negative [Figure and ].\nThe serum calcium was normal, and lipid profile showed normolipemia. Clinical examination did not reveal any soft-tissue xanthomas. The patient was able to return to his normal activities 4 weeks after the operation. Postoperative imaging as per routine following anterior decompression and fixation consisted of plain X-ray cervical spine AP and lateral views [Figure and ]. Postoperative computed tomography (CT) of the same area was planned at the 6-month follow-up both to see the integrity of fixation as well as to rule out the tumor recurrence, but the patient was not able to reach out for imaging because of pandemic restrictions. Currently, he is 1 year out of his surgery, with no reported complaint as per telephonic follow-up. | The mother of a 1-year-old girl initially noticed reduced activity of the lower limbs which worsened over 8 days. On examination paraplegia, exaggerated deep tendon reflexes and positive Babinski sign were noted. No other neurological deficit found. Cutaneous lesions were absent. Magnetic resonance imaging (MRI) of the spine showed intradural extramedullary (IDEM) lesion at D6–D8 level suggestive of a benign nerve sheath tumor [].\nTotal excision of the tumor was done. Gross specimen included multiple bits yellowish-white, soft to firm. Histopathology showed tumor composed of sheets of spherical, ovoid, and spindle cells with vesicular bland nuclei. Many Touton giant cells were seen [].\nOn immunohistochemistry, the tumor cells were strongly positive for CD68 (histiocytic marker) and weakly for CD163 []. Cells were negative for s100 protein, CD1a, glial fibrillary acidic protein, epithelial membrane antigen. Special stain such as Zeil Neelson, Gomori methenamine silver, and periodic acid-Schiff stain were negative, thus ruling out mycobacteria and fungi as etiologic agents. Thus, a diagnosis of JXG was made. The infant showed full clinical recovery. The patient was managed with regular follow-up only and is doing well until date. | 0 | Write a detailed clinical case vignette based on the following key phrases: Histiocytic lesions, Spinal cord compression, Xanthoma/xanthogranuloma |
A 50-year-old male patient was referred to our hospital, with a 4-month history of neck pain and gradually progressive weakness of all four limbs. Over the past 2 weeks, the patient noticed significant deterioration and became bed bound. The patient denies any previous history of trauma, malignancy, or medical history of note. Clinical examination revealed spastic quadriparesis of 3/5 with 80% decreased sensation of all modalities below C5. Sphincters were found to be intact.\nPlain X-ray cervical spine on anterior-posterior (AP) view showed a large lucent lesion replacing the C5 vertebra with loss of the left pedicle []. The magnetic resonance imaging (MRI) of the cervical spine demonstrated a T1 and T2 isointense, expansile mass lesion involving the C5 vertebral body extending into the left pedicle but sparing neural foramina. On T2, small hyperintense intralesional cavity was noted in the anterior part. The lesion extended across the C5/6 intervertebral disc and into the anteroinferior part of the C4 vertebra. Significant cord compression with absent thecal sac against C5 was noted. The lesion enhanced homogeneously with gadolinium and appeared completely extradural [Figure -]. Based on imaging, metastasis, myeloma, and tuberculous granuloma were suspected.\nThe patient underwent standard anterior cervical decompression and fixation. Peroperatively, tumor appeared as a well-encapsulated, firm, multinodular mass, yellow tan in color, 3 cm × 3 cm × 2 cm in size. There was a small necrotic part corresponding to the T2 hyperintensity. The lesion was excised in piecemeal fashion [] to completely decompress the cord. No clue of bone was found within the mass. At this point, differential was further narrowed to granulomatous lesion like tuberculosis. Histology revealed sheets of histiocytes with foamy cytoplasm and bland nuclei. Focal cholesterol clefts and multinucleated giant cells were also noticed. There was no cellular atypia. CD68 was positive and S100 was found to be negative [Figure and ].\nThe serum calcium was normal, and lipid profile showed normolipemia. Clinical examination did not reveal any soft-tissue xanthomas. The patient was able to return to his normal activities 4 weeks after the operation. Postoperative imaging as per routine following anterior decompression and fixation consisted of plain X-ray cervical spine AP and lateral views [Figure and ]. Postoperative computed tomography (CT) of the same area was planned at the 6-month follow-up both to see the integrity of fixation as well as to rule out the tumor recurrence, but the patient was not able to reach out for imaging because of pandemic restrictions. Currently, he is 1 year out of his surgery, with no reported complaint as per telephonic follow-up. | A 15-year-old male presented with pain in the nape of the neck and gradually progressive ascending weakness of all four limbs along with bladder and bowel involvement for 3 years. On examination, tone was increased in all four limbs with 4/5 power in bilateral upper limbs and 2/5 power in both lower limbs, along with exaggerated deep tendon reflexes in all four limbs. Superficial abdominal reflexes were absent and, bilateral planters were extensor. He had 70–80% sensory loss below T4 level. His routine laboratory studies including hematological and biochemical parameters were within normal limits.\nComputerised tomography scan of the cervical spine revealed a large osseo-destructive lesion involving body of C5and C6 vertebra which was causing spinal canal compromise [Figure and ]. The lesion was involving surrounding structures with extension into the submuscular plane anteriorly. On magnetic resonance imaging (MRI) evaluation, the mass was isointense on T1 and T2 weighted sequences with minimal contrast enhancement. It was causing distrucation of C5 and C6 vertebra body with compression of thecal sac [Figure –].\nUsing anterior cervical approach, the tumor resection was performed. Intra-operatively, the tumor involved whole of the C5 and C6 vertebral body. There was a soft tissue component of the tumor, which was extending into the submuscular plane. Inside the capsule, the tumor consisted of cheesy material that was minimally vascular. Total tumor excision was done with tricorticate iliac crest bone grafting and plate and screw fixation between C4 and C7 vertebral bodies.\nMicroscopic examination showed spindle cells disposed of in intersecting fascicles intermingled with mononuclear xanthomatous cells displaying round nuclei with dense chromatin inconspicuous nucleoli and pale eosinophilic finely vacuolated chromatin. Spindle cells display bland appearing nuclear chromatin and eosinophilic cytoplasm. Occasional cells displayed nuclear atypia and enlargement. Scattered neutrophils were seen admixed with these cells. Focal areas of calcification and occasional osteoclasts like giant cells were also seen. Interspersed areas showed degenerated bony trabeculae and cartilage [Figure and ].\nPostoperatively, patient had significant improvement in neck pain and spasticity in all four limbs. At follow-up of 6 months, he was doing well. | 0 | Write a detailed clinical case vignette based on the following key phrases: Histiocytic lesions, Spinal cord compression, Xanthoma/xanthogranuloma |
A 50-year-old male patient was referred to our hospital, with a 4-month history of neck pain and gradually progressive weakness of all four limbs. Over the past 2 weeks, the patient noticed significant deterioration and became bed bound. The patient denies any previous history of trauma, malignancy, or medical history of note. Clinical examination revealed spastic quadriparesis of 3/5 with 80% decreased sensation of all modalities below C5. Sphincters were found to be intact.\nPlain X-ray cervical spine on anterior-posterior (AP) view showed a large lucent lesion replacing the C5 vertebra with loss of the left pedicle []. The magnetic resonance imaging (MRI) of the cervical spine demonstrated a T1 and T2 isointense, expansile mass lesion involving the C5 vertebral body extending into the left pedicle but sparing neural foramina. On T2, small hyperintense intralesional cavity was noted in the anterior part. The lesion extended across the C5/6 intervertebral disc and into the anteroinferior part of the C4 vertebra. Significant cord compression with absent thecal sac against C5 was noted. The lesion enhanced homogeneously with gadolinium and appeared completely extradural [Figure -]. Based on imaging, metastasis, myeloma, and tuberculous granuloma were suspected.\nThe patient underwent standard anterior cervical decompression and fixation. Peroperatively, tumor appeared as a well-encapsulated, firm, multinodular mass, yellow tan in color, 3 cm × 3 cm × 2 cm in size. There was a small necrotic part corresponding to the T2 hyperintensity. The lesion was excised in piecemeal fashion [] to completely decompress the cord. No clue of bone was found within the mass. At this point, differential was further narrowed to granulomatous lesion like tuberculosis. Histology revealed sheets of histiocytes with foamy cytoplasm and bland nuclei. Focal cholesterol clefts and multinucleated giant cells were also noticed. There was no cellular atypia. CD68 was positive and S100 was found to be negative [Figure and ].\nThe serum calcium was normal, and lipid profile showed normolipemia. Clinical examination did not reveal any soft-tissue xanthomas. The patient was able to return to his normal activities 4 weeks after the operation. Postoperative imaging as per routine following anterior decompression and fixation consisted of plain X-ray cervical spine AP and lateral views [Figure and ]. Postoperative computed tomography (CT) of the same area was planned at the 6-month follow-up both to see the integrity of fixation as well as to rule out the tumor recurrence, but the patient was not able to reach out for imaging because of pandemic restrictions. Currently, he is 1 year out of his surgery, with no reported complaint as per telephonic follow-up. | A 29-year-old male patient presented with right side weakness and left side numbness that occurred just after a car accident. Upon physical examination, there was no abnormal finding, except for tenderness in the upper cervical area. Routine laboratory tests demonstrated normal results. Motor weakness of right side extremity was disclosed as 1/5 for arm and 2/5 for leg with slightly hyperactivity of deep tendon reflex. Sensory examination showed significantly decreased pain, temperature, vibration, and discriminatory touch sensations below the left C2 dermatome. Babinski's reflex and Hoffman's sign were present on the left side. Based on the symptoms and neurological findings, the assumptive diagnosis of Brown-Sequard syndrome was made. The patient had no symptoms or past medical history related to a cervical spine lesion before the trauma. Cervical plain films revealed straightening of the cervical spine with loss of normal lordosis and widening between the posterior arch of the atlas and spinous process of the axis. A computed tomography (CT) scan of the upper cervical spine revealed a large round mass which measured 25×18×24 mm with relatively homogeneous strong enhancement and was located between the C1 and C2 interspinous space. In addition, the CT scan showed cortical osseous erosions at the lower C1 posterior arc and upper C2 spinous process. Cervical magnetic resonance images (MRI) revealed an epidural dumbbell-shaped mass traversing the C1 to C2 interspinous space, compressing the surrounding structures (). The spinal cord was compressed by the lesion which was isointense on T1-weighted image (WI) and mixed hypointense on T2WI with homogenous strong enhanced after gadolinium administration. Radiologic analysis indicated possible lymphoma, meningioma, schwannoma or other neuronal tumors.\nThe patient underwent a surgery immediately for decompression of the spinal cord. A total tumor resection was performed with only a partial laminectomy from the lower half of the C1 to upper half of C2 without any instrumentation and access to the tumor. After partial lamicectomy, an extradural and yellowish mass was exposed with well-demarcated from the surrounding structures but adhesive at the epidural area. Under microscopy, the tumor was delicately dissected, and en bloc removal was achieved (). The mass was ovoid, soft like gelatin, avascular, and well-encapsulated, measuring 25 mm at the largest diameter, and was adherented to the outer layer of the dura mater. And, there was no root invasion. On the basis of the clinical and operative features, we thought that the lesion originated from the outer aspect of the dura mater. Upon sectioning, the mass was particularly yellow and white with cystic components ().\nAfter surgery, the patient's right hemiparesis fully recovered in 2 weeks. However, a slightly tingling numbness on his left arm remained and was controlled through medication in the outpatient clinic for 4 months. Currently, the patient has no symptoms without instability of cervical spine. Cervical MRI with gadolinium enhancement at 2 years after surgery demonstrated no recurrent lesion ().\nHistological examination of the tumor uncovered focal aggregation cells with ample, clear, and foamy cytoplasm in a background of loose, fibrous stroma of xanthic appearance. Large, round cells had irregular vesicular nuclei which were regular and small with condensed chromatin (). The specimen was underwent to immunohistochemical testing, yielding strongly positive for CD 68 and lysozyme stain, which confirmed that the large foam cells were histiocytes. The test with CD-1a was negative, thus ruling out Langerhans' cell histiocytosis. GFAP stain and S-100 protein were negative. Thus, a diagnosis of xanthogranuloma was established. | 0 | Write a detailed clinical case vignette based on the following key phrases: Histiocytic lesions, Spinal cord compression, Xanthoma/xanthogranuloma |
A 15-year-old male presented with pain in the nape of the neck and gradually progressive ascending weakness of all four limbs along with bladder and bowel involvement for 3 years. On examination, tone was increased in all four limbs with 4/5 power in bilateral upper limbs and 2/5 power in both lower limbs, along with exaggerated deep tendon reflexes in all four limbs. Superficial abdominal reflexes were absent and, bilateral planters were extensor. He had 70–80% sensory loss below T4 level. His routine laboratory studies including hematological and biochemical parameters were within normal limits.\nComputerised tomography scan of the cervical spine revealed a large osseo-destructive lesion involving body of C5and C6 vertebra which was causing spinal canal compromise [Figure and ]. The lesion was involving surrounding structures with extension into the submuscular plane anteriorly. On magnetic resonance imaging (MRI) evaluation, the mass was isointense on T1 and T2 weighted sequences with minimal contrast enhancement. It was causing distrucation of C5 and C6 vertebra body with compression of thecal sac [Figure –].\nUsing anterior cervical approach, the tumor resection was performed. Intra-operatively, the tumor involved whole of the C5 and C6 vertebral body. There was a soft tissue component of the tumor, which was extending into the submuscular plane. Inside the capsule, the tumor consisted of cheesy material that was minimally vascular. Total tumor excision was done with tricorticate iliac crest bone grafting and plate and screw fixation between C4 and C7 vertebral bodies.\nMicroscopic examination showed spindle cells disposed of in intersecting fascicles intermingled with mononuclear xanthomatous cells displaying round nuclei with dense chromatin inconspicuous nucleoli and pale eosinophilic finely vacuolated chromatin. Spindle cells display bland appearing nuclear chromatin and eosinophilic cytoplasm. Occasional cells displayed nuclear atypia and enlargement. Scattered neutrophils were seen admixed with these cells. Focal areas of calcification and occasional osteoclasts like giant cells were also seen. Interspersed areas showed degenerated bony trabeculae and cartilage [Figure and ].\nPostoperatively, patient had significant improvement in neck pain and spasticity in all four limbs. At follow-up of 6 months, he was doing well. | The mother of a 1-year-old girl initially noticed reduced activity of the lower limbs which worsened over 8 days. On examination paraplegia, exaggerated deep tendon reflexes and positive Babinski sign were noted. No other neurological deficit found. Cutaneous lesions were absent. Magnetic resonance imaging (MRI) of the spine showed intradural extramedullary (IDEM) lesion at D6–D8 level suggestive of a benign nerve sheath tumor [].\nTotal excision of the tumor was done. Gross specimen included multiple bits yellowish-white, soft to firm. Histopathology showed tumor composed of sheets of spherical, ovoid, and spindle cells with vesicular bland nuclei. Many Touton giant cells were seen [].\nOn immunohistochemistry, the tumor cells were strongly positive for CD68 (histiocytic marker) and weakly for CD163 []. Cells were negative for s100 protein, CD1a, glial fibrillary acidic protein, epithelial membrane antigen. Special stain such as Zeil Neelson, Gomori methenamine silver, and periodic acid-Schiff stain were negative, thus ruling out mycobacteria and fungi as etiologic agents. Thus, a diagnosis of JXG was made. The infant showed full clinical recovery. The patient was managed with regular follow-up only and is doing well until date. | 0 | Write a detailed clinical case vignette based on the following key phrases: Histiocytic lesions, Spinal cord compression, Xanthoma/xanthogranuloma |
A 29-year-old male patient presented with right side weakness and left side numbness that occurred just after a car accident. Upon physical examination, there was no abnormal finding, except for tenderness in the upper cervical area. Routine laboratory tests demonstrated normal results. Motor weakness of right side extremity was disclosed as 1/5 for arm and 2/5 for leg with slightly hyperactivity of deep tendon reflex. Sensory examination showed significantly decreased pain, temperature, vibration, and discriminatory touch sensations below the left C2 dermatome. Babinski's reflex and Hoffman's sign were present on the left side. Based on the symptoms and neurological findings, the assumptive diagnosis of Brown-Sequard syndrome was made. The patient had no symptoms or past medical history related to a cervical spine lesion before the trauma. Cervical plain films revealed straightening of the cervical spine with loss of normal lordosis and widening between the posterior arch of the atlas and spinous process of the axis. A computed tomography (CT) scan of the upper cervical spine revealed a large round mass which measured 25×18×24 mm with relatively homogeneous strong enhancement and was located between the C1 and C2 interspinous space. In addition, the CT scan showed cortical osseous erosions at the lower C1 posterior arc and upper C2 spinous process. Cervical magnetic resonance images (MRI) revealed an epidural dumbbell-shaped mass traversing the C1 to C2 interspinous space, compressing the surrounding structures (). The spinal cord was compressed by the lesion which was isointense on T1-weighted image (WI) and mixed hypointense on T2WI with homogenous strong enhanced after gadolinium administration. Radiologic analysis indicated possible lymphoma, meningioma, schwannoma or other neuronal tumors.\nThe patient underwent a surgery immediately for decompression of the spinal cord. A total tumor resection was performed with only a partial laminectomy from the lower half of the C1 to upper half of C2 without any instrumentation and access to the tumor. After partial lamicectomy, an extradural and yellowish mass was exposed with well-demarcated from the surrounding structures but adhesive at the epidural area. Under microscopy, the tumor was delicately dissected, and en bloc removal was achieved (). The mass was ovoid, soft like gelatin, avascular, and well-encapsulated, measuring 25 mm at the largest diameter, and was adherented to the outer layer of the dura mater. And, there was no root invasion. On the basis of the clinical and operative features, we thought that the lesion originated from the outer aspect of the dura mater. Upon sectioning, the mass was particularly yellow and white with cystic components ().\nAfter surgery, the patient's right hemiparesis fully recovered in 2 weeks. However, a slightly tingling numbness on his left arm remained and was controlled through medication in the outpatient clinic for 4 months. Currently, the patient has no symptoms without instability of cervical spine. Cervical MRI with gadolinium enhancement at 2 years after surgery demonstrated no recurrent lesion ().\nHistological examination of the tumor uncovered focal aggregation cells with ample, clear, and foamy cytoplasm in a background of loose, fibrous stroma of xanthic appearance. Large, round cells had irregular vesicular nuclei which were regular and small with condensed chromatin (). The specimen was underwent to immunohistochemical testing, yielding strongly positive for CD 68 and lysozyme stain, which confirmed that the large foam cells were histiocytes. The test with CD-1a was negative, thus ruling out Langerhans' cell histiocytosis. GFAP stain and S-100 protein were negative. Thus, a diagnosis of xanthogranuloma was established. | The mother of a 1-year-old girl initially noticed reduced activity of the lower limbs which worsened over 8 days. On examination paraplegia, exaggerated deep tendon reflexes and positive Babinski sign were noted. No other neurological deficit found. Cutaneous lesions were absent. Magnetic resonance imaging (MRI) of the spine showed intradural extramedullary (IDEM) lesion at D6–D8 level suggestive of a benign nerve sheath tumor [].\nTotal excision of the tumor was done. Gross specimen included multiple bits yellowish-white, soft to firm. Histopathology showed tumor composed of sheets of spherical, ovoid, and spindle cells with vesicular bland nuclei. Many Touton giant cells were seen [].\nOn immunohistochemistry, the tumor cells were strongly positive for CD68 (histiocytic marker) and weakly for CD163 []. Cells were negative for s100 protein, CD1a, glial fibrillary acidic protein, epithelial membrane antigen. Special stain such as Zeil Neelson, Gomori methenamine silver, and periodic acid-Schiff stain were negative, thus ruling out mycobacteria and fungi as etiologic agents. Thus, a diagnosis of JXG was made. The infant showed full clinical recovery. The patient was managed with regular follow-up only and is doing well until date. | 0 | Write a detailed clinical case vignette based on the following key phrases: Histiocytic lesions, Spinal cord compression, Xanthoma/xanthogranuloma |
A 29-year-old male patient presented with right side weakness and left side numbness that occurred just after a car accident. Upon physical examination, there was no abnormal finding, except for tenderness in the upper cervical area. Routine laboratory tests demonstrated normal results. Motor weakness of right side extremity was disclosed as 1/5 for arm and 2/5 for leg with slightly hyperactivity of deep tendon reflex. Sensory examination showed significantly decreased pain, temperature, vibration, and discriminatory touch sensations below the left C2 dermatome. Babinski's reflex and Hoffman's sign were present on the left side. Based on the symptoms and neurological findings, the assumptive diagnosis of Brown-Sequard syndrome was made. The patient had no symptoms or past medical history related to a cervical spine lesion before the trauma. Cervical plain films revealed straightening of the cervical spine with loss of normal lordosis and widening between the posterior arch of the atlas and spinous process of the axis. A computed tomography (CT) scan of the upper cervical spine revealed a large round mass which measured 25×18×24 mm with relatively homogeneous strong enhancement and was located between the C1 and C2 interspinous space. In addition, the CT scan showed cortical osseous erosions at the lower C1 posterior arc and upper C2 spinous process. Cervical magnetic resonance images (MRI) revealed an epidural dumbbell-shaped mass traversing the C1 to C2 interspinous space, compressing the surrounding structures (). The spinal cord was compressed by the lesion which was isointense on T1-weighted image (WI) and mixed hypointense on T2WI with homogenous strong enhanced after gadolinium administration. Radiologic analysis indicated possible lymphoma, meningioma, schwannoma or other neuronal tumors.\nThe patient underwent a surgery immediately for decompression of the spinal cord. A total tumor resection was performed with only a partial laminectomy from the lower half of the C1 to upper half of C2 without any instrumentation and access to the tumor. After partial lamicectomy, an extradural and yellowish mass was exposed with well-demarcated from the surrounding structures but adhesive at the epidural area. Under microscopy, the tumor was delicately dissected, and en bloc removal was achieved (). The mass was ovoid, soft like gelatin, avascular, and well-encapsulated, measuring 25 mm at the largest diameter, and was adherented to the outer layer of the dura mater. And, there was no root invasion. On the basis of the clinical and operative features, we thought that the lesion originated from the outer aspect of the dura mater. Upon sectioning, the mass was particularly yellow and white with cystic components ().\nAfter surgery, the patient's right hemiparesis fully recovered in 2 weeks. However, a slightly tingling numbness on his left arm remained and was controlled through medication in the outpatient clinic for 4 months. Currently, the patient has no symptoms without instability of cervical spine. Cervical MRI with gadolinium enhancement at 2 years after surgery demonstrated no recurrent lesion ().\nHistological examination of the tumor uncovered focal aggregation cells with ample, clear, and foamy cytoplasm in a background of loose, fibrous stroma of xanthic appearance. Large, round cells had irregular vesicular nuclei which were regular and small with condensed chromatin (). The specimen was underwent to immunohistochemical testing, yielding strongly positive for CD 68 and lysozyme stain, which confirmed that the large foam cells were histiocytes. The test with CD-1a was negative, thus ruling out Langerhans' cell histiocytosis. GFAP stain and S-100 protein were negative. Thus, a diagnosis of xanthogranuloma was established. | A 15-year-old male presented with pain in the nape of the neck and gradually progressive ascending weakness of all four limbs along with bladder and bowel involvement for 3 years. On examination, tone was increased in all four limbs with 4/5 power in bilateral upper limbs and 2/5 power in both lower limbs, along with exaggerated deep tendon reflexes in all four limbs. Superficial abdominal reflexes were absent and, bilateral planters were extensor. He had 70–80% sensory loss below T4 level. His routine laboratory studies including hematological and biochemical parameters were within normal limits.\nComputerised tomography scan of the cervical spine revealed a large osseo-destructive lesion involving body of C5and C6 vertebra which was causing spinal canal compromise [Figure and ]. The lesion was involving surrounding structures with extension into the submuscular plane anteriorly. On magnetic resonance imaging (MRI) evaluation, the mass was isointense on T1 and T2 weighted sequences with minimal contrast enhancement. It was causing distrucation of C5 and C6 vertebra body with compression of thecal sac [Figure –].\nUsing anterior cervical approach, the tumor resection was performed. Intra-operatively, the tumor involved whole of the C5 and C6 vertebral body. There was a soft tissue component of the tumor, which was extending into the submuscular plane. Inside the capsule, the tumor consisted of cheesy material that was minimally vascular. Total tumor excision was done with tricorticate iliac crest bone grafting and plate and screw fixation between C4 and C7 vertebral bodies.\nMicroscopic examination showed spindle cells disposed of in intersecting fascicles intermingled with mononuclear xanthomatous cells displaying round nuclei with dense chromatin inconspicuous nucleoli and pale eosinophilic finely vacuolated chromatin. Spindle cells display bland appearing nuclear chromatin and eosinophilic cytoplasm. Occasional cells displayed nuclear atypia and enlargement. Scattered neutrophils were seen admixed with these cells. Focal areas of calcification and occasional osteoclasts like giant cells were also seen. Interspersed areas showed degenerated bony trabeculae and cartilage [Figure and ].\nPostoperatively, patient had significant improvement in neck pain and spasticity in all four limbs. At follow-up of 6 months, he was doing well. | 0 | Write a detailed clinical case vignette based on the following key phrases: Histiocytic lesions, Spinal cord compression, Xanthoma/xanthogranuloma |
A 58-year-old man presented to us three years ago with a history of progressive anterior neck swelling and hoarseness of voice for the past one month. On clinical examination, there was palpable left anterior neck swelling with cervical lymphadenopathy. A core needle biopsy of the lesion revealed invasive poorly differentiated carcinoma. He defaulted our follow-up and opted for a left hemithyroidectomy at another institution. The histopathological report was intrathyroid thymic carcinoma. He refused adjuvant radiotherapy despite being counselled and subsequently defaulted follow-up.\nRecently, the patient presented to us again with progressive hoarseness of voice and intermittent haemoptysis. He had shortness of breath, especially when lying flat, but no difficulty in swallowing. On clinical examination, there was a left supraclavicular mass measuring 2 × 2 cm. The mass was immobile and hard in consistency. The anterior aspect of the neck showed an irregular hard mass measuring 3 × 4 cm. An indirect laryngoscopy examination revealed a left vocal cord palsy, likely from his previous presentation. An intraluminal mass was seen over the posterior tracheal wall, occupying a third of the tracheal lumen. We proceeded with a contrasted computed tomography (CECT) of the neck and thorax, which revealed a lobulated hypodense soft tissue mass measuring 4.3 × 3.9 × 5.2 cm. It occupied the left thyroid bed from the C7/T1 to the T2/T3 vertebral level, with left retrosternal extension and tracheal deviation to the right. The soft tissue mass had displaced the left common carotid artery and internal jugular vein laterally. As the CECT showed (, ), the soft tissue mass had caused more than 80% of tracheal luminal narrowing. At retrosternal, multiple matted lymph nodes were seen.\nImage-guided fine needle aspiration cytology (FNAC) of the soft tissue mass at the left thyroid bed showed malignant cells suggestive of intrathyroid thymic carcinoma (CASTLE). After a multidisciplinary meeting, we planned for curative resection and tracheostomy. Intraoperative tracheoscopic examination showed a soft tissue mass measuring 3 cm in length at the posterior tracheal wall, 2.1 cm from the vocal cord (). The mass was debrided to achieve an adequate diameter of tracheal lumen distal to the tracheostomy insertion. The left supraclavicular solid tumour densely adhered to the left great vessels. Due to a high risk of injury to the great vessels, we proceeded with tumour debulking only.\nThe histopathology report showed an unencapsulated tumour that displayed lobular architecture. The tumour lobules were composed of sheets of neoplastic polygonal cells rimmed by a variable amount of mature lymphocytes. The neoplastic cells were mildly pleomorphic, and lymphovascular invasion was present. Skeletal muscle bundles attached at the periphery showed focal tumour cell infiltration (, ). Immunohistochemical studies were immunoreactive for CD5 (membranous pattern) and CD117 but negative for thyroglobulin and TTF1 (, ). The proliferative index, Ki67, was approximately 20%.\nPostoperative care was unremarkable, and no immediate complications were noted. The patient was counselled for adjuvant chemoradiotherapy, but he was not amenable to this course of action. He is currently under regular follow-up with no disease progression six months after the operation. | Patient 3 was a 67-year-old female who presented with hoarseness and dysphagia for a few weeks. CT scans showed a 3.8 cm left thyroid tumor with superior mediastinal and tracheal involvements, i.e., stage pT4bN0M0. Complete resection of the tumor was successfully achieved by total thyroidectomy and central neck dissection. Postsurgical chemotherapy was applied (docetaxel 120 mg/m2 and cisplatin 30 mg/m2), followed by a course of intensity modulated radiotherapy (70 Gy at 2.5 Gy per fraction). She remains well 4 years after diagnosis. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 58-year-old man presented to us three years ago with a history of progressive anterior neck swelling and hoarseness of voice for the past one month. On clinical examination, there was palpable left anterior neck swelling with cervical lymphadenopathy. A core needle biopsy of the lesion revealed invasive poorly differentiated carcinoma. He defaulted our follow-up and opted for a left hemithyroidectomy at another institution. The histopathological report was intrathyroid thymic carcinoma. He refused adjuvant radiotherapy despite being counselled and subsequently defaulted follow-up.\nRecently, the patient presented to us again with progressive hoarseness of voice and intermittent haemoptysis. He had shortness of breath, especially when lying flat, but no difficulty in swallowing. On clinical examination, there was a left supraclavicular mass measuring 2 × 2 cm. The mass was immobile and hard in consistency. The anterior aspect of the neck showed an irregular hard mass measuring 3 × 4 cm. An indirect laryngoscopy examination revealed a left vocal cord palsy, likely from his previous presentation. An intraluminal mass was seen over the posterior tracheal wall, occupying a third of the tracheal lumen. We proceeded with a contrasted computed tomography (CECT) of the neck and thorax, which revealed a lobulated hypodense soft tissue mass measuring 4.3 × 3.9 × 5.2 cm. It occupied the left thyroid bed from the C7/T1 to the T2/T3 vertebral level, with left retrosternal extension and tracheal deviation to the right. The soft tissue mass had displaced the left common carotid artery and internal jugular vein laterally. As the CECT showed (, ), the soft tissue mass had caused more than 80% of tracheal luminal narrowing. At retrosternal, multiple matted lymph nodes were seen.\nImage-guided fine needle aspiration cytology (FNAC) of the soft tissue mass at the left thyroid bed showed malignant cells suggestive of intrathyroid thymic carcinoma (CASTLE). After a multidisciplinary meeting, we planned for curative resection and tracheostomy. Intraoperative tracheoscopic examination showed a soft tissue mass measuring 3 cm in length at the posterior tracheal wall, 2.1 cm from the vocal cord (). The mass was debrided to achieve an adequate diameter of tracheal lumen distal to the tracheostomy insertion. The left supraclavicular solid tumour densely adhered to the left great vessels. Due to a high risk of injury to the great vessels, we proceeded with tumour debulking only.\nThe histopathology report showed an unencapsulated tumour that displayed lobular architecture. The tumour lobules were composed of sheets of neoplastic polygonal cells rimmed by a variable amount of mature lymphocytes. The neoplastic cells were mildly pleomorphic, and lymphovascular invasion was present. Skeletal muscle bundles attached at the periphery showed focal tumour cell infiltration (, ). Immunohistochemical studies were immunoreactive for CD5 (membranous pattern) and CD117 but negative for thyroglobulin and TTF1 (, ). The proliferative index, Ki67, was approximately 20%.\nPostoperative care was unremarkable, and no immediate complications were noted. The patient was counselled for adjuvant chemoradiotherapy, but he was not amenable to this course of action. He is currently under regular follow-up with no disease progression six months after the operation. | Patient 1 was a 61-year-old female who presented with a 5-cm anterior neck mass with retrosternal extension. She was surgically treated with a right thyroid lobectomy at another institution. The lesion was interpreted to be a poorly differentiated papillary thyroid cancer. Postoperative treatment consisted of sequential and combined chemoradiotherapy. Local recurrence in the region of the right thyroid occurred 20 months later, and radiotherapy was performed to relieve symptoms. Six years later, the patient had local recurrence again and was referred to our institution for further treatment. After curative wide local excision and central neck dissection, postoperative radiotherapy was given to the thyroid bed and bilateral cervical lymph node areas (55 Gy/16 fractions). Histological re-evaluation of the recurrent tumor specimens revealed CASTLE (pT4bN1M0). A third local recurrence was diagnosed 1 year later, and concurrent chemotherapy (paclitaxel 210 mg/m2 and cisplatin 40 mg/m2) and radiotherapy (50.4 Gy /28 fractions) were applied. The patient died from acute respiratory distress 1 month after the initiation of treatment. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 58-year-old man presented to us three years ago with a history of progressive anterior neck swelling and hoarseness of voice for the past one month. On clinical examination, there was palpable left anterior neck swelling with cervical lymphadenopathy. A core needle biopsy of the lesion revealed invasive poorly differentiated carcinoma. He defaulted our follow-up and opted for a left hemithyroidectomy at another institution. The histopathological report was intrathyroid thymic carcinoma. He refused adjuvant radiotherapy despite being counselled and subsequently defaulted follow-up.\nRecently, the patient presented to us again with progressive hoarseness of voice and intermittent haemoptysis. He had shortness of breath, especially when lying flat, but no difficulty in swallowing. On clinical examination, there was a left supraclavicular mass measuring 2 × 2 cm. The mass was immobile and hard in consistency. The anterior aspect of the neck showed an irregular hard mass measuring 3 × 4 cm. An indirect laryngoscopy examination revealed a left vocal cord palsy, likely from his previous presentation. An intraluminal mass was seen over the posterior tracheal wall, occupying a third of the tracheal lumen. We proceeded with a contrasted computed tomography (CECT) of the neck and thorax, which revealed a lobulated hypodense soft tissue mass measuring 4.3 × 3.9 × 5.2 cm. It occupied the left thyroid bed from the C7/T1 to the T2/T3 vertebral level, with left retrosternal extension and tracheal deviation to the right. The soft tissue mass had displaced the left common carotid artery and internal jugular vein laterally. As the CECT showed (, ), the soft tissue mass had caused more than 80% of tracheal luminal narrowing. At retrosternal, multiple matted lymph nodes were seen.\nImage-guided fine needle aspiration cytology (FNAC) of the soft tissue mass at the left thyroid bed showed malignant cells suggestive of intrathyroid thymic carcinoma (CASTLE). After a multidisciplinary meeting, we planned for curative resection and tracheostomy. Intraoperative tracheoscopic examination showed a soft tissue mass measuring 3 cm in length at the posterior tracheal wall, 2.1 cm from the vocal cord (). The mass was debrided to achieve an adequate diameter of tracheal lumen distal to the tracheostomy insertion. The left supraclavicular solid tumour densely adhered to the left great vessels. Due to a high risk of injury to the great vessels, we proceeded with tumour debulking only.\nThe histopathology report showed an unencapsulated tumour that displayed lobular architecture. The tumour lobules were composed of sheets of neoplastic polygonal cells rimmed by a variable amount of mature lymphocytes. The neoplastic cells were mildly pleomorphic, and lymphovascular invasion was present. Skeletal muscle bundles attached at the periphery showed focal tumour cell infiltration (, ). Immunohistochemical studies were immunoreactive for CD5 (membranous pattern) and CD117 but negative for thyroglobulin and TTF1 (, ). The proliferative index, Ki67, was approximately 20%.\nPostoperative care was unremarkable, and no immediate complications were noted. The patient was counselled for adjuvant chemoradiotherapy, but he was not amenable to this course of action. He is currently under regular follow-up with no disease progression six months after the operation. | A 60-year-old gentleman with past medical history of cervical disk herniation presented with a 3-year history of progressive fatigue and bilateral upper extremity weakness. MRI was performed to evaluate the spine and was consistent with cervical spinal stenosis. T2 non-contrast images incidentally revealed an infiltrating mass in the left thyroid that was incompletely visualized. Ultrasound-guided fine needle aspiration showed atypia of undetermined significance. The patient was referred to surgery and was noted to have dysphonia but no stridor or dysphagia. The differential diagnosis was felt to include papillary thyroid carcinoma, follicular thyroid carcinoma, rare thyroid neoplasms including Hurthle cell carcinoma or anaplastic carcinoma, abscess, or autoimmune thyroid disease. He underwent total thyroidectomy with central neck dissection. On exploration, the thyroid mass was seen encasing the left recurrent laryngeal nerve and was shaved off the nerve and the trachea. The nerve was not stimulatable after resection and the patient developed left vocal cord paralysis. The tumor grossly measured 7 cm with extrathyroidal extension. There was lymphvascular space invasion and perineural invasion. Three lymph nodes were identified, and all were negative. Surgical margins had extensive involvement of tumor. Histopathologic analysis of the specimen showed high-grade carcinoma with thymus-like differentiation. Immunohistochemical staining was positive for pan-CK, p63 (cyokeratin and epithelial markers) and negative for chromogranin, thyroglobulin, and calcitonin (neuroendocrine, papillary and follicular thyroid, and medullary makers, respectively).\nThe tumor was arranged in broad, pushing, smooth-bordered islands abutted against a lymphocyte rich stroma. The lobules of tumor were surrounded by lymphocytes and plasma cells (Figures and ). The tumor cells were squamoid and syncytial to spindled, with eosinophilic cytoplasm, and the nuclei show mild pleomorphism with pale vesicular chromatin. The neoplastic cells were strongly immunoreactive for pancytokeratin and p63, as shown in Figures and . These cells were also strongly positive for CD5, which marks background T lymphocytes (Figure ). The neoplastic cells were non-reactive with TTF1 and thyroglobulin.\nPostoperative staging surveillance with PET scan was performed for systemic staging and due to positive margins. It showed no abnormal FDG uptake at distant sites and increased uptake at the operative bed, indeterminate between residual disease and postoperative changes. The patient underwent adjuvant treatment with radiation therapy to 64 Gy in 32 fractions with intensity modulated radiotherapy. Concurrent chemotherapy was given based on his extensive positive margins for radiosensitization. A dose of 60 Gy was delivered to the bilateral neck including paratracheal nodes into the upper mediastinum, with a 4 Gy sequential boost to the operative site. A coronal section of the radiotherapy plan is shown in Figure . Six cycles of intravenous concurrent cisplatin 35 mg/m2 were infused. During therapy, he developed grade 2 skin reaction characterized by dry desquamation and moderate erythema of the neck and upper chest. Skin moisturizer was used for therapy. He experienced grade 2 esophagitis and mucositis of the oropharynx and grade 2 nausea requiring topical analgesics, oral narcotics, antiemetics, and intravenous fluid administration. Weight decreased from 108 to 95.5 kg by the end of therapy. Nutritional counseling by dietician and oral nutritional supplementation were provided. With chemotherapy, the patient developed a tinnitus grade 1. Toxicities are reported per the Common Terminology Criteria for Adverse Events ().\nAfter treatment, the patient initially experienced continued weight loss, and as a result underwent percutaneous endoscopic gastrostomy tube placement. Five months after end of treatment, the tube was removed after the patient was able to gain significant weight and completely obtain nutrition per oral route for 6 weeks. Dysphagia to solid foods is improving. He experienced persistent dysphonia secondary to left vocal cord paralysis since surgery and underwent left medialization thyroplasty 1 year later. He displayed improvement in voice quality. At 3-years follow-up after completion of radiotherapy, our patient is without evidence of locally recurrent or distant disease. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 58-year-old man presented to us three years ago with a history of progressive anterior neck swelling and hoarseness of voice for the past one month. On clinical examination, there was palpable left anterior neck swelling with cervical lymphadenopathy. A core needle biopsy of the lesion revealed invasive poorly differentiated carcinoma. He defaulted our follow-up and opted for a left hemithyroidectomy at another institution. The histopathological report was intrathyroid thymic carcinoma. He refused adjuvant radiotherapy despite being counselled and subsequently defaulted follow-up.\nRecently, the patient presented to us again with progressive hoarseness of voice and intermittent haemoptysis. He had shortness of breath, especially when lying flat, but no difficulty in swallowing. On clinical examination, there was a left supraclavicular mass measuring 2 × 2 cm. The mass was immobile and hard in consistency. The anterior aspect of the neck showed an irregular hard mass measuring 3 × 4 cm. An indirect laryngoscopy examination revealed a left vocal cord palsy, likely from his previous presentation. An intraluminal mass was seen over the posterior tracheal wall, occupying a third of the tracheal lumen. We proceeded with a contrasted computed tomography (CECT) of the neck and thorax, which revealed a lobulated hypodense soft tissue mass measuring 4.3 × 3.9 × 5.2 cm. It occupied the left thyroid bed from the C7/T1 to the T2/T3 vertebral level, with left retrosternal extension and tracheal deviation to the right. The soft tissue mass had displaced the left common carotid artery and internal jugular vein laterally. As the CECT showed (, ), the soft tissue mass had caused more than 80% of tracheal luminal narrowing. At retrosternal, multiple matted lymph nodes were seen.\nImage-guided fine needle aspiration cytology (FNAC) of the soft tissue mass at the left thyroid bed showed malignant cells suggestive of intrathyroid thymic carcinoma (CASTLE). After a multidisciplinary meeting, we planned for curative resection and tracheostomy. Intraoperative tracheoscopic examination showed a soft tissue mass measuring 3 cm in length at the posterior tracheal wall, 2.1 cm from the vocal cord (). The mass was debrided to achieve an adequate diameter of tracheal lumen distal to the tracheostomy insertion. The left supraclavicular solid tumour densely adhered to the left great vessels. Due to a high risk of injury to the great vessels, we proceeded with tumour debulking only.\nThe histopathology report showed an unencapsulated tumour that displayed lobular architecture. The tumour lobules were composed of sheets of neoplastic polygonal cells rimmed by a variable amount of mature lymphocytes. The neoplastic cells were mildly pleomorphic, and lymphovascular invasion was present. Skeletal muscle bundles attached at the periphery showed focal tumour cell infiltration (, ). Immunohistochemical studies were immunoreactive for CD5 (membranous pattern) and CD117 but negative for thyroglobulin and TTF1 (, ). The proliferative index, Ki67, was approximately 20%.\nPostoperative care was unremarkable, and no immediate complications were noted. The patient was counselled for adjuvant chemoradiotherapy, but he was not amenable to this course of action. He is currently under regular follow-up with no disease progression six months after the operation. | Patient 2 was a 48-year-old male pathologically diagnosed with stage pT4aN0M0 CASTLE postsurgery. He presented with rapidly growing thyroid nodules with fixation to underlying structures. He underwent total thyroidectomy with central neck dissection. On exploration, the thyroid mass was seen extended to the left laryngeal nerve, strap muscle, trachea, and esophagus. Curative wide local excision was attempted on patients with gross residual local disease. Postoperative external beam radiation therapy (50.4 Gy/28 fractions) was given to the thyroid bed and bilateral cervical lymph node area. He remained well 2 years post therapy with no palpable neck disease. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 58-year-old man presented to us three years ago with a history of progressive anterior neck swelling and hoarseness of voice for the past one month. On clinical examination, there was palpable left anterior neck swelling with cervical lymphadenopathy. A core needle biopsy of the lesion revealed invasive poorly differentiated carcinoma. He defaulted our follow-up and opted for a left hemithyroidectomy at another institution. The histopathological report was intrathyroid thymic carcinoma. He refused adjuvant radiotherapy despite being counselled and subsequently defaulted follow-up.\nRecently, the patient presented to us again with progressive hoarseness of voice and intermittent haemoptysis. He had shortness of breath, especially when lying flat, but no difficulty in swallowing. On clinical examination, there was a left supraclavicular mass measuring 2 × 2 cm. The mass was immobile and hard in consistency. The anterior aspect of the neck showed an irregular hard mass measuring 3 × 4 cm. An indirect laryngoscopy examination revealed a left vocal cord palsy, likely from his previous presentation. An intraluminal mass was seen over the posterior tracheal wall, occupying a third of the tracheal lumen. We proceeded with a contrasted computed tomography (CECT) of the neck and thorax, which revealed a lobulated hypodense soft tissue mass measuring 4.3 × 3.9 × 5.2 cm. It occupied the left thyroid bed from the C7/T1 to the T2/T3 vertebral level, with left retrosternal extension and tracheal deviation to the right. The soft tissue mass had displaced the left common carotid artery and internal jugular vein laterally. As the CECT showed (, ), the soft tissue mass had caused more than 80% of tracheal luminal narrowing. At retrosternal, multiple matted lymph nodes were seen.\nImage-guided fine needle aspiration cytology (FNAC) of the soft tissue mass at the left thyroid bed showed malignant cells suggestive of intrathyroid thymic carcinoma (CASTLE). After a multidisciplinary meeting, we planned for curative resection and tracheostomy. Intraoperative tracheoscopic examination showed a soft tissue mass measuring 3 cm in length at the posterior tracheal wall, 2.1 cm from the vocal cord (). The mass was debrided to achieve an adequate diameter of tracheal lumen distal to the tracheostomy insertion. The left supraclavicular solid tumour densely adhered to the left great vessels. Due to a high risk of injury to the great vessels, we proceeded with tumour debulking only.\nThe histopathology report showed an unencapsulated tumour that displayed lobular architecture. The tumour lobules were composed of sheets of neoplastic polygonal cells rimmed by a variable amount of mature lymphocytes. The neoplastic cells were mildly pleomorphic, and lymphovascular invasion was present. Skeletal muscle bundles attached at the periphery showed focal tumour cell infiltration (, ). Immunohistochemical studies were immunoreactive for CD5 (membranous pattern) and CD117 but negative for thyroglobulin and TTF1 (, ). The proliferative index, Ki67, was approximately 20%.\nPostoperative care was unremarkable, and no immediate complications were noted. The patient was counselled for adjuvant chemoradiotherapy, but he was not amenable to this course of action. He is currently under regular follow-up with no disease progression six months after the operation. | A 34-year-old woman presented with a 2-month history of sore throat. The patient had no notable medical or family history. Physical examination revealed a palpable right thyroid mass. Thyroid function and calcium tests were within normal limits. Ultrasound showed a 1.7-cm, well demarcated, heterogeneous, low echoic solid mass in the lower pole of the right thyroid (). A computed tomography scan of the neck revealed a 3.3-cm, exophytic, low-attenuated mass in the lower pole of the right thyroid. The FNA biopsy of the mass was performed, which was accompanied by the standard Papanicolaou stain.\nThe smear was composed of not only cohesive three dimensional clusters and sheets but also singly scattered cells (). The tumor cells showed mild anisonucleosis and high nuclear : cytoplasmic (N/C) ratios. Nuclei were round to ovoid with irregular nuclear contours and small, distinct nucleoli (). The nuclear chromatin was vesicular or coarsely granular. Nuclear grooves were rarely seen. Cytoplasm was scanty and amphophilic. Cytoplasmic border was indistinct. Some tumor cells showed dense orangeophilic cytoplasm, which is suggestive of individual cell keratinization (). Some lymphocytes were found on the background and within clusters, which was accompanied by the presence of a few stripped nuclei (). Mitotic figures were rarely seen. Intranuclear cytoplasmic inclusions were not observed. Papillary or follicular structure was not observed. The aspirate was interpreted as a high-grade malignant thyroid neoplasm without further definitive classification. The total thyroidectomy was performed.\nOn gross examination, the thyroid contained a 4.0-cm solid mass replacing most of the right thyroid. The mass had a lobular, tan-colored cut surface and a firm consistency (). On microscopic examination, the tumor was well-circumscribed and it was composed of variably sized and irregularly shaped lobules of cohesive polygonal tumor cells which were separated by bands of dense fibrous stroma (). The tumor cells had high N/C ratios, eosinophilic cytoplasm and ill-defined cell borders. The nuclei were vesicular or coarsely granular with prominent nucleoli. The mitotic figures were sparse. There was mild lymphoplasmacytic infiltration within the tumor nodules and septae. Cytoplasmic keratinization was seen. The tumor cells showed positivity for CD5, carcinoembryonic antigen, high molecular weight keratin (HMWK), cytokeratin 5 (CK5), and p63. The tumor was negative for thyroid transcription factor-1, thyroglobulin, and calcitonin. Vascular invasion and extensive extra-thyroidal extension were present. One regional lymph node was found and involved by tumor.\nOver a 27-month follow-up period, the patient had no evidence of tumor recurrence. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 58-year-old man presented to us three years ago with a history of progressive anterior neck swelling and hoarseness of voice for the past one month. On clinical examination, there was palpable left anterior neck swelling with cervical lymphadenopathy. A core needle biopsy of the lesion revealed invasive poorly differentiated carcinoma. He defaulted our follow-up and opted for a left hemithyroidectomy at another institution. The histopathological report was intrathyroid thymic carcinoma. He refused adjuvant radiotherapy despite being counselled and subsequently defaulted follow-up.\nRecently, the patient presented to us again with progressive hoarseness of voice and intermittent haemoptysis. He had shortness of breath, especially when lying flat, but no difficulty in swallowing. On clinical examination, there was a left supraclavicular mass measuring 2 × 2 cm. The mass was immobile and hard in consistency. The anterior aspect of the neck showed an irregular hard mass measuring 3 × 4 cm. An indirect laryngoscopy examination revealed a left vocal cord palsy, likely from his previous presentation. An intraluminal mass was seen over the posterior tracheal wall, occupying a third of the tracheal lumen. We proceeded with a contrasted computed tomography (CECT) of the neck and thorax, which revealed a lobulated hypodense soft tissue mass measuring 4.3 × 3.9 × 5.2 cm. It occupied the left thyroid bed from the C7/T1 to the T2/T3 vertebral level, with left retrosternal extension and tracheal deviation to the right. The soft tissue mass had displaced the left common carotid artery and internal jugular vein laterally. As the CECT showed (, ), the soft tissue mass had caused more than 80% of tracheal luminal narrowing. At retrosternal, multiple matted lymph nodes were seen.\nImage-guided fine needle aspiration cytology (FNAC) of the soft tissue mass at the left thyroid bed showed malignant cells suggestive of intrathyroid thymic carcinoma (CASTLE). After a multidisciplinary meeting, we planned for curative resection and tracheostomy. Intraoperative tracheoscopic examination showed a soft tissue mass measuring 3 cm in length at the posterior tracheal wall, 2.1 cm from the vocal cord (). The mass was debrided to achieve an adequate diameter of tracheal lumen distal to the tracheostomy insertion. The left supraclavicular solid tumour densely adhered to the left great vessels. Due to a high risk of injury to the great vessels, we proceeded with tumour debulking only.\nThe histopathology report showed an unencapsulated tumour that displayed lobular architecture. The tumour lobules were composed of sheets of neoplastic polygonal cells rimmed by a variable amount of mature lymphocytes. The neoplastic cells were mildly pleomorphic, and lymphovascular invasion was present. Skeletal muscle bundles attached at the periphery showed focal tumour cell infiltration (, ). Immunohistochemical studies were immunoreactive for CD5 (membranous pattern) and CD117 but negative for thyroglobulin and TTF1 (, ). The proliferative index, Ki67, was approximately 20%.\nPostoperative care was unremarkable, and no immediate complications were noted. The patient was counselled for adjuvant chemoradiotherapy, but he was not amenable to this course of action. He is currently under regular follow-up with no disease progression six months after the operation. | A 79-year old woman was hospitalized on April 25, 2018, at which time she had progressive dyspnea over the past 5 weeks, productive cough, poor appetite, and weight loss of 5 kg over the last 6 months currently weighing 81 kg. Her medical history included an invasive ductal breast cancer pT1cN0M0 G2 ER 90%, PR 20% 20 years ago treated with lumpectomy, radiotherapy, and tamoxifen until 2004, and an undifferentiated cancer of the right parotid pT1 (17 mm) pN2 (1/2 positive in level I, 1/11 positive in level II, no extranodal extensions) pM0 diagnosed 2008 and treated with a total parotidectomy achieving R0 margins and a selective neck dissection levels II-IV followed by radiotherapy.\nOn physical examination, the patient was in mild respiratory distress. The pulmonary exam revealed dullness to percussion, decreased fremitus, and decreased breathing sounds in up to two-thirds of the left lung field. Oxygen saturation was 92% with the patient breathing ambient air, and arterial blood gases showed partial respiratory insufficiency. A chest X-ray confirmed a left-sided pleural effusion, and thoracentesis removed 1.5 liters of turbid fluid. Biochemical analysis of the fluid established an exudative etiology. Atypical cells with immunocytochemical positivity for p40, cytokeratin 5, and p63 in the cytopathology specimen were primarily suggestive of a non-small cell lung cancer, and a CT scan of the thorax showed a mass (4.2 × 2.2 × 2.8 cm) in the right upper lobe and extensive cardiophrenic, mediastinal, and hilar lymphadenopathy as well as enlarged lymph nodes along the left arteria mammaria interna. An FDG PET/CT scan confirmed these findings, and the scan revealed a hypermetabolic activity in mesenteric lymph nodes and at the left pleura (). Thoracoscopy and biopsy of the parietal pleura were performed. Surprisingly, histopathological examination revealed a non-small cell carcinoma () expressing CD5 (), CD117 (), and p63 all indicating a carcinoma of thymic origin (). With no signs of a thymic pathology, the histopathological diagnosis of CASTLE was established, and as additional immunostainings for CD5 () and CD117 () in the specimen of the parotid cancer diagnosed 2008 were positive also, a rare case of a CASTLE tumor of the parotid healed at the primary and neck sites, but with late metastatic dissemination was diagnosed.\nAs CD117 was highly overexpressed in the tumor, exons 9, 11, 13, 14, and 17 of the C-KIT gene were analyzed after extraction of genomic DNA by PCR using specific primers, but no common mutation was found consistent with previous reports (, ). Anticancer immunity and potential response to immunotherapy were assessed by PD-L1 staining of the pleural tumor, which showed a high expression level of 60% ().\nThe patient underwent talc pleurodesis and left partial pleurectomy for recurrent pleural effusions. Systemically, disseminated metastatic disease was treated with the monoclonal PD-L1 inhibitor pembrolizumab at a dose 200 mg every 3 weeks. Four months after the start of immunotherapy a follow-up FDG PET/CT study showed a partial remission with a moderate morphologic reduction of the tumor load, but a markedly reduced metabolic activity at all tumor sites (). Pembrolizumab treatment continued at a dose of 200 mg IV every 3 weeks. The patient showed a mild skin rash treated with low dose prednisolone (20 mg with tapering) for 2 weeks. No other adverse events associated with pembrolizumab were observed, and the therapy was tolerated well. At the last follow-up, the patient was in good clinical condition and had gained 3 kg of weight. On March 28th, 2019, a second follow-up FDG PET/CT showed no residual pleural effusion (not shown) and a further regression of lesions in the right lung and lymph nodes. Residual focal FDG-Uptake was seen in the left pleura (). | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 58-year-old man presented to us three years ago with a history of progressive anterior neck swelling and hoarseness of voice for the past one month. On clinical examination, there was palpable left anterior neck swelling with cervical lymphadenopathy. A core needle biopsy of the lesion revealed invasive poorly differentiated carcinoma. He defaulted our follow-up and opted for a left hemithyroidectomy at another institution. The histopathological report was intrathyroid thymic carcinoma. He refused adjuvant radiotherapy despite being counselled and subsequently defaulted follow-up.\nRecently, the patient presented to us again with progressive hoarseness of voice and intermittent haemoptysis. He had shortness of breath, especially when lying flat, but no difficulty in swallowing. On clinical examination, there was a left supraclavicular mass measuring 2 × 2 cm. The mass was immobile and hard in consistency. The anterior aspect of the neck showed an irregular hard mass measuring 3 × 4 cm. An indirect laryngoscopy examination revealed a left vocal cord palsy, likely from his previous presentation. An intraluminal mass was seen over the posterior tracheal wall, occupying a third of the tracheal lumen. We proceeded with a contrasted computed tomography (CECT) of the neck and thorax, which revealed a lobulated hypodense soft tissue mass measuring 4.3 × 3.9 × 5.2 cm. It occupied the left thyroid bed from the C7/T1 to the T2/T3 vertebral level, with left retrosternal extension and tracheal deviation to the right. The soft tissue mass had displaced the left common carotid artery and internal jugular vein laterally. As the CECT showed (, ), the soft tissue mass had caused more than 80% of tracheal luminal narrowing. At retrosternal, multiple matted lymph nodes were seen.\nImage-guided fine needle aspiration cytology (FNAC) of the soft tissue mass at the left thyroid bed showed malignant cells suggestive of intrathyroid thymic carcinoma (CASTLE). After a multidisciplinary meeting, we planned for curative resection and tracheostomy. Intraoperative tracheoscopic examination showed a soft tissue mass measuring 3 cm in length at the posterior tracheal wall, 2.1 cm from the vocal cord (). The mass was debrided to achieve an adequate diameter of tracheal lumen distal to the tracheostomy insertion. The left supraclavicular solid tumour densely adhered to the left great vessels. Due to a high risk of injury to the great vessels, we proceeded with tumour debulking only.\nThe histopathology report showed an unencapsulated tumour that displayed lobular architecture. The tumour lobules were composed of sheets of neoplastic polygonal cells rimmed by a variable amount of mature lymphocytes. The neoplastic cells were mildly pleomorphic, and lymphovascular invasion was present. Skeletal muscle bundles attached at the periphery showed focal tumour cell infiltration (, ). Immunohistochemical studies were immunoreactive for CD5 (membranous pattern) and CD117 but negative for thyroglobulin and TTF1 (, ). The proliferative index, Ki67, was approximately 20%.\nPostoperative care was unremarkable, and no immediate complications were noted. The patient was counselled for adjuvant chemoradiotherapy, but he was not amenable to this course of action. He is currently under regular follow-up with no disease progression six months after the operation. | A 52-year-old woman was referred to our hospital because of severe dyspnea and the presence of a neck tumor. She had experienced dyspnea for the past 6 months and had recently noticed bloody sputum. A computed tomography examination of the neck revealed a thyroid tumor about 8 cm in size. The tumor had invaded the trachea, obstructing 90% of the tracheal lumen (fig. ). An emergency tracheotomy was performed to maintain an airway through the tumor tissue. On biopsy, the tumor was diagnosed as CASTLE, suggesting a favorable prognosis if a complete resection could be performed. Magnetic resonance imaging and an esophageal fibroscopy examination revealed a low possibility of esophageal invasion (fig. ). Under general anesthesia, a thyroidectomy was initially performed via a neck incision. The invaded portion of the trachea was sharply dissected. A partial sternotomy was performed at the level of the 2nd intercostal space to identify the distal portion of the intact trachea and to mobilize the inferior tracheal segment. The trachea was transversely opened to find the intact portion. The length of the resected trachea was 6 cm (fig. ). Bilateral recurrent nerves were macroscopically preserved. We performed a suprahyoid release to mobilize the superior tracheal segment, but these mobilizations were insufficient to perform an anastomosis. Thus, to allow additional mobilization, we performed a clamshell thoracotomy and incised the pericardium 360° around the hilum, dissecting the pulmonary ligament bilaterally. These procedures allowed an anastomosis. An end-to-end bronchial anastomosis was carried out using running 4-0 PDS II sutures (Ethicon, Somerville, N.J., USA) for the membranous portion and interrupted 4-0 PDS II sutures for the cartilaginous portion. The bronchial anastomosis was wrapped with an omental pedicle flap that was lifted up through a retrosternal route via a laparotomy. A gastrostomy was also performed for postoperative enteral nutrition. For management after surgery, a chin suture was placed. Neck mobilization was restricted for the placement of the chin suture and airway fixation. After confirming an improvement in the laryngeal edema using a laryngoscopy, extubation was performed on postoperative day (POD) 17. The patient exhibited swallowing disturbances and underwent swallowing rehabilitation. Enteral nutrition was provided through the gastrostomy. The patient was discharged on POD 31, and per os feeding was started on POD 51. Radiotherapy (60 Gy in 40 daily fractions of 1.5 Gy) was performed as an adjuvant therapy. Throughout the clinical course, no major complications, including swallowing disturbances, hoarseness or anastomotic problems, were observed. A histopathological examination of the resected specimen revealed the characteristic morphology of CASTLE (fig. ); immunohistochemistry was positive for CD5 (fig. ) but negative for thyroid transcription factor 1 (TTF-1; fig. ).\nEleven months after surgery, lower back pain and right leg numbness developed and led to gait inability. Multiple lung and bone recurrences were observed, but no local recurrence. In order to ease the back pain, palliative radiotherapy (35 Gy in 14 fractions) to the bone metastasis was administered. Twelve months after surgery, drainage of pleural effusion was performed. She died of pleural metastasis 14 months after the initial diagnosis of CASTLE. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 58-year-old man presented to us three years ago with a history of progressive anterior neck swelling and hoarseness of voice for the past one month. On clinical examination, there was palpable left anterior neck swelling with cervical lymphadenopathy. A core needle biopsy of the lesion revealed invasive poorly differentiated carcinoma. He defaulted our follow-up and opted for a left hemithyroidectomy at another institution. The histopathological report was intrathyroid thymic carcinoma. He refused adjuvant radiotherapy despite being counselled and subsequently defaulted follow-up.\nRecently, the patient presented to us again with progressive hoarseness of voice and intermittent haemoptysis. He had shortness of breath, especially when lying flat, but no difficulty in swallowing. On clinical examination, there was a left supraclavicular mass measuring 2 × 2 cm. The mass was immobile and hard in consistency. The anterior aspect of the neck showed an irregular hard mass measuring 3 × 4 cm. An indirect laryngoscopy examination revealed a left vocal cord palsy, likely from his previous presentation. An intraluminal mass was seen over the posterior tracheal wall, occupying a third of the tracheal lumen. We proceeded with a contrasted computed tomography (CECT) of the neck and thorax, which revealed a lobulated hypodense soft tissue mass measuring 4.3 × 3.9 × 5.2 cm. It occupied the left thyroid bed from the C7/T1 to the T2/T3 vertebral level, with left retrosternal extension and tracheal deviation to the right. The soft tissue mass had displaced the left common carotid artery and internal jugular vein laterally. As the CECT showed (, ), the soft tissue mass had caused more than 80% of tracheal luminal narrowing. At retrosternal, multiple matted lymph nodes were seen.\nImage-guided fine needle aspiration cytology (FNAC) of the soft tissue mass at the left thyroid bed showed malignant cells suggestive of intrathyroid thymic carcinoma (CASTLE). After a multidisciplinary meeting, we planned for curative resection and tracheostomy. Intraoperative tracheoscopic examination showed a soft tissue mass measuring 3 cm in length at the posterior tracheal wall, 2.1 cm from the vocal cord (). The mass was debrided to achieve an adequate diameter of tracheal lumen distal to the tracheostomy insertion. The left supraclavicular solid tumour densely adhered to the left great vessels. Due to a high risk of injury to the great vessels, we proceeded with tumour debulking only.\nThe histopathology report showed an unencapsulated tumour that displayed lobular architecture. The tumour lobules were composed of sheets of neoplastic polygonal cells rimmed by a variable amount of mature lymphocytes. The neoplastic cells were mildly pleomorphic, and lymphovascular invasion was present. Skeletal muscle bundles attached at the periphery showed focal tumour cell infiltration (, ). Immunohistochemical studies were immunoreactive for CD5 (membranous pattern) and CD117 but negative for thyroglobulin and TTF1 (, ). The proliferative index, Ki67, was approximately 20%.\nPostoperative care was unremarkable, and no immediate complications were noted. The patient was counselled for adjuvant chemoradiotherapy, but he was not amenable to this course of action. He is currently under regular follow-up with no disease progression six months after the operation. | A 76-year-old man was referred to our hospital for investigation of a 20-day history of rapidly deteriorating dyspnea and severe cough. This was associated with dysphagia and cervical pain. The patient was an ex-marble craftsman and an active smoker. His medical history included chronic pulmonary obstructive disease (COPD). A chest X-ray (CXR) showed an imprint at the right side of the trachea. Thyroid ultrasound (US) revealed a large and heterogenous thyroid gland and the presence of a calcified mass, 4.76 × 4.01 cm with high blood perfusion in the right upper lobe of the gland. A contrast-enhanced computed tomography (CT) scan of the neck and thorax reported the corresponding lesion as heterogenous, with irregular borders, extending from the sternoclavicular joints to the upper borders of the thyroid gland. The mass was surrounding the trachea anterolaterally displacing it to the left and the right common artery to the right (). There were no enlarged lymph nodes. Bronchoscopy revealed a fracture of the first tracheal ring but there was no evidence of tumour invasion (). Cytology from bronchial lavage was negative for cancer cells. Laboratory tests were within normal limits with the exception of elevated D-dimers (2,994 μg/L) and elevated white blood cells (19.27 K/μL). Tumour markers were within normal range values.\nOn admission, the patient was intubated, due to acute respiratory failure, and he was admitted to the intensive care unit (ICU). Subsequently, he underwent an emergency procedure and a collar incision was carried out. At surgery, a suspicious looking tumour, which was in continuity with the superior pole of the right thyroid lobe, the sheath of right common carotid artery, and the anterolateral portion of the cartilaginous trachea, was identified. Macroscopically, the tumour looked whitish and scleroelastic with moderately regular borders and was firmly adhered to the aforementioned structures. After the right recurrent laryngeal nerve was identified, the tumour was carefully dissected off the right thyroid lobe and the adventitia of the right common carotid artery. A similar approach was carried out to dissect the tumour off the left lobe; hence, an en bloc tumour resection with total thyroidectomy and a regional lymph node dissection was performed. Rapid frozen section biopsy of the tumour in the right thyroid lobe proved to be malignant with features of squamous cell carcinoma and negative surgical margins.\nPostoperatively, the patient experienced swallowing difficulty and hoarseness and a tracheostomy was performed on day 2.\nFinal pathology examination confirmed frozen section's diagnosis of a squamous cell carcinoma invading the right lobe of the thyroid gland, as well as extracapsular structures of the left lobe. Microscopically, the tumour was described as nonencapsulated, irregularly lobulated, with broad fibrous septa, and lymphoplasmacytic, eosinophilic, histiocytic, and multinuclear infiltration. The neoplastic cells were largely polyhedral or oval with eosinophilic or amphophilic cytoplasm, arranged in islands or nests that showed focal keratinization. The nuclei were vesicular or hyperchromatic with nucleoli. A panel of immunohistochemical stains was performed; the tumour was positive for p63, CD117, CKH, CD5, and CD3 in the lymphocytic part (Figures , , and ). It was also negative for the expression of TTF1.\nThe patient subsequently improved and was discharged home on postoperative day 20. The patient did not receive any additional treatment. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 58-year-old man presented to us three years ago with a history of progressive anterior neck swelling and hoarseness of voice for the past one month. On clinical examination, there was palpable left anterior neck swelling with cervical lymphadenopathy. A core needle biopsy of the lesion revealed invasive poorly differentiated carcinoma. He defaulted our follow-up and opted for a left hemithyroidectomy at another institution. The histopathological report was intrathyroid thymic carcinoma. He refused adjuvant radiotherapy despite being counselled and subsequently defaulted follow-up.\nRecently, the patient presented to us again with progressive hoarseness of voice and intermittent haemoptysis. He had shortness of breath, especially when lying flat, but no difficulty in swallowing. On clinical examination, there was a left supraclavicular mass measuring 2 × 2 cm. The mass was immobile and hard in consistency. The anterior aspect of the neck showed an irregular hard mass measuring 3 × 4 cm. An indirect laryngoscopy examination revealed a left vocal cord palsy, likely from his previous presentation. An intraluminal mass was seen over the posterior tracheal wall, occupying a third of the tracheal lumen. We proceeded with a contrasted computed tomography (CECT) of the neck and thorax, which revealed a lobulated hypodense soft tissue mass measuring 4.3 × 3.9 × 5.2 cm. It occupied the left thyroid bed from the C7/T1 to the T2/T3 vertebral level, with left retrosternal extension and tracheal deviation to the right. The soft tissue mass had displaced the left common carotid artery and internal jugular vein laterally. As the CECT showed (, ), the soft tissue mass had caused more than 80% of tracheal luminal narrowing. At retrosternal, multiple matted lymph nodes were seen.\nImage-guided fine needle aspiration cytology (FNAC) of the soft tissue mass at the left thyroid bed showed malignant cells suggestive of intrathyroid thymic carcinoma (CASTLE). After a multidisciplinary meeting, we planned for curative resection and tracheostomy. Intraoperative tracheoscopic examination showed a soft tissue mass measuring 3 cm in length at the posterior tracheal wall, 2.1 cm from the vocal cord (). The mass was debrided to achieve an adequate diameter of tracheal lumen distal to the tracheostomy insertion. The left supraclavicular solid tumour densely adhered to the left great vessels. Due to a high risk of injury to the great vessels, we proceeded with tumour debulking only.\nThe histopathology report showed an unencapsulated tumour that displayed lobular architecture. The tumour lobules were composed of sheets of neoplastic polygonal cells rimmed by a variable amount of mature lymphocytes. The neoplastic cells were mildly pleomorphic, and lymphovascular invasion was present. Skeletal muscle bundles attached at the periphery showed focal tumour cell infiltration (, ). Immunohistochemical studies were immunoreactive for CD5 (membranous pattern) and CD117 but negative for thyroglobulin and TTF1 (, ). The proliferative index, Ki67, was approximately 20%.\nPostoperative care was unremarkable, and no immediate complications were noted. The patient was counselled for adjuvant chemoradiotherapy, but he was not amenable to this course of action. He is currently under regular follow-up with no disease progression six months after the operation. | The patient, a 28-year-old man with a 3-month-old mass in the neck was referred to our department. The mass, located on the left thyroid lobe, was elastic, hard, showed limited mobility, and measured approximately 30 × 20 mm. There was no remarkable tenderness over the mass.\nBlood examination results were normal, including levels of thyroid-stimulating hormone, free thyroid hormone, and thyroglobulin (Table ). Ultrasonography revealed a hypoechoic solid mass in the lower pole of the left thyroid lobe with a poorly marginated heterogeneous pattern (Fig. ). Computed tomography revealed a heterogenic tumor in the left thyroid lobe (Fig. ). 18F-fluorodeoxyglucose (18F-FDG) accumulated in the thyroid tumor on positron-emission tomography. The standardized uptake value of 18F-FDG was 5.29 (Fig. ). Fine-needle aspiration cytology (FNAC) showed a number of lymphoid cells without atypia that were similar to those seen in malignant lymphoma of the thyroid (Fig. ).\nAs we diagnosed a highly malignant thyroid cancer (cT2N0M0), we performed a left hemithyroidectomy with central neck dissection (ND). Histopathological findings showed that the tumor was composed of irregular insular nests of neoplastic cells and had an expansive growth pattern in the thyroid (Fig. ). The carcinoma cells had round nuclei or polymorphonuclei with well-defined nucleoli and ill-defined cell borders (Fig. ). Immunohistochemical studies showed that the tumor was partially positive for CD5, positive for Ki-67 (approx. 30%), and negative for CD20, calcitonin, and thyroglobulin (Fig. ). Based on these histopathological findings, the tumor was finally diagnosed as CASTLE. There were no signs of recurrence without additional treatment after 10 years of follow-up. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 58-year-old man presented to us three years ago with a history of progressive anterior neck swelling and hoarseness of voice for the past one month. On clinical examination, there was palpable left anterior neck swelling with cervical lymphadenopathy. A core needle biopsy of the lesion revealed invasive poorly differentiated carcinoma. He defaulted our follow-up and opted for a left hemithyroidectomy at another institution. The histopathological report was intrathyroid thymic carcinoma. He refused adjuvant radiotherapy despite being counselled and subsequently defaulted follow-up.\nRecently, the patient presented to us again with progressive hoarseness of voice and intermittent haemoptysis. He had shortness of breath, especially when lying flat, but no difficulty in swallowing. On clinical examination, there was a left supraclavicular mass measuring 2 × 2 cm. The mass was immobile and hard in consistency. The anterior aspect of the neck showed an irregular hard mass measuring 3 × 4 cm. An indirect laryngoscopy examination revealed a left vocal cord palsy, likely from his previous presentation. An intraluminal mass was seen over the posterior tracheal wall, occupying a third of the tracheal lumen. We proceeded with a contrasted computed tomography (CECT) of the neck and thorax, which revealed a lobulated hypodense soft tissue mass measuring 4.3 × 3.9 × 5.2 cm. It occupied the left thyroid bed from the C7/T1 to the T2/T3 vertebral level, with left retrosternal extension and tracheal deviation to the right. The soft tissue mass had displaced the left common carotid artery and internal jugular vein laterally. As the CECT showed (, ), the soft tissue mass had caused more than 80% of tracheal luminal narrowing. At retrosternal, multiple matted lymph nodes were seen.\nImage-guided fine needle aspiration cytology (FNAC) of the soft tissue mass at the left thyroid bed showed malignant cells suggestive of intrathyroid thymic carcinoma (CASTLE). After a multidisciplinary meeting, we planned for curative resection and tracheostomy. Intraoperative tracheoscopic examination showed a soft tissue mass measuring 3 cm in length at the posterior tracheal wall, 2.1 cm from the vocal cord (). The mass was debrided to achieve an adequate diameter of tracheal lumen distal to the tracheostomy insertion. The left supraclavicular solid tumour densely adhered to the left great vessels. Due to a high risk of injury to the great vessels, we proceeded with tumour debulking only.\nThe histopathology report showed an unencapsulated tumour that displayed lobular architecture. The tumour lobules were composed of sheets of neoplastic polygonal cells rimmed by a variable amount of mature lymphocytes. The neoplastic cells were mildly pleomorphic, and lymphovascular invasion was present. Skeletal muscle bundles attached at the periphery showed focal tumour cell infiltration (, ). Immunohistochemical studies were immunoreactive for CD5 (membranous pattern) and CD117 but negative for thyroglobulin and TTF1 (, ). The proliferative index, Ki67, was approximately 20%.\nPostoperative care was unremarkable, and no immediate complications were noted. The patient was counselled for adjuvant chemoradiotherapy, but he was not amenable to this course of action. He is currently under regular follow-up with no disease progression six months after the operation. | A 60-year-old woman, who was diagnosed with cancer of the thyroid gland in 2015, was treated with total thyroidectomy and lymph node dissection. Her postoperative pathology showed carcinoma showing thymus-like differentiation (CASTLE). She was discharged on Levothyroxine 100 mg/day. She did not attend her follow-up appointments. After 2 years, the patient found a lump in the left side of her neck. The tumor grew very slowly and did not cause any symptoms; hence, she did not seek medical advice. In May 2020, about 3 years after the appearance of her left neck tumor, the patient was admitted to our hospital with shortness of breath due to tracheal compression from the tumor. Clinical examination showed enlarged solid painless cervical lymph nodes at levels III and IV, stuck in a block size of 3 × 6 cm in diameter. There was no involvement of the hypopharynx, and her vocal cords were intact. CT scan of her neck revealed a 16 × 54 mm lesion compressing the trachea, and several enlarged left cervical lymph nodes with the size of about 25 mm (Fig. ). The patient was offered a tracheostomy and tracheal stent before undertaking further investigations.\nHer subsequent fluorine-18-labeled fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) scan revealed previous total thyroidectomy, no evidence of recurrence in the surgical bed, enlarged left cervical lymph nodes at levels III and IV, and extranodal extension into sternocleidomastoid muscle measuring 29 × 43x59 mm, with the maximum standardized uptake value (SUV max) of 6.8. Her thymus measured 19 × 31x37mm, with increased metabolism of FDG (SUV max 3.6). There was a 33 × 39x49 mm mass on the left side of the anterior mediastinum, showing an undefined margin with neighboring organs in the trachea and the thorax (SUV max 6.0). It also showed multiple lymph nodes near the lower trachea, next to the superior vena cava, posterior thymus, and aortic loop; the largest node was 26 × 40 mm, with increased metabolism of FDG (SUV max 7.9). The other organs were unaffected. There was no metastasis in other organs (Fig. ).\nThyroid function tests showed TSH: 0.00726 mIU/L[0,4-5], FT4: 22.34 pmol/L [12-22] and FT3: 4.0 pmol/L [1, 3-3, 1]. We reviewed the pathology of the current cervical lymph node specimen and that of the thyroid specimen from 5 years ago (2015). This showed that the thyroid tumor consisted of invasive cells which were poorly differentiated with squamous cell morphology, different from conventional thyroid carcinoma. The immunohistochemistry (IHC) analysis showed positive staining with CK5, CK7, p63, C-kit, CD5 and negative with TTF-1, Thyroglobulin, GATA3, ER, CD99, and NKX2.2, confirming CASTLE pathology in both the specimens.\nThe patient was diagnosed with a recurrence of the CASTLE thyroid gland in cervical lymph nodes. She was assessed by thoracic, and head and neck surgeons for the possibility of removing the maximum of recurrent tumors. However, it was considered a difficult, high-risk procedure; she and her family declined to go ahead with the surgery. Then, we offered the patient treatment with palliative radiotherapy. However, she and her family declined the treatment and got the patient discharged from the hospital. She was followed up without intervention and is currently stable 15 months after the recurrence. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
Patient 3 was a 67-year-old female who presented with hoarseness and dysphagia for a few weeks. CT scans showed a 3.8 cm left thyroid tumor with superior mediastinal and tracheal involvements, i.e., stage pT4bN0M0. Complete resection of the tumor was successfully achieved by total thyroidectomy and central neck dissection. Postsurgical chemotherapy was applied (docetaxel 120 mg/m2 and cisplatin 30 mg/m2), followed by a course of intensity modulated radiotherapy (70 Gy at 2.5 Gy per fraction). She remains well 4 years after diagnosis. | Patient 1 was a 61-year-old female who presented with a 5-cm anterior neck mass with retrosternal extension. She was surgically treated with a right thyroid lobectomy at another institution. The lesion was interpreted to be a poorly differentiated papillary thyroid cancer. Postoperative treatment consisted of sequential and combined chemoradiotherapy. Local recurrence in the region of the right thyroid occurred 20 months later, and radiotherapy was performed to relieve symptoms. Six years later, the patient had local recurrence again and was referred to our institution for further treatment. After curative wide local excision and central neck dissection, postoperative radiotherapy was given to the thyroid bed and bilateral cervical lymph node areas (55 Gy/16 fractions). Histological re-evaluation of the recurrent tumor specimens revealed CASTLE (pT4bN1M0). A third local recurrence was diagnosed 1 year later, and concurrent chemotherapy (paclitaxel 210 mg/m2 and cisplatin 40 mg/m2) and radiotherapy (50.4 Gy /28 fractions) were applied. The patient died from acute respiratory distress 1 month after the initiation of treatment. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 60-year-old gentleman with past medical history of cervical disk herniation presented with a 3-year history of progressive fatigue and bilateral upper extremity weakness. MRI was performed to evaluate the spine and was consistent with cervical spinal stenosis. T2 non-contrast images incidentally revealed an infiltrating mass in the left thyroid that was incompletely visualized. Ultrasound-guided fine needle aspiration showed atypia of undetermined significance. The patient was referred to surgery and was noted to have dysphonia but no stridor or dysphagia. The differential diagnosis was felt to include papillary thyroid carcinoma, follicular thyroid carcinoma, rare thyroid neoplasms including Hurthle cell carcinoma or anaplastic carcinoma, abscess, or autoimmune thyroid disease. He underwent total thyroidectomy with central neck dissection. On exploration, the thyroid mass was seen encasing the left recurrent laryngeal nerve and was shaved off the nerve and the trachea. The nerve was not stimulatable after resection and the patient developed left vocal cord paralysis. The tumor grossly measured 7 cm with extrathyroidal extension. There was lymphvascular space invasion and perineural invasion. Three lymph nodes were identified, and all were negative. Surgical margins had extensive involvement of tumor. Histopathologic analysis of the specimen showed high-grade carcinoma with thymus-like differentiation. Immunohistochemical staining was positive for pan-CK, p63 (cyokeratin and epithelial markers) and negative for chromogranin, thyroglobulin, and calcitonin (neuroendocrine, papillary and follicular thyroid, and medullary makers, respectively).\nThe tumor was arranged in broad, pushing, smooth-bordered islands abutted against a lymphocyte rich stroma. The lobules of tumor were surrounded by lymphocytes and plasma cells (Figures and ). The tumor cells were squamoid and syncytial to spindled, with eosinophilic cytoplasm, and the nuclei show mild pleomorphism with pale vesicular chromatin. The neoplastic cells were strongly immunoreactive for pancytokeratin and p63, as shown in Figures and . These cells were also strongly positive for CD5, which marks background T lymphocytes (Figure ). The neoplastic cells were non-reactive with TTF1 and thyroglobulin.\nPostoperative staging surveillance with PET scan was performed for systemic staging and due to positive margins. It showed no abnormal FDG uptake at distant sites and increased uptake at the operative bed, indeterminate between residual disease and postoperative changes. The patient underwent adjuvant treatment with radiation therapy to 64 Gy in 32 fractions with intensity modulated radiotherapy. Concurrent chemotherapy was given based on his extensive positive margins for radiosensitization. A dose of 60 Gy was delivered to the bilateral neck including paratracheal nodes into the upper mediastinum, with a 4 Gy sequential boost to the operative site. A coronal section of the radiotherapy plan is shown in Figure . Six cycles of intravenous concurrent cisplatin 35 mg/m2 were infused. During therapy, he developed grade 2 skin reaction characterized by dry desquamation and moderate erythema of the neck and upper chest. Skin moisturizer was used for therapy. He experienced grade 2 esophagitis and mucositis of the oropharynx and grade 2 nausea requiring topical analgesics, oral narcotics, antiemetics, and intravenous fluid administration. Weight decreased from 108 to 95.5 kg by the end of therapy. Nutritional counseling by dietician and oral nutritional supplementation were provided. With chemotherapy, the patient developed a tinnitus grade 1. Toxicities are reported per the Common Terminology Criteria for Adverse Events ().\nAfter treatment, the patient initially experienced continued weight loss, and as a result underwent percutaneous endoscopic gastrostomy tube placement. Five months after end of treatment, the tube was removed after the patient was able to gain significant weight and completely obtain nutrition per oral route for 6 weeks. Dysphagia to solid foods is improving. He experienced persistent dysphonia secondary to left vocal cord paralysis since surgery and underwent left medialization thyroplasty 1 year later. He displayed improvement in voice quality. At 3-years follow-up after completion of radiotherapy, our patient is without evidence of locally recurrent or distant disease. | Patient 3 was a 67-year-old female who presented with hoarseness and dysphagia for a few weeks. CT scans showed a 3.8 cm left thyroid tumor with superior mediastinal and tracheal involvements, i.e., stage pT4bN0M0. Complete resection of the tumor was successfully achieved by total thyroidectomy and central neck dissection. Postsurgical chemotherapy was applied (docetaxel 120 mg/m2 and cisplatin 30 mg/m2), followed by a course of intensity modulated radiotherapy (70 Gy at 2.5 Gy per fraction). She remains well 4 years after diagnosis. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
Patient 3 was a 67-year-old female who presented with hoarseness and dysphagia for a few weeks. CT scans showed a 3.8 cm left thyroid tumor with superior mediastinal and tracheal involvements, i.e., stage pT4bN0M0. Complete resection of the tumor was successfully achieved by total thyroidectomy and central neck dissection. Postsurgical chemotherapy was applied (docetaxel 120 mg/m2 and cisplatin 30 mg/m2), followed by a course of intensity modulated radiotherapy (70 Gy at 2.5 Gy per fraction). She remains well 4 years after diagnosis. | Patient 2 was a 48-year-old male pathologically diagnosed with stage pT4aN0M0 CASTLE postsurgery. He presented with rapidly growing thyroid nodules with fixation to underlying structures. He underwent total thyroidectomy with central neck dissection. On exploration, the thyroid mass was seen extended to the left laryngeal nerve, strap muscle, trachea, and esophagus. Curative wide local excision was attempted on patients with gross residual local disease. Postoperative external beam radiation therapy (50.4 Gy/28 fractions) was given to the thyroid bed and bilateral cervical lymph node area. He remained well 2 years post therapy with no palpable neck disease. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 34-year-old woman presented with a 2-month history of sore throat. The patient had no notable medical or family history. Physical examination revealed a palpable right thyroid mass. Thyroid function and calcium tests were within normal limits. Ultrasound showed a 1.7-cm, well demarcated, heterogeneous, low echoic solid mass in the lower pole of the right thyroid (). A computed tomography scan of the neck revealed a 3.3-cm, exophytic, low-attenuated mass in the lower pole of the right thyroid. The FNA biopsy of the mass was performed, which was accompanied by the standard Papanicolaou stain.\nThe smear was composed of not only cohesive three dimensional clusters and sheets but also singly scattered cells (). The tumor cells showed mild anisonucleosis and high nuclear : cytoplasmic (N/C) ratios. Nuclei were round to ovoid with irregular nuclear contours and small, distinct nucleoli (). The nuclear chromatin was vesicular or coarsely granular. Nuclear grooves were rarely seen. Cytoplasm was scanty and amphophilic. Cytoplasmic border was indistinct. Some tumor cells showed dense orangeophilic cytoplasm, which is suggestive of individual cell keratinization (). Some lymphocytes were found on the background and within clusters, which was accompanied by the presence of a few stripped nuclei (). Mitotic figures were rarely seen. Intranuclear cytoplasmic inclusions were not observed. Papillary or follicular structure was not observed. The aspirate was interpreted as a high-grade malignant thyroid neoplasm without further definitive classification. The total thyroidectomy was performed.\nOn gross examination, the thyroid contained a 4.0-cm solid mass replacing most of the right thyroid. The mass had a lobular, tan-colored cut surface and a firm consistency (). On microscopic examination, the tumor was well-circumscribed and it was composed of variably sized and irregularly shaped lobules of cohesive polygonal tumor cells which were separated by bands of dense fibrous stroma (). The tumor cells had high N/C ratios, eosinophilic cytoplasm and ill-defined cell borders. The nuclei were vesicular or coarsely granular with prominent nucleoli. The mitotic figures were sparse. There was mild lymphoplasmacytic infiltration within the tumor nodules and septae. Cytoplasmic keratinization was seen. The tumor cells showed positivity for CD5, carcinoembryonic antigen, high molecular weight keratin (HMWK), cytokeratin 5 (CK5), and p63. The tumor was negative for thyroid transcription factor-1, thyroglobulin, and calcitonin. Vascular invasion and extensive extra-thyroidal extension were present. One regional lymph node was found and involved by tumor.\nOver a 27-month follow-up period, the patient had no evidence of tumor recurrence. | Patient 3 was a 67-year-old female who presented with hoarseness and dysphagia for a few weeks. CT scans showed a 3.8 cm left thyroid tumor with superior mediastinal and tracheal involvements, i.e., stage pT4bN0M0. Complete resection of the tumor was successfully achieved by total thyroidectomy and central neck dissection. Postsurgical chemotherapy was applied (docetaxel 120 mg/m2 and cisplatin 30 mg/m2), followed by a course of intensity modulated radiotherapy (70 Gy at 2.5 Gy per fraction). She remains well 4 years after diagnosis. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 79-year old woman was hospitalized on April 25, 2018, at which time she had progressive dyspnea over the past 5 weeks, productive cough, poor appetite, and weight loss of 5 kg over the last 6 months currently weighing 81 kg. Her medical history included an invasive ductal breast cancer pT1cN0M0 G2 ER 90%, PR 20% 20 years ago treated with lumpectomy, radiotherapy, and tamoxifen until 2004, and an undifferentiated cancer of the right parotid pT1 (17 mm) pN2 (1/2 positive in level I, 1/11 positive in level II, no extranodal extensions) pM0 diagnosed 2008 and treated with a total parotidectomy achieving R0 margins and a selective neck dissection levels II-IV followed by radiotherapy.\nOn physical examination, the patient was in mild respiratory distress. The pulmonary exam revealed dullness to percussion, decreased fremitus, and decreased breathing sounds in up to two-thirds of the left lung field. Oxygen saturation was 92% with the patient breathing ambient air, and arterial blood gases showed partial respiratory insufficiency. A chest X-ray confirmed a left-sided pleural effusion, and thoracentesis removed 1.5 liters of turbid fluid. Biochemical analysis of the fluid established an exudative etiology. Atypical cells with immunocytochemical positivity for p40, cytokeratin 5, and p63 in the cytopathology specimen were primarily suggestive of a non-small cell lung cancer, and a CT scan of the thorax showed a mass (4.2 × 2.2 × 2.8 cm) in the right upper lobe and extensive cardiophrenic, mediastinal, and hilar lymphadenopathy as well as enlarged lymph nodes along the left arteria mammaria interna. An FDG PET/CT scan confirmed these findings, and the scan revealed a hypermetabolic activity in mesenteric lymph nodes and at the left pleura (). Thoracoscopy and biopsy of the parietal pleura were performed. Surprisingly, histopathological examination revealed a non-small cell carcinoma () expressing CD5 (), CD117 (), and p63 all indicating a carcinoma of thymic origin (). With no signs of a thymic pathology, the histopathological diagnosis of CASTLE was established, and as additional immunostainings for CD5 () and CD117 () in the specimen of the parotid cancer diagnosed 2008 were positive also, a rare case of a CASTLE tumor of the parotid healed at the primary and neck sites, but with late metastatic dissemination was diagnosed.\nAs CD117 was highly overexpressed in the tumor, exons 9, 11, 13, 14, and 17 of the C-KIT gene were analyzed after extraction of genomic DNA by PCR using specific primers, but no common mutation was found consistent with previous reports (, ). Anticancer immunity and potential response to immunotherapy were assessed by PD-L1 staining of the pleural tumor, which showed a high expression level of 60% ().\nThe patient underwent talc pleurodesis and left partial pleurectomy for recurrent pleural effusions. Systemically, disseminated metastatic disease was treated with the monoclonal PD-L1 inhibitor pembrolizumab at a dose 200 mg every 3 weeks. Four months after the start of immunotherapy a follow-up FDG PET/CT study showed a partial remission with a moderate morphologic reduction of the tumor load, but a markedly reduced metabolic activity at all tumor sites (). Pembrolizumab treatment continued at a dose of 200 mg IV every 3 weeks. The patient showed a mild skin rash treated with low dose prednisolone (20 mg with tapering) for 2 weeks. No other adverse events associated with pembrolizumab were observed, and the therapy was tolerated well. At the last follow-up, the patient was in good clinical condition and had gained 3 kg of weight. On March 28th, 2019, a second follow-up FDG PET/CT showed no residual pleural effusion (not shown) and a further regression of lesions in the right lung and lymph nodes. Residual focal FDG-Uptake was seen in the left pleura (). | Patient 3 was a 67-year-old female who presented with hoarseness and dysphagia for a few weeks. CT scans showed a 3.8 cm left thyroid tumor with superior mediastinal and tracheal involvements, i.e., stage pT4bN0M0. Complete resection of the tumor was successfully achieved by total thyroidectomy and central neck dissection. Postsurgical chemotherapy was applied (docetaxel 120 mg/m2 and cisplatin 30 mg/m2), followed by a course of intensity modulated radiotherapy (70 Gy at 2.5 Gy per fraction). She remains well 4 years after diagnosis. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 52-year-old woman was referred to our hospital because of severe dyspnea and the presence of a neck tumor. She had experienced dyspnea for the past 6 months and had recently noticed bloody sputum. A computed tomography examination of the neck revealed a thyroid tumor about 8 cm in size. The tumor had invaded the trachea, obstructing 90% of the tracheal lumen (fig. ). An emergency tracheotomy was performed to maintain an airway through the tumor tissue. On biopsy, the tumor was diagnosed as CASTLE, suggesting a favorable prognosis if a complete resection could be performed. Magnetic resonance imaging and an esophageal fibroscopy examination revealed a low possibility of esophageal invasion (fig. ). Under general anesthesia, a thyroidectomy was initially performed via a neck incision. The invaded portion of the trachea was sharply dissected. A partial sternotomy was performed at the level of the 2nd intercostal space to identify the distal portion of the intact trachea and to mobilize the inferior tracheal segment. The trachea was transversely opened to find the intact portion. The length of the resected trachea was 6 cm (fig. ). Bilateral recurrent nerves were macroscopically preserved. We performed a suprahyoid release to mobilize the superior tracheal segment, but these mobilizations were insufficient to perform an anastomosis. Thus, to allow additional mobilization, we performed a clamshell thoracotomy and incised the pericardium 360° around the hilum, dissecting the pulmonary ligament bilaterally. These procedures allowed an anastomosis. An end-to-end bronchial anastomosis was carried out using running 4-0 PDS II sutures (Ethicon, Somerville, N.J., USA) for the membranous portion and interrupted 4-0 PDS II sutures for the cartilaginous portion. The bronchial anastomosis was wrapped with an omental pedicle flap that was lifted up through a retrosternal route via a laparotomy. A gastrostomy was also performed for postoperative enteral nutrition. For management after surgery, a chin suture was placed. Neck mobilization was restricted for the placement of the chin suture and airway fixation. After confirming an improvement in the laryngeal edema using a laryngoscopy, extubation was performed on postoperative day (POD) 17. The patient exhibited swallowing disturbances and underwent swallowing rehabilitation. Enteral nutrition was provided through the gastrostomy. The patient was discharged on POD 31, and per os feeding was started on POD 51. Radiotherapy (60 Gy in 40 daily fractions of 1.5 Gy) was performed as an adjuvant therapy. Throughout the clinical course, no major complications, including swallowing disturbances, hoarseness or anastomotic problems, were observed. A histopathological examination of the resected specimen revealed the characteristic morphology of CASTLE (fig. ); immunohistochemistry was positive for CD5 (fig. ) but negative for thyroid transcription factor 1 (TTF-1; fig. ).\nEleven months after surgery, lower back pain and right leg numbness developed and led to gait inability. Multiple lung and bone recurrences were observed, but no local recurrence. In order to ease the back pain, palliative radiotherapy (35 Gy in 14 fractions) to the bone metastasis was administered. Twelve months after surgery, drainage of pleural effusion was performed. She died of pleural metastasis 14 months after the initial diagnosis of CASTLE. | Patient 3 was a 67-year-old female who presented with hoarseness and dysphagia for a few weeks. CT scans showed a 3.8 cm left thyroid tumor with superior mediastinal and tracheal involvements, i.e., stage pT4bN0M0. Complete resection of the tumor was successfully achieved by total thyroidectomy and central neck dissection. Postsurgical chemotherapy was applied (docetaxel 120 mg/m2 and cisplatin 30 mg/m2), followed by a course of intensity modulated radiotherapy (70 Gy at 2.5 Gy per fraction). She remains well 4 years after diagnosis. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 76-year-old man was referred to our hospital for investigation of a 20-day history of rapidly deteriorating dyspnea and severe cough. This was associated with dysphagia and cervical pain. The patient was an ex-marble craftsman and an active smoker. His medical history included chronic pulmonary obstructive disease (COPD). A chest X-ray (CXR) showed an imprint at the right side of the trachea. Thyroid ultrasound (US) revealed a large and heterogenous thyroid gland and the presence of a calcified mass, 4.76 × 4.01 cm with high blood perfusion in the right upper lobe of the gland. A contrast-enhanced computed tomography (CT) scan of the neck and thorax reported the corresponding lesion as heterogenous, with irregular borders, extending from the sternoclavicular joints to the upper borders of the thyroid gland. The mass was surrounding the trachea anterolaterally displacing it to the left and the right common artery to the right (). There were no enlarged lymph nodes. Bronchoscopy revealed a fracture of the first tracheal ring but there was no evidence of tumour invasion (). Cytology from bronchial lavage was negative for cancer cells. Laboratory tests were within normal limits with the exception of elevated D-dimers (2,994 μg/L) and elevated white blood cells (19.27 K/μL). Tumour markers were within normal range values.\nOn admission, the patient was intubated, due to acute respiratory failure, and he was admitted to the intensive care unit (ICU). Subsequently, he underwent an emergency procedure and a collar incision was carried out. At surgery, a suspicious looking tumour, which was in continuity with the superior pole of the right thyroid lobe, the sheath of right common carotid artery, and the anterolateral portion of the cartilaginous trachea, was identified. Macroscopically, the tumour looked whitish and scleroelastic with moderately regular borders and was firmly adhered to the aforementioned structures. After the right recurrent laryngeal nerve was identified, the tumour was carefully dissected off the right thyroid lobe and the adventitia of the right common carotid artery. A similar approach was carried out to dissect the tumour off the left lobe; hence, an en bloc tumour resection with total thyroidectomy and a regional lymph node dissection was performed. Rapid frozen section biopsy of the tumour in the right thyroid lobe proved to be malignant with features of squamous cell carcinoma and negative surgical margins.\nPostoperatively, the patient experienced swallowing difficulty and hoarseness and a tracheostomy was performed on day 2.\nFinal pathology examination confirmed frozen section's diagnosis of a squamous cell carcinoma invading the right lobe of the thyroid gland, as well as extracapsular structures of the left lobe. Microscopically, the tumour was described as nonencapsulated, irregularly lobulated, with broad fibrous septa, and lymphoplasmacytic, eosinophilic, histiocytic, and multinuclear infiltration. The neoplastic cells were largely polyhedral or oval with eosinophilic or amphophilic cytoplasm, arranged in islands or nests that showed focal keratinization. The nuclei were vesicular or hyperchromatic with nucleoli. A panel of immunohistochemical stains was performed; the tumour was positive for p63, CD117, CKH, CD5, and CD3 in the lymphocytic part (Figures , , and ). It was also negative for the expression of TTF1.\nThe patient subsequently improved and was discharged home on postoperative day 20. The patient did not receive any additional treatment. | Patient 3 was a 67-year-old female who presented with hoarseness and dysphagia for a few weeks. CT scans showed a 3.8 cm left thyroid tumor with superior mediastinal and tracheal involvements, i.e., stage pT4bN0M0. Complete resection of the tumor was successfully achieved by total thyroidectomy and central neck dissection. Postsurgical chemotherapy was applied (docetaxel 120 mg/m2 and cisplatin 30 mg/m2), followed by a course of intensity modulated radiotherapy (70 Gy at 2.5 Gy per fraction). She remains well 4 years after diagnosis. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
The patient, a 28-year-old man with a 3-month-old mass in the neck was referred to our department. The mass, located on the left thyroid lobe, was elastic, hard, showed limited mobility, and measured approximately 30 × 20 mm. There was no remarkable tenderness over the mass.\nBlood examination results were normal, including levels of thyroid-stimulating hormone, free thyroid hormone, and thyroglobulin (Table ). Ultrasonography revealed a hypoechoic solid mass in the lower pole of the left thyroid lobe with a poorly marginated heterogeneous pattern (Fig. ). Computed tomography revealed a heterogenic tumor in the left thyroid lobe (Fig. ). 18F-fluorodeoxyglucose (18F-FDG) accumulated in the thyroid tumor on positron-emission tomography. The standardized uptake value of 18F-FDG was 5.29 (Fig. ). Fine-needle aspiration cytology (FNAC) showed a number of lymphoid cells without atypia that were similar to those seen in malignant lymphoma of the thyroid (Fig. ).\nAs we diagnosed a highly malignant thyroid cancer (cT2N0M0), we performed a left hemithyroidectomy with central neck dissection (ND). Histopathological findings showed that the tumor was composed of irregular insular nests of neoplastic cells and had an expansive growth pattern in the thyroid (Fig. ). The carcinoma cells had round nuclei or polymorphonuclei with well-defined nucleoli and ill-defined cell borders (Fig. ). Immunohistochemical studies showed that the tumor was partially positive for CD5, positive for Ki-67 (approx. 30%), and negative for CD20, calcitonin, and thyroglobulin (Fig. ). Based on these histopathological findings, the tumor was finally diagnosed as CASTLE. There were no signs of recurrence without additional treatment after 10 years of follow-up. | Patient 3 was a 67-year-old female who presented with hoarseness and dysphagia for a few weeks. CT scans showed a 3.8 cm left thyroid tumor with superior mediastinal and tracheal involvements, i.e., stage pT4bN0M0. Complete resection of the tumor was successfully achieved by total thyroidectomy and central neck dissection. Postsurgical chemotherapy was applied (docetaxel 120 mg/m2 and cisplatin 30 mg/m2), followed by a course of intensity modulated radiotherapy (70 Gy at 2.5 Gy per fraction). She remains well 4 years after diagnosis. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 60-year-old woman, who was diagnosed with cancer of the thyroid gland in 2015, was treated with total thyroidectomy and lymph node dissection. Her postoperative pathology showed carcinoma showing thymus-like differentiation (CASTLE). She was discharged on Levothyroxine 100 mg/day. She did not attend her follow-up appointments. After 2 years, the patient found a lump in the left side of her neck. The tumor grew very slowly and did not cause any symptoms; hence, she did not seek medical advice. In May 2020, about 3 years after the appearance of her left neck tumor, the patient was admitted to our hospital with shortness of breath due to tracheal compression from the tumor. Clinical examination showed enlarged solid painless cervical lymph nodes at levels III and IV, stuck in a block size of 3 × 6 cm in diameter. There was no involvement of the hypopharynx, and her vocal cords were intact. CT scan of her neck revealed a 16 × 54 mm lesion compressing the trachea, and several enlarged left cervical lymph nodes with the size of about 25 mm (Fig. ). The patient was offered a tracheostomy and tracheal stent before undertaking further investigations.\nHer subsequent fluorine-18-labeled fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) scan revealed previous total thyroidectomy, no evidence of recurrence in the surgical bed, enlarged left cervical lymph nodes at levels III and IV, and extranodal extension into sternocleidomastoid muscle measuring 29 × 43x59 mm, with the maximum standardized uptake value (SUV max) of 6.8. Her thymus measured 19 × 31x37mm, with increased metabolism of FDG (SUV max 3.6). There was a 33 × 39x49 mm mass on the left side of the anterior mediastinum, showing an undefined margin with neighboring organs in the trachea and the thorax (SUV max 6.0). It also showed multiple lymph nodes near the lower trachea, next to the superior vena cava, posterior thymus, and aortic loop; the largest node was 26 × 40 mm, with increased metabolism of FDG (SUV max 7.9). The other organs were unaffected. There was no metastasis in other organs (Fig. ).\nThyroid function tests showed TSH: 0.00726 mIU/L[0,4-5], FT4: 22.34 pmol/L [12-22] and FT3: 4.0 pmol/L [1, 3-3, 1]. We reviewed the pathology of the current cervical lymph node specimen and that of the thyroid specimen from 5 years ago (2015). This showed that the thyroid tumor consisted of invasive cells which were poorly differentiated with squamous cell morphology, different from conventional thyroid carcinoma. The immunohistochemistry (IHC) analysis showed positive staining with CK5, CK7, p63, C-kit, CD5 and negative with TTF-1, Thyroglobulin, GATA3, ER, CD99, and NKX2.2, confirming CASTLE pathology in both the specimens.\nThe patient was diagnosed with a recurrence of the CASTLE thyroid gland in cervical lymph nodes. She was assessed by thoracic, and head and neck surgeons for the possibility of removing the maximum of recurrent tumors. However, it was considered a difficult, high-risk procedure; she and her family declined to go ahead with the surgery. Then, we offered the patient treatment with palliative radiotherapy. However, she and her family declined the treatment and got the patient discharged from the hospital. She was followed up without intervention and is currently stable 15 months after the recurrence. | Patient 3 was a 67-year-old female who presented with hoarseness and dysphagia for a few weeks. CT scans showed a 3.8 cm left thyroid tumor with superior mediastinal and tracheal involvements, i.e., stage pT4bN0M0. Complete resection of the tumor was successfully achieved by total thyroidectomy and central neck dissection. Postsurgical chemotherapy was applied (docetaxel 120 mg/m2 and cisplatin 30 mg/m2), followed by a course of intensity modulated radiotherapy (70 Gy at 2.5 Gy per fraction). She remains well 4 years after diagnosis. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 60-year-old gentleman with past medical history of cervical disk herniation presented with a 3-year history of progressive fatigue and bilateral upper extremity weakness. MRI was performed to evaluate the spine and was consistent with cervical spinal stenosis. T2 non-contrast images incidentally revealed an infiltrating mass in the left thyroid that was incompletely visualized. Ultrasound-guided fine needle aspiration showed atypia of undetermined significance. The patient was referred to surgery and was noted to have dysphonia but no stridor or dysphagia. The differential diagnosis was felt to include papillary thyroid carcinoma, follicular thyroid carcinoma, rare thyroid neoplasms including Hurthle cell carcinoma or anaplastic carcinoma, abscess, or autoimmune thyroid disease. He underwent total thyroidectomy with central neck dissection. On exploration, the thyroid mass was seen encasing the left recurrent laryngeal nerve and was shaved off the nerve and the trachea. The nerve was not stimulatable after resection and the patient developed left vocal cord paralysis. The tumor grossly measured 7 cm with extrathyroidal extension. There was lymphvascular space invasion and perineural invasion. Three lymph nodes were identified, and all were negative. Surgical margins had extensive involvement of tumor. Histopathologic analysis of the specimen showed high-grade carcinoma with thymus-like differentiation. Immunohistochemical staining was positive for pan-CK, p63 (cyokeratin and epithelial markers) and negative for chromogranin, thyroglobulin, and calcitonin (neuroendocrine, papillary and follicular thyroid, and medullary makers, respectively).\nThe tumor was arranged in broad, pushing, smooth-bordered islands abutted against a lymphocyte rich stroma. The lobules of tumor were surrounded by lymphocytes and plasma cells (Figures and ). The tumor cells were squamoid and syncytial to spindled, with eosinophilic cytoplasm, and the nuclei show mild pleomorphism with pale vesicular chromatin. The neoplastic cells were strongly immunoreactive for pancytokeratin and p63, as shown in Figures and . These cells were also strongly positive for CD5, which marks background T lymphocytes (Figure ). The neoplastic cells were non-reactive with TTF1 and thyroglobulin.\nPostoperative staging surveillance with PET scan was performed for systemic staging and due to positive margins. It showed no abnormal FDG uptake at distant sites and increased uptake at the operative bed, indeterminate between residual disease and postoperative changes. The patient underwent adjuvant treatment with radiation therapy to 64 Gy in 32 fractions with intensity modulated radiotherapy. Concurrent chemotherapy was given based on his extensive positive margins for radiosensitization. A dose of 60 Gy was delivered to the bilateral neck including paratracheal nodes into the upper mediastinum, with a 4 Gy sequential boost to the operative site. A coronal section of the radiotherapy plan is shown in Figure . Six cycles of intravenous concurrent cisplatin 35 mg/m2 were infused. During therapy, he developed grade 2 skin reaction characterized by dry desquamation and moderate erythema of the neck and upper chest. Skin moisturizer was used for therapy. He experienced grade 2 esophagitis and mucositis of the oropharynx and grade 2 nausea requiring topical analgesics, oral narcotics, antiemetics, and intravenous fluid administration. Weight decreased from 108 to 95.5 kg by the end of therapy. Nutritional counseling by dietician and oral nutritional supplementation were provided. With chemotherapy, the patient developed a tinnitus grade 1. Toxicities are reported per the Common Terminology Criteria for Adverse Events ().\nAfter treatment, the patient initially experienced continued weight loss, and as a result underwent percutaneous endoscopic gastrostomy tube placement. Five months after end of treatment, the tube was removed after the patient was able to gain significant weight and completely obtain nutrition per oral route for 6 weeks. Dysphagia to solid foods is improving. He experienced persistent dysphonia secondary to left vocal cord paralysis since surgery and underwent left medialization thyroplasty 1 year later. He displayed improvement in voice quality. At 3-years follow-up after completion of radiotherapy, our patient is without evidence of locally recurrent or distant disease. | Patient 1 was a 61-year-old female who presented with a 5-cm anterior neck mass with retrosternal extension. She was surgically treated with a right thyroid lobectomy at another institution. The lesion was interpreted to be a poorly differentiated papillary thyroid cancer. Postoperative treatment consisted of sequential and combined chemoradiotherapy. Local recurrence in the region of the right thyroid occurred 20 months later, and radiotherapy was performed to relieve symptoms. Six years later, the patient had local recurrence again and was referred to our institution for further treatment. After curative wide local excision and central neck dissection, postoperative radiotherapy was given to the thyroid bed and bilateral cervical lymph node areas (55 Gy/16 fractions). Histological re-evaluation of the recurrent tumor specimens revealed CASTLE (pT4bN1M0). A third local recurrence was diagnosed 1 year later, and concurrent chemotherapy (paclitaxel 210 mg/m2 and cisplatin 40 mg/m2) and radiotherapy (50.4 Gy /28 fractions) were applied. The patient died from acute respiratory distress 1 month after the initiation of treatment. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
Patient 2 was a 48-year-old male pathologically diagnosed with stage pT4aN0M0 CASTLE postsurgery. He presented with rapidly growing thyroid nodules with fixation to underlying structures. He underwent total thyroidectomy with central neck dissection. On exploration, the thyroid mass was seen extended to the left laryngeal nerve, strap muscle, trachea, and esophagus. Curative wide local excision was attempted on patients with gross residual local disease. Postoperative external beam radiation therapy (50.4 Gy/28 fractions) was given to the thyroid bed and bilateral cervical lymph node area. He remained well 2 years post therapy with no palpable neck disease. | Patient 1 was a 61-year-old female who presented with a 5-cm anterior neck mass with retrosternal extension. She was surgically treated with a right thyroid lobectomy at another institution. The lesion was interpreted to be a poorly differentiated papillary thyroid cancer. Postoperative treatment consisted of sequential and combined chemoradiotherapy. Local recurrence in the region of the right thyroid occurred 20 months later, and radiotherapy was performed to relieve symptoms. Six years later, the patient had local recurrence again and was referred to our institution for further treatment. After curative wide local excision and central neck dissection, postoperative radiotherapy was given to the thyroid bed and bilateral cervical lymph node areas (55 Gy/16 fractions). Histological re-evaluation of the recurrent tumor specimens revealed CASTLE (pT4bN1M0). A third local recurrence was diagnosed 1 year later, and concurrent chemotherapy (paclitaxel 210 mg/m2 and cisplatin 40 mg/m2) and radiotherapy (50.4 Gy /28 fractions) were applied. The patient died from acute respiratory distress 1 month after the initiation of treatment. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 34-year-old woman presented with a 2-month history of sore throat. The patient had no notable medical or family history. Physical examination revealed a palpable right thyroid mass. Thyroid function and calcium tests were within normal limits. Ultrasound showed a 1.7-cm, well demarcated, heterogeneous, low echoic solid mass in the lower pole of the right thyroid (). A computed tomography scan of the neck revealed a 3.3-cm, exophytic, low-attenuated mass in the lower pole of the right thyroid. The FNA biopsy of the mass was performed, which was accompanied by the standard Papanicolaou stain.\nThe smear was composed of not only cohesive three dimensional clusters and sheets but also singly scattered cells (). The tumor cells showed mild anisonucleosis and high nuclear : cytoplasmic (N/C) ratios. Nuclei were round to ovoid with irregular nuclear contours and small, distinct nucleoli (). The nuclear chromatin was vesicular or coarsely granular. Nuclear grooves were rarely seen. Cytoplasm was scanty and amphophilic. Cytoplasmic border was indistinct. Some tumor cells showed dense orangeophilic cytoplasm, which is suggestive of individual cell keratinization (). Some lymphocytes were found on the background and within clusters, which was accompanied by the presence of a few stripped nuclei (). Mitotic figures were rarely seen. Intranuclear cytoplasmic inclusions were not observed. Papillary or follicular structure was not observed. The aspirate was interpreted as a high-grade malignant thyroid neoplasm without further definitive classification. The total thyroidectomy was performed.\nOn gross examination, the thyroid contained a 4.0-cm solid mass replacing most of the right thyroid. The mass had a lobular, tan-colored cut surface and a firm consistency (). On microscopic examination, the tumor was well-circumscribed and it was composed of variably sized and irregularly shaped lobules of cohesive polygonal tumor cells which were separated by bands of dense fibrous stroma (). The tumor cells had high N/C ratios, eosinophilic cytoplasm and ill-defined cell borders. The nuclei were vesicular or coarsely granular with prominent nucleoli. The mitotic figures were sparse. There was mild lymphoplasmacytic infiltration within the tumor nodules and septae. Cytoplasmic keratinization was seen. The tumor cells showed positivity for CD5, carcinoembryonic antigen, high molecular weight keratin (HMWK), cytokeratin 5 (CK5), and p63. The tumor was negative for thyroid transcription factor-1, thyroglobulin, and calcitonin. Vascular invasion and extensive extra-thyroidal extension were present. One regional lymph node was found and involved by tumor.\nOver a 27-month follow-up period, the patient had no evidence of tumor recurrence. | Patient 1 was a 61-year-old female who presented with a 5-cm anterior neck mass with retrosternal extension. She was surgically treated with a right thyroid lobectomy at another institution. The lesion was interpreted to be a poorly differentiated papillary thyroid cancer. Postoperative treatment consisted of sequential and combined chemoradiotherapy. Local recurrence in the region of the right thyroid occurred 20 months later, and radiotherapy was performed to relieve symptoms. Six years later, the patient had local recurrence again and was referred to our institution for further treatment. After curative wide local excision and central neck dissection, postoperative radiotherapy was given to the thyroid bed and bilateral cervical lymph node areas (55 Gy/16 fractions). Histological re-evaluation of the recurrent tumor specimens revealed CASTLE (pT4bN1M0). A third local recurrence was diagnosed 1 year later, and concurrent chemotherapy (paclitaxel 210 mg/m2 and cisplatin 40 mg/m2) and radiotherapy (50.4 Gy /28 fractions) were applied. The patient died from acute respiratory distress 1 month after the initiation of treatment. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 79-year old woman was hospitalized on April 25, 2018, at which time she had progressive dyspnea over the past 5 weeks, productive cough, poor appetite, and weight loss of 5 kg over the last 6 months currently weighing 81 kg. Her medical history included an invasive ductal breast cancer pT1cN0M0 G2 ER 90%, PR 20% 20 years ago treated with lumpectomy, radiotherapy, and tamoxifen until 2004, and an undifferentiated cancer of the right parotid pT1 (17 mm) pN2 (1/2 positive in level I, 1/11 positive in level II, no extranodal extensions) pM0 diagnosed 2008 and treated with a total parotidectomy achieving R0 margins and a selective neck dissection levels II-IV followed by radiotherapy.\nOn physical examination, the patient was in mild respiratory distress. The pulmonary exam revealed dullness to percussion, decreased fremitus, and decreased breathing sounds in up to two-thirds of the left lung field. Oxygen saturation was 92% with the patient breathing ambient air, and arterial blood gases showed partial respiratory insufficiency. A chest X-ray confirmed a left-sided pleural effusion, and thoracentesis removed 1.5 liters of turbid fluid. Biochemical analysis of the fluid established an exudative etiology. Atypical cells with immunocytochemical positivity for p40, cytokeratin 5, and p63 in the cytopathology specimen were primarily suggestive of a non-small cell lung cancer, and a CT scan of the thorax showed a mass (4.2 × 2.2 × 2.8 cm) in the right upper lobe and extensive cardiophrenic, mediastinal, and hilar lymphadenopathy as well as enlarged lymph nodes along the left arteria mammaria interna. An FDG PET/CT scan confirmed these findings, and the scan revealed a hypermetabolic activity in mesenteric lymph nodes and at the left pleura (). Thoracoscopy and biopsy of the parietal pleura were performed. Surprisingly, histopathological examination revealed a non-small cell carcinoma () expressing CD5 (), CD117 (), and p63 all indicating a carcinoma of thymic origin (). With no signs of a thymic pathology, the histopathological diagnosis of CASTLE was established, and as additional immunostainings for CD5 () and CD117 () in the specimen of the parotid cancer diagnosed 2008 were positive also, a rare case of a CASTLE tumor of the parotid healed at the primary and neck sites, but with late metastatic dissemination was diagnosed.\nAs CD117 was highly overexpressed in the tumor, exons 9, 11, 13, 14, and 17 of the C-KIT gene were analyzed after extraction of genomic DNA by PCR using specific primers, but no common mutation was found consistent with previous reports (, ). Anticancer immunity and potential response to immunotherapy were assessed by PD-L1 staining of the pleural tumor, which showed a high expression level of 60% ().\nThe patient underwent talc pleurodesis and left partial pleurectomy for recurrent pleural effusions. Systemically, disseminated metastatic disease was treated with the monoclonal PD-L1 inhibitor pembrolizumab at a dose 200 mg every 3 weeks. Four months after the start of immunotherapy a follow-up FDG PET/CT study showed a partial remission with a moderate morphologic reduction of the tumor load, but a markedly reduced metabolic activity at all tumor sites (). Pembrolizumab treatment continued at a dose of 200 mg IV every 3 weeks. The patient showed a mild skin rash treated with low dose prednisolone (20 mg with tapering) for 2 weeks. No other adverse events associated with pembrolizumab were observed, and the therapy was tolerated well. At the last follow-up, the patient was in good clinical condition and had gained 3 kg of weight. On March 28th, 2019, a second follow-up FDG PET/CT showed no residual pleural effusion (not shown) and a further regression of lesions in the right lung and lymph nodes. Residual focal FDG-Uptake was seen in the left pleura (). | Patient 1 was a 61-year-old female who presented with a 5-cm anterior neck mass with retrosternal extension. She was surgically treated with a right thyroid lobectomy at another institution. The lesion was interpreted to be a poorly differentiated papillary thyroid cancer. Postoperative treatment consisted of sequential and combined chemoradiotherapy. Local recurrence in the region of the right thyroid occurred 20 months later, and radiotherapy was performed to relieve symptoms. Six years later, the patient had local recurrence again and was referred to our institution for further treatment. After curative wide local excision and central neck dissection, postoperative radiotherapy was given to the thyroid bed and bilateral cervical lymph node areas (55 Gy/16 fractions). Histological re-evaluation of the recurrent tumor specimens revealed CASTLE (pT4bN1M0). A third local recurrence was diagnosed 1 year later, and concurrent chemotherapy (paclitaxel 210 mg/m2 and cisplatin 40 mg/m2) and radiotherapy (50.4 Gy /28 fractions) were applied. The patient died from acute respiratory distress 1 month after the initiation of treatment. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
A 52-year-old woman was referred to our hospital because of severe dyspnea and the presence of a neck tumor. She had experienced dyspnea for the past 6 months and had recently noticed bloody sputum. A computed tomography examination of the neck revealed a thyroid tumor about 8 cm in size. The tumor had invaded the trachea, obstructing 90% of the tracheal lumen (fig. ). An emergency tracheotomy was performed to maintain an airway through the tumor tissue. On biopsy, the tumor was diagnosed as CASTLE, suggesting a favorable prognosis if a complete resection could be performed. Magnetic resonance imaging and an esophageal fibroscopy examination revealed a low possibility of esophageal invasion (fig. ). Under general anesthesia, a thyroidectomy was initially performed via a neck incision. The invaded portion of the trachea was sharply dissected. A partial sternotomy was performed at the level of the 2nd intercostal space to identify the distal portion of the intact trachea and to mobilize the inferior tracheal segment. The trachea was transversely opened to find the intact portion. The length of the resected trachea was 6 cm (fig. ). Bilateral recurrent nerves were macroscopically preserved. We performed a suprahyoid release to mobilize the superior tracheal segment, but these mobilizations were insufficient to perform an anastomosis. Thus, to allow additional mobilization, we performed a clamshell thoracotomy and incised the pericardium 360° around the hilum, dissecting the pulmonary ligament bilaterally. These procedures allowed an anastomosis. An end-to-end bronchial anastomosis was carried out using running 4-0 PDS II sutures (Ethicon, Somerville, N.J., USA) for the membranous portion and interrupted 4-0 PDS II sutures for the cartilaginous portion. The bronchial anastomosis was wrapped with an omental pedicle flap that was lifted up through a retrosternal route via a laparotomy. A gastrostomy was also performed for postoperative enteral nutrition. For management after surgery, a chin suture was placed. Neck mobilization was restricted for the placement of the chin suture and airway fixation. After confirming an improvement in the laryngeal edema using a laryngoscopy, extubation was performed on postoperative day (POD) 17. The patient exhibited swallowing disturbances and underwent swallowing rehabilitation. Enteral nutrition was provided through the gastrostomy. The patient was discharged on POD 31, and per os feeding was started on POD 51. Radiotherapy (60 Gy in 40 daily fractions of 1.5 Gy) was performed as an adjuvant therapy. Throughout the clinical course, no major complications, including swallowing disturbances, hoarseness or anastomotic problems, were observed. A histopathological examination of the resected specimen revealed the characteristic morphology of CASTLE (fig. ); immunohistochemistry was positive for CD5 (fig. ) but negative for thyroid transcription factor 1 (TTF-1; fig. ).\nEleven months after surgery, lower back pain and right leg numbness developed and led to gait inability. Multiple lung and bone recurrences were observed, but no local recurrence. In order to ease the back pain, palliative radiotherapy (35 Gy in 14 fractions) to the bone metastasis was administered. Twelve months after surgery, drainage of pleural effusion was performed. She died of pleural metastasis 14 months after the initial diagnosis of CASTLE. | Patient 1 was a 61-year-old female who presented with a 5-cm anterior neck mass with retrosternal extension. She was surgically treated with a right thyroid lobectomy at another institution. The lesion was interpreted to be a poorly differentiated papillary thyroid cancer. Postoperative treatment consisted of sequential and combined chemoradiotherapy. Local recurrence in the region of the right thyroid occurred 20 months later, and radiotherapy was performed to relieve symptoms. Six years later, the patient had local recurrence again and was referred to our institution for further treatment. After curative wide local excision and central neck dissection, postoperative radiotherapy was given to the thyroid bed and bilateral cervical lymph node areas (55 Gy/16 fractions). Histological re-evaluation of the recurrent tumor specimens revealed CASTLE (pT4bN1M0). A third local recurrence was diagnosed 1 year later, and concurrent chemotherapy (paclitaxel 210 mg/m2 and cisplatin 40 mg/m2) and radiotherapy (50.4 Gy /28 fractions) were applied. The patient died from acute respiratory distress 1 month after the initiation of treatment. | 1 | Write a detailed clinical case vignette based on the following key phrases: Thyroid Cancer, CASTLE (Carcinoma showing Thymus-like Differentiation), Neck Mass |
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