bluuebunny/crossref_metadata_2025 huggingface dataset:
Metastatic Mullerian adenocarcinoma involving the pancreas initially misdiagnosed as an intraductal papillary mucinous neoplasm (IPMN): A case report, and lessons learned
Authors: Liang G , Saeed O A , Cramer H M
Published: November 2023

Introduction/Objective

Metastases to the pancreas from Mullerian primaries are rare and can be confused with primary pancreatic malignancies.

Methods/Case Report

A 49-year-old woman presented with right lower abdominal pain. She denied any prior history of malignancy but had undergone hysterectomy and bilateral salpingo-oophorectomy for unknown reasons. CT scan revealed an 8.9 cm predominantly cystic mass in the pancreas, highly suspicious of an invasive mucinous neoplasm. EUS-guided FNA of the pancreas showed large, complex papillary fronds of mucinous epithelial cells in a background of abundant extracellular mucin, findings that were interpreted as an intraductal papillary mucinous neoplasm (IPMN) with foci of high-grade dysplasia and possible adenocarcinoma. A subsequent PET/CT scan demonstrated a 3.9 cm hepatic mass and a 3.8 cm perihepatic lesion. FNA of the liver was interpreted as metastatic pancreatic adenocarcinoma. Despite aggressive treatment, her disease progressed. Molecular profiling of her liver tumor demonstrated a pathogenic KRAS G12D mutation and a PDGFRA variant of uncertain significance. Genomic (DNA) and transcriptomic (RNA) analysis indicated a 96% probability that the patient had ovarian or fallopian tube adenocarcinoma and a 4% probability of uterine endometrial adenocarcinoma rather than metastatic pancreatic adenocarcinoma. A panel of immunocytochemical stains was performed on the pancreas and liver FNAs. The tumor cells were strongly positive for CK7, CK19, ER, and WT1, focally positive for PAX8 (nuclear) and P53 (wild-type), and negative for CK20, CDX2, and SATB2.

Results (if a Case Study enter NA)

NA

Conclusion

These findings corroborated the molecular analysis suggesting that the patient had metastatic Mullerian adenocarcinoma rather than metastatic pancreatic adenocarcinoma. Her oncologic treatment was adjusted accordingly.
Retrieving single-cells and cell clusters from cytology direct smear slides for genomic evaluation
Authors: Zhu Y , Lowe A C , Aragon A , Gonzalez-Pena V , Gawad C
Published: November 2023

Introduction/Objective

The key role of cytopathologists is to generate diagnoses based on cytologically stained samples. However, diagnoses increasingly need genomic data in addition to morphologic information. By current methods, morphologically diagnostic samples may be inadequate to perform comprehensive ancillary testing.

Therefore, there is a great need for a workflow that can reliably identify and retrieve target cells based on their morphological features. This workflow should also enable highly sensitive molecular profiling from individual cells or small clusters of cells, allowing accurate detection of genomic alterations like copy number variations (CNVs) and single nucleotide variations (SNVs).

Methods/Case Report

Previously, we established a workflow that combines the RareCyte (Seattle, WA) cell-retrieval platform with primary template-directed amplification (PTA) to generate whole genome libraries from Propidium iodide (PI) stained effusion tumor single-cells or cell clusters and detected copy number variation. In this study, we modified and expanded the assay to identify and retrieve malignant or suspicious cells from Diff-Quik direct smear slides from patient pleural effusion specimens. The samples were further subjected to PTA amplification and library preparation for future copy number variation (CNV) evaluation. In parallel, patient cells from PI-stained smear slides were also obtained and processed to serve as controls.

Results (if a Case Study enter NA)

Direct smear slides were made from patient pleural fluid (N=5). In contrast to the previous PI staining, which only highlighted nuclei under fluorescence microscopy, Diff-Quik staining allowed nuclear and cytoplasmic morphology evaluation to identify target cells via light microscope. The XYZ coordinates of each target cell or cluster were further documented in the RareCyte Imager software to facilitate cell retrieval. We have successfully collected 39 single-cell and 18 bulk-cell samples from 5 different cases. PTA amplification has been completed and DNA yields of amplified libraries are promising.

Conclusion

We demonstrated that our modified cell retrieval platform can reliably obtain both single-cell or cluster samples based on morphology from cytologic smear slides made from patient effusion specimens. These collected samples were shown to be compatible with downstream amplification and library preparation enabling CNV evaluation and future deep sequencing for diagnostic, prognostic, and predictive pathogenic mutation identification.
A RARE PRESENTATION OF SMALL CELL LUNG CARCINOMA METASTASIZING TO PHEOCHROMOCYTOMA
Authors: Asad M , Sieber S C
Published: November 2023

Introduction/Objective

Tumor-to-tumor metastasis is a rare phenomenon in which primary tumor cells metastasize hematogenously into another unrelated tumor. We report an interesting case of a tumor-to-tumor metastasis of small cell lung carcinoma to pheochromocytoma of the adrenal gland.

Methods/Case Report

A 75-year-old female former cigarette smoker with a past medical history of hypertension presented with a two-day history of dyspnea and epigastric pain. Physical examination demonstrated elevated blood pressure only. A computerized axial tomography scan of the chest and abdomen showed an incidental 3.1 cm right adrenal mass and right hilar lymphadenopathy. Detailed biochemical workup showed elevated serum epinephrine, norepinephrine, and 24-hour urine metanephrine. Adrenalectomy showed a 5.8 cm tumor mass with solid lobulated yellow to pink-tan and focally hemorrhagic cut surfaces. Microscopic examination showed a neoplasm composed of two distinct components. The predominant component consisted of nests ("zellballen") of large polygonal cells with variable nuclear pleomorphism, abundant amphophilic to basophilic cytoplasm, and a delicate intervening fibrovascular stroma, without significant mitotic activity. The minor component was characterized by sheets and nests of "small blue cells" with round to ovoid nuclei, stippled nuclear chromatin pattern, scanty cytoplasm, patchy necrosis, and high mitotic activity (>20 mitoses per 10 HPF). Foci of small cell carcinoma deposits were also present in the lymphatic channels of pheochromocytoma. Immunohistochemical stains demonstrated cytoplasmic staining of predominant component tumor cells for synaptophysin, chromogranin, and the absence of staining for TTF-1, CK CAM 5.2, and Ki-67. However, minor component tumor cells demonstrated cytoplasmic staining for synaptophysin, membranous staining for CK CAM 5.2, and nuclear staining for TTF-1 and Ki-67(60%). A final diagnosis of composite pheochromocytoma (predominant) and small cell neuroendocrine carcinoma (minor) was rendered. The possibility of a” collision” tumor comprising primary small cell carcinoma of pulmonary origin metastatic to an adrenal pheochromocytoma in light of the patient's cigarette smoking history and the presence of right hilar lymphadenopathy was favored. The subsequent biopsy of the right subcarinal adenopathy showed small cell carcinoma.

Results (if a Case Study enter NA)

NA

Conclusion

To the best of our knowledge, this is the only case of metastasis to a pheochromocytoma from a small cell neuroendocrine lung carcinoma.
Extraskeletal Osteosarcoma with Extensive Gastrointestinal Tract Involvement
Authors: Mirzaei Baboli K , Salehiazar S , Jackson D , Rector A R , Mousavi S
Published: November 2023

Introduction/Objective

Extraskeletal osteosarcoma (ESOS) is a rare aggressive mesenchymal tumor that originates from soft tissues or visceral organs, differentiating it from primary bone osteosarcoma. ESOS represents less than 5% of all osteosarcomas and less than 1% of soft tissue sarcomas. Primary ESOS in the gastrointestinal tract (GIT) is exceedingly uncommon, with a low incidence rate

Methods/Case Report

A 70 year-old-man with a history of hypertension, Myasthenia Gravis, hypothyroidism (status post total thyroidectomy), and skin cancer presented to the clinic with a chief complaint of abdominal fullness of 4 months duration. Primary work-up revealed anemia (Hemoglobin 7 g/dl) and peripheral blood smear was suggestive of bone marrow infiltration. Evaluation with upper gastrointestinal endoscopy and ultrasound demonstrated regular mucosal folds of the stomach with a solid-cystic lesion between the body of the pancreas and the posterior aspect of the stomach. The patient underwent a fine needle biopsy showing a spindle cell lesion with positive immunohistochemistry (IHC) for Smooth Muscle Actin (SMA), beta-Catenin, CD34, and negative for panCK, DOG-1, and CD117; suggestive of leiomyoma. The patient underwent a total gastrectomy, distal pancreatectomy, and splenectomy. Examination of the specimen confirmed a high-grade malignant spindle cell lesion with numerous associated osteoclast-like multinucleated giant cells, and focal areas consistent with osteoid changes. The IHC was requested and the cells were positive for WT1 and Muscle Specific Actin, SATB2, and Ki-67>90% whereas the cells were negative for SMA, Myo-D1, Myogenin, S100, SOX10, MART1, CD34, CD31, and Calretinin. Also, the genetic analysis for MDM2 was negative. These findings confirmed the diagnosis of ESOS with abdominal visceral involvement (stomach, pancreas, and omentum)

Results (if a Case Study enter NA)

NA

Conclusion

ESOS is an extremely rare condition that demands a comprehensive evaluation involving clinical, radiological, histopathological, and immunohistochemical analyses to establish an accurate diagnosis. Distinguishing ESOS from primary bone osteosarcoma is vital due to the different treatment strategies. Additionally, ESOS requires a unique therapeutic approach compared to more prevalent gastrointestinal sarcomas like GIST or leiomyosarcoma.

The mainstay of ESOS treatment entails complete surgical resection of the tumor with clear margins, accompanied by adjuvant chemotherapy. Ensuring precise diagnosis is of utmost importance to provide suitable and effective treatment
Clinicopathologic Features of Autoimmune Metaplastic Atrophic Gastritis (AMAG) in an Underserved Community
Authors: Gaddam S , Kisha S , Tawil T , Theisen B , Husain S
Published: November 2023

Introduction/Objective

Autoimmune metaplastic gastritis (AMAG), typically associated with pernicious anemia and subsequent risk of gastric neoplasia, is an entity often underrecognized in routine gastric biopsies and can lead to delay in the diagnosis and management of patients. In this study, we analyze the demographics and clinical features of AMAG in an inner city, underserved population with histopathologic and serologic correlation.

Methods/Case Report

We retrospectively collected data on patients diagnosed with or with probable AMAG over a 29-month period from Jan 2020-May 2022 and correlated the histopathology with demographics and clinical presentation. We retrieved prior biopsies on available cases.

Results (if a Case Study enter NA)

Our Data Demonstrates:

-Almost equal prevalence of AMAG in African American and Caucasian patients, with a higher rate of incidence in older population (68% over the age of 60 years), and in female patients.

-43.5% had antibody testing and only 25% of patients had testing for Gastrin levels, but the diagnosis of AMAG was frequently associated with a positive laboratory finding (78.7% and 74.3%, respectively) when performed.

-57.8% had clinically documented anemia, among those, 88.9% had microcytic or normocytic anemia. Only 11.1% had macrocytic anemia

-A history of prior biopsies demonstrating chronic gastritis was seen in 33% of patients.

Conclusion

Our study demonstrates that patients with AMAG come from diverse backgrounds and frequently lack laboratory findings typically associated with pernicious anemia at the time of diagnosis. As such, pathologists and gastroenterologists may benefit from a lower threshold when considering which patients to evaluate for AMAG.

Complete laboratory evaluations were infrequent, which is a limitation, but this may be secondary to a lack of clinical knowledge regarding appropriate testing and an underserved patient population which lacks good continuity of care.
Unveiling the Unexpected: A Metastatic AFP-Producing Carcinoma of Gastric Origin Masquerading as Hepatocellular Carcinoma on Liver Biopsy
Authors: Aydin H B , Subasi N B , Abdelwahab H , Lee H
Published: November 2023

Introduction/Objective

Alpha-fetoprotein (AFP)-producing gastric carcinoma (AFPGC) is a rare and aggressive subtype of gastric cancer characterized by the production of AFP. It is well known for its morphological heterogeneity, displaying a wide spectrum of histological patterns that can mimic other tumor types including hepatocellular carcinoma (HCC), which is similarly associated with AFP elevation, complicating its identification. We report a case of an AFPGC encountered in a liver biopsy.

Methods/Case Report

A 60-year-old male presented with chronic abdominal pain and unintentional weight loss over the past few months with associated mild constipation and nausea. The patient denied melena or hematochezia. An abdominal CT scan revealed multiple round lesions within the liver, the largest measuring 4.9 cm, without evident primary site. AFP and CEA were elevated at 2883.0 ng/ml and 36.7 ng/ml, respectively. A liver biopsy demonstrated a proliferation of polygonal cells with eosinophilic to clear cytoplasm in a predominantly trabecular and focally acinar/pseudoglandular growth pattern. The tumor cells were positive for Glypican-3, SALL4, CDX-2 and AE1/AE3 while negative for HepPar-1 and Arginase-1. While the tumor's immunoprofile and histomorphology along with elevated serum AFP suggested poorly differentiated HCC, the multifocality of lesions raised a possibility of a metastatic AFPGC. Subsequent upper endoscopic ultrasound prompted by pathology report revealed a large gastric mass and the biopsy showed conventional adenocarcinoma with similar immunoprofile. Colonoscopy showed a cecal polyp consisting of a diffuse proliferation of signet ring cells without a precursor, consistent with metastatic AFPGC.

Results (if a Case Study enter NA)

NA

Conclusion

AFPGC is known for its morphologic heterogeneity and mimicry of other entities. This case highlights the importance of considering metastatic AFPGC when tumors with unusual presentation show SALL4 positivity in the setting of elevated AFP. Hepatoid morphology of AFPGC in the liver can be a diagnostic pitfall especially when the primary site is unknown.
An Exceptional Rarity: Coexisting Amebic Enterocolitis and Metastatic Colon Adenocarcinoma Deceptively Resembling Crohn's Disease Grossly
Authors: Huang H , Salahi W
Published: November 2023

Introduction/Objective

Amebiasis is a parasitic infection that is rare in developed countries but mimic crohn’s disease or colon malignancy. Amoeboma of the large intestine, a subtype of amebiasis, is an even rarer condition that can be challenging to differentiate from colorectal carcinoma. While co-occurrence of these two conditions is exceedingly rare, we present a unique case of severe fulminant necrotizing colitis with perforation, which coexisted with metastatic colon adenocarcinoma.

Methods/Case Report

A 54-year-old female with no travel history, recently diagnosed with sigmoid colon cancer and liver metastasis, presented with acute worsening of her chronic abdominal pain. Upon examination, a sigmoid/rectal mass with local invasion of the bladder and left salpinx, along with liver metastases, was discovered. The patient underwent an emergent exploratory laparotomy, total abdominal colectomy, and end ileostomy due to concerns for perforation.

The colon's mucosa displayed a cobblestone-like appearance with diffusely raised tan areas, erosions, exudate, and intervening brown mucosa (Figure 1). Microscopic examination revealed amebic trophozoites invading submucosa diffusely with ingested RBCs in cytoplasm, small round nucleus with dot-like karyosome (figure 2), involving the colon and attached terminal ileum, with perforations in the terminal ileum and ascending colon. The trophozoites were further confirmed by negative CD68 stain, and strongly PAS stain.

Additionally, metastatic adenocarcinoma (figure 3-5) was found in two lymph nodes, along with tumor deposits in the mesenteric fat, but no primary adenocarcinoma was identified despite extensive search and sampling.

Postoperatively, the patient completed an antibiotic course for Entamoeba, with a smaller lesion near the gallbladder responded, the hepatic lesion remained unchanged, indicating metastatic disease.

Results (if a Case Study enter NA)

NA

Conclusion

Although the colectomy specimen's gross findinds initially suggested Crohn's disease, the overall microscopic features favored amebic colitis due to the presence of numerous amebic trophozoites and the patient's lack of IBD history.This case highlights the potential coexistence of colonic amebiasis and colorectal carcinoma, which can mimic each other. This case serves as a reminder to avoid delayed diagnosis and treatment of ameboma infection in patients with colon malignancy history, even in non-endemic countries like the United States. .
Intracholecystic Tubular Non-Mucinous Neoplasm: A Distinct Entity
Authors: Fernandez-Hazoury D A , Salehiazar S , Kim H , Salahi W
Published: November 2023

Introduction/Objective

Intracholecystic tubular non-mucinous neoplasm (ICTN) of the gallbladder is a pre-invasive high-grade tumor of non-mucinous tubules which is a rare entity. Histologic morphology distinguishes this tumor from other more common gallbladder neoplasms like intracholecystic papillary neoplasm (ICPN) or gallbladder pyloric adenoma.

Methods/Case Report

A 62-year-old female with no significant past medical history was found to have elevated transaminase in routine lab work. Ultrasound incidentally revealed a pedunculated 1.7 cm polyp in the gallbladder, along with numerous smaller and unmeasured polyps. Six months later, a repeat ultrasound revealed multiple echogenic polyps with the largest polyp measuring 2 cm. She had a laparoscopic cholecystectomy. During surgery, the contents of the polyp were iatrogenically expulsed were submitted as a seperate specimen. Grossly, the gallbladder contents consisted of multiple irregular red soft tissue fragments measuring up to 2 cm, and the gallbladder had an attached cystic lymph node and diffuse small yellow polyps along the wall. The gallbladder and node were entirely evaluated microscopically and revealed diffuse cholesterolosis with cholesterol polyps without dysplasia. The gallbladder content revealed a polypoid neoloplasm with cauliflower-like surfaces, back-to-back tubules, and squamous morules. The lining epithelial cells are cuboidal in shape with vesicular pale nuclei with prominent nucleoli and scant cytoplasm. No papillary structures with fibrovascular core were identified. These findings confirmed the diagnosis of ICTN.

Results (if a Case Study enter NA)

NA

Conclusion

ICTN commonly presents as detached sludge mimicking granular material within the gallbladder lumen and can be a “disappearing” polyp. There is a clear distinction between ICTN and ICPN based on histological features. ICTN is characterized exclusively by high-grade features, while ICPN can be either low-grade or high-grade with high risk of invasion. ICTN is characterized by pedunculated polyps with a thin stalk and multilobulated growth, typically in the background of a relatively unremarkable gallbladder with a crowded proliferation of small tubular units without a fibrovascular core. Pyloric gland adenomas usually don’t show dysplasia and are less than 1 cm. It is important to submit any identified content of the gallbladder. Keeping this rare entity in mind as a differential diagnosis is essential for gallbladder neoplasms.
The Utility of Phosphohistone-H3 Immunohistochemistry (PHH3) in Pheochromocytomas
Authors: Canete Portillo S , Theriault A , Rosas F , Magi-Galluzzi C
Published: November 2023

Introduction/Objective

Most pheochromocytomas (PCC) are sporadic; ~40% present as part of familial syndromes and some cases are associated with germline mutations of succinate dehydrogenase (SDH) genes. Few cases are malignant. The Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) is a scoring system to predict behavior that includes evaluation of mitotic count, among other parameters. PHH3 has been used to assess mitosis in breast and glial tumors. The aim of this study was to assess the potential utility of PHH3 in pheochromocytomas.

Methods/Case Report

50 cases of PCC diagnosed between 2016 and 2020 at our institution were included in the study. Clinicopathologic data was recorded. One hematoxylin and eosin (H&E) slide was selected per case and mitotic count was evaluated at 40x high power-fields (HPF). PHH3 was performed on the same block to assess mitoses.

Cells with dark and coarsely clumped staining were considered positive for PHH3. PHH3-positive cells were counted also at 40x HPF. In one case with bilateral pheochromocytomas, the largest tumor was evaluated. Follow-up was available in 39 cases and recorded as no evidence of disease (NED), alive with disease (AWD), or dead of disease (DOD).

Results (if a Case Study enter NA)

21 patients were female, 29 were males. Age ranged from 24 to 95 (mean: 56). Tumor size ranged from 1.1 to 16.0 cm (mean: 5.6 cm). Mean follow-up was 13.6 months (1-32 months range). Mitotic count ranged from 0 to 7 and 0 to 21 per 10 HPFs, on H&E and PHH3, respectively. PHH3 showed higher mitotic count in 33 cases compared to H&E. In 11 cases, mitotic count was equal on H&E and PHH3, and in the remaining cases, mitotic count was higher on H&E than PHH3. In cases with PASS score ≥ 4, 50% and 27% of cases had mitotic count >3/10HPF on PHH3 and H&E, respectively. Follow-up showed two patients AWD and one DOD, all of whom had PASS score ≥ 4, with mitotic count on H&E 7, 1 and 6 and mitotic count on PHH3 8, 4, and 21 respectively.

Conclusion

This is the first study evaluating the use of PHH3 in pheochromocytomas. Overall, mitotic count was higher on PHH3 than on H&E. This indicates PHH3 is a helpful tool in counting mitosis. Since the PASS score is an imperfect system to predict outcome, additional studies are needed to evaluate the use of PHH3 in prognostic scoring systems for PCC.
Prostein expression in human tumors: A tissue microarray study on 19,202 tumors
Authors: Viehweger F , Lennartz M , Blessin N C , Dum D , Burandt E , Sauter G , Simon R , Kluth M , Steurer S , Clauditz T S , Kind S
Published: November 2023

Introduction/Objective

Prostein (P501S), also termed solute carrier family 45 member 3 (SLC45A3) is an androgen regulated protein which is preferentially expressed in prostate epithelial cells. Because of its frequent expression in prostate cancer, prostein was suggested a diagnostic prostate cancer marker.

Methods/Case Report

In order to comprehensively assess the diagnostic utility of prostein immunohistochemistry, a tissue microarray containing 19,202 samples from 152 different tumor types and subtypes as well as 608 samples of 76 different normal tissue types was analyzed by immunohistochemistry.

Results (if a Case Study enter NA)

Prostein immunostaining was typically cytoplasmic, granular and perinuclear and predominated in prostate cancer. Prostein positivity was seen in 96.7% of 419 prostate cancers including 78.3% with a strong staining. In 12,233 extra-prostatic tumors, prostein positivity was observed in 9.8% of cases but only 0.4% had a strong staining. Extra-prostatic prostein positive tumors were 50 different tumor categories, 12 of which included at least one strongly positive case. Extra-prostatic tumors with highest rates of prostein positivity included salivary gland tumors (7.6%-44.4%), neuroendocrine neoplasms (15.8%-44.4%), adenocarcinomas of the gastrointestinal tract (7.3%-14.8%), biliopancreatic adenocarcinomas (3.6%-38.7%), hepatocellular carcinomas (8.1%), and adenocarcinomas of other organs (up to 21%).

Conclusion

In summary, our data provide a comprehensive overview on prostein expression in human cancers. Prostein is a highly sensitive prostate cancer marker occurring in >96% of prostate cancers. Because prostein can also be expressed in various other tumor entities, labeling of a tumor mass as a prostate cancer should not be based on prostein positivity alone.
Retroperitoneal sarcomatoid yolk sac tumor in a chemotherapy-naïve patient with testicular teratoma
Authors: Zhang B , Jia L , Kapur P
Published: November 2023

Introduction/Objective

Sarcomatoid yolk sac tumor (SYST) is a rare histologic subtype of yolk sac tumor. In patients with testicular germ cell tumor, it is most frequently seen at the metastatic sites in the post-chemotherapy setting. SYST is rarely reported in chemotherapy-naïve testicular cancer patients in the existing literature. Herein, we report a rare concurrent retroperitoneal SYST in a chemotherapy-naïve testicular teratoma patient.

Methods/Case Report

A 37-year-old male presented with a 3.4 cm right testicular mass and a radical orchiectomy was performed, which demonstrated a pT1 teratoma with a separate adjacent regression fibrous nodule. His staging computed tomography revealed a heterogeneously enhancing and lobulated retroperitoneal mass measuring 9.6 cm in the greatest dimension. One month later, the mass enlarged to 26.3 cm. Fine needle aspiration biopsy revealed malignant spindle cell neoplasm with myxoid features, which displayed immunoreactivities for AE1/AE3, SALL4 and glypican 3, while was negative for OCT3/4, myogenin, SMA, desmin, S100 and CD34. Based on these findings, it was most compatible with SYST. The subsequent resection specimen showed a mass with soft fleshy to rubbery and friable gelatinous cut surfaces, and thorough tissue sections revealed the same histologic and immunophenotypic features as the prior biopsy. Therefore, a final diagnosis of SYST was rendered. Four cycles of cisplatin, etoposide, and ifosfamide (VIP) were administered, and the patient tolerated it well at his 6-month follow-up.

Results (if a Case Study enter NA)

NA

Conclusion

Here we presented a rare concurrent retroperitoneal SYST in a chemotherapy-naïve male patient who initially presented with a testicular teratoma. Due to its rarity, SYST may cause a diagnostic dilemma, and our case could raise awareness of this uncommon event and potentially expand the tumor spectrum.
Adenomatoid tumor with extensive reactive smooth muscle: a diagnostic dilemma
Authors: Usman A , Cain S , Hamza A , Meyer A R
Published: November 2023

Introduction/Objective

Adenomatoid tumors are slow-growing benign tumors of mesothelial origin. They comprise 30% of all tumors of the paratesticular area. In this location, they commonly involve the epididymis, spermatic cord, ejaculatory duct and rarely testicular parenchyma. Histologically, a spectrum of growth patterns can be seen, such as adenoid, tubular, glandular, solid, or cystic. In some cases, a smooth muscle component can also be present. The purpose of this report is to describe a case of adenomatoid tumor with exuberant smooth muscle component, mimicking leiomyoma.

Methods/Case Report

A 28-year-old male presented with painless left inguinal bulge. He noticed it five years prior, and it had been slowly growing since then. Examination revealed a paratesticular mass which was subsequently excised. Grossly, the tumor was firm with smooth, white-tan cut surfaces. Microscopically, the tumor was well- circumscribed, showing predominance of smooth muscle bundles and rare cuboidal to ovoid cells in tubular growth pattern. In the background there was little intervening stroma. Immunohistochemical stains showed the cuboidal cells to be positive for WT1 and calretinin, confirming the mesothelial origin. Diagnosis of adenomatoid tumor was rendered. In contrast to adenomatoid tumors, leiomyomas are whiter and firmer on gross examination and lack the characteristic tubules or cystic spaces lined by mesothelial cells.

Results (if a Case Study enter NA)

N/A

Conclusion

Exuberant smooth muscle differentiation in adenomatoid tumor can mislead to diagnosis of leiomyoma. Careful inspection for tubules of mesothelial origin and appropriate immunohistochemical stains are key to accurate diagnosis. Pathologists need to be aware of this potential pitfall.
10-year Observation of A Rare Presentation of Pure Fibromyxoid Nephrogenic Adenoma in the Renal Pelvis
Authors: Ren D
Published: November 2023

Introduction/Objective

Pure fibromyxoid NA is an unusual rare variant that typically presents with a well circumscribed bulbous, or tumefacient architecture composed of mitotically inactive bland spindled cells and rare tubular structures within a myxoid or collagenized stroma. Here we report a rare case of pure fibromyxoid NA involving the renal pelvis with over 10-year clinical observation and comparison and correlation of biopsy and resection findings. To the best of our knowledge, this is the first reported case of pure fibromyxoid NA in renal pelvis with close comparison and correlation of biopsy and resection findings with over 10-year clinical manifestation, hoping that knowledge of this long-term rare spindle cell lesion will enhance awareness of pathologists to carefully consider this unusual entity, especially in the biopsy specimen in which typical histologic features of fibromyxoid NA are usually absent, as well as prevent misdiagnosis and overtreatment of a typically benign process.

Methods/Case Report

A retrospective case study of a 37-year-old female with multiple previous biopsy of the nodule in the renal pelvis, and final resection specimen. Biopsies of the mass showed predominantly bland spindle cell lesion with smooth muscle differentiation and scant urothelial epithelial cells, but no tubular structure identified at the biopsy specimen. On the final resection specimen, a urothelium-lining polypoid nodule composing of abundant bland spindle- shaped cells and scattered conventional tubules lined by a single layer of cuboidal epithelial cells in hobnail arrangement that are positive for PAX8 and GATA3 was identified. These morphological and immunohistochemical findings were consistent with the diagnosis of pure fibromyxoid NA.

Results (if a Case Study enter NA)

NA

Conclusion

To the best of our knowledge, this is the first description of a pure fibromyxoid NA of the renal pelvis with more than 10-year clinical observation, as well as comparison and correlation of biopsy and resection findings.Close observation and comparison of the findinigs between biopsy and resection specimen will enhance awareness of pathologists to carefully consider this unusual entity, especially in the biopsy specimen in which typical histologic features of fibromyxoid NA are usually absent, as well as prevent misdiagnosis and overtreatment of a typically benign process.
Perceived Value of Graduate Education in Medical Laboratory Science
Authors: Grimaldi A , Viboud G
Published: November 2023

Introduction/Objective

A current challenge in the field of laboratory medicine is the limited availability of career advancement opportunities. While it is recognized that graduate education in general enhances professional skills and increases the likelihood of promotion, most medical laboratory scientists (MLS) do not pursue a master’s degree (MS). The reasons behind this trend are not clear, and there is a lack of literature on the perceived value of a MS degree in the field. To address this issue, a 25-questions anonymous online questionnaire was conducted among laboratory professionals.

Methods/Case Report

The survey was distributed through ASCLS forum, social media and MLS alumni email lists, which constitutes a non probability convenience sample.

Results (if a Case Study enter NA)

A total of 219 laboratory professionals participated in the study, including 115 in leadership positions and 104 working as medical technologists. Among those in leadership positions, 69% had a master’s degree or higher, compared to only 31% of laboratorians. Regarding the perceived value of graduate education in MLS, 62% of laboratory professionals with an MS degree were of the opinion that it enhances the competitiveness of MLS, whereas only 34% of respondents holding a bachelor's degree shared this viewpoint.

Moreover, a majority of participants without a MS expressed skepticism regarding the superior analytical and soft skills of individuals with master's degrees. Surprisingly, although 87% respondents in leadership positions perceive a master's degree as helpful for career advancement, this sentiment was only shared by 55% of laboratorians.

Interestingly, only half of the respondents with a bachelor's degree expressed their intention to pursue graduate education.

Conclusion

Despite recognition among leaders and professionals with advanced degrees that graduate education improves skills and promotes career growth, many medical technologists fail to perceive its value. Raising awareness about the benefits of pursuing graduate education can be advantageous for laboratory technologists.
A rare case of EBV-positive gray zone lymphoma
Authors: Hanan A , Khan A I , Geetha S , Khan A , Sheikh-Fayyaz S
Published: November 2023

Introduction/Objective

Gray zone lymphoma (GZL) is a rare and novel category of lymphoid neoplasms, first described in 2005. It is derived from B-cells and exhibits characteristics that fall between diffuse large B-cell lymphoma (DLBCL) and classical Hodgkin lymphoma(cHL). Its association with EBV is extremely rare. Here we present one such case

Methods/Case Report

80-year-old female presented with progressive neck swelling and significant weight loss for over a month. It was not associated with fever, pain, or night sweats. Her past medical history was significant for hypertension, cerebrovascular accident, breast carcinoma status post-mastectomy, and non-specific B-cell lymphoma on complete remission for 9 years (post rituximab therapy). Complete blood count and metabolic profile were within normal limits. A PET scan revealed hypermetabolic cervical, supraclavicular, right axillary, and mediastinal lymph nodes, suspicious for lymphoma recurrence. The patient underwent an excisional biopsy of the left cervical lymph node which on microscopy showed lymphoid tissue in a nodular pattern with fibrosis and large areas of necrosis. The well-preserved areas showed lymphohistiocytic proliferation composed of large atypical lymphoid cells with prominent nucleoli. An area with increased eosinophils was also noted. Immunohistochemical staining showed strong positivity for EBER, CD30, MUM1, and OCT2 with dim PAX5 positivity. The cells were negative for CD20 and BOB1.

Cytogenetics and molecular studies did not reveal any rearrangements or mutations. Flow cytometric study was inconclusive due to insufficient cells. A diagnosis of EBV-positive GZL was rendered. She has received brentuximab and her symptoms are improving with regressing lymph node size.

Results (if a Case Study enter NA)

NA

Conclusion

The presence of EBV positivity in GZL further complicates the diagnostic process, as it adds another layer of complexity in determining the appropriate classification and treatment. Our case highlights the importance of comprehensive morphologic phenotypic and cytogenetic investigation to diagnose GZL.
Disseminated histoplasmosis diagnosed on peripheral blood smear: a case report
Authors: Benchbani H , Hamidpour S
Published: November 2023

Introduction/Objective

Histoplasmosis is an infection caused by the inhalation of a dimorphic fungus, Histoplasma capsulatum. H. Capsulatum var capsulatum is the main variety we encounter in the United States and is endemic in the Mississippi river valley. In immunocompetent individuals the infection is usually asymptomatic or presents as flu- like symptoms. Disseminated histoplasmosis is a severe and often fatal opportunistic infection if not treated, usually seen in immunocompromised patients.

Methods/Case Report

We report a case of a 27-year-old female intra-venous drug user with past medical history of untreated HIV diagnosed in 2018, who is being admitted for suspected necrotizing fasciitis of the vulva. On admission, her complete blood count shows pancytopenia with a white blood cell count of 2.9 × 109/L (NR 4.3-10.8 × 109/L), hemoglobin 6.1 g/dL (NR 12-16 g/dL), and a platelet count of 43 × 109/L (NR 150-400 × 109/L). A peripheral blood smear obtained showing rare neutrophils with intracellular organism highly suggestive of histoplasma capsulatum. CT Chest showing diffuse ground glass opacities and a subsequent cytology from bronchoalveolar lavage performed revealed numerous macrophages filled with ovoid organism surrounded by a cytoplasmic halo, GMS stain highlights the rim consistent with histoplasma capsulatum. Patient expired after one week in the ICU for severe acute respiratory distress syndrome, sepsis, histoplasmosis, strep pneumonia and hepatic failure.

Results (if a Case Study enter NA)

NA

Conclusion

Careful examination of Peripheral blood smear along with correct clinical history can aid in early diagnosis of disseminated histoplasmosis.
Solving the mystery of Reed Sternberg like cells in a composite Peripheral T Cell Lymphoma with Epstein Barr Virus and Epstein Barr Virus driven Lymphoproliferative Disorder
Authors: Siddiq M , Shiwlani S , Ibrar W , Sonam F , Hashim M
Published: November 2023

Introduction/Objective

Nodal PTCL with EBV positivity can occasionally contain Reed Sternberg-like cells, and EBV- LPD can mimic Hodgkin lymphoma. Coexistence of these two unusual lymphomas in the same lymph node is very rare. We present a case of a 56-year-old male with Composite EBV+ PTCL NOS and EBV LPD, with positive B-cell gene rearrangement and negative T-cell gene rearrangement presenting a significant diagnostic challenge.

Methods/Case Report

A 56-year-old male with a history of abdominal tuberculosis presented with lethargy, fatigue, and poor appetite. Physical examination revealed generalized lymphadenopathy. CT scan confirmed lymphadenopathy in bilateral axilla, supraclavicular lymph nodes along with lytic lesions on illiac crest. Ill defined splenic lesions raised concerns for metastasis. Cervical lymph node and bone marrow biopsies were performed. Flow cytometry on the lymph node was not done due to tuberculosis history. Flow cytometry on the bone marrow showed an inverted CD4:CD8 ratio. H&E sections of the lymph node revealed a polymorphic lymphoid infiltrate with prominent Reed Sternberg-like cells. Immunohistochemical staining showed CD30-positive and CD45-negative large cells. T-cell and B-cell gene rearrangements were performed. Initially, B-cell gene rearrangements were positive, while T-gamma was negative. Further testing by IHC and morphological evaluation revealed two distinct cell populations, one with CD3, CD5, and CD7 positivity with partial weak positivity for PD1, CD43, and CD8 and the other consisting of B-cells expressing CD30, EBV, MUM1, and partial expression of BCL6 rare CD79a, and partial weak Pax5. Outside molecular testing showed positive T-cell gene rearrangements. The diagnosis of Composite EBV+ Peripheral T Cell Lymphoma, not otherwise specified, with cytotoxic profile along with EBV driven LPD was made. Bone marrow studies confirmed involvement by the same lymphoma.

Results (if a Case Study enter NA)

NA

Conclusion

Identifying EBV-positive Reed Sternberg-like cells in both EBV LPD and PTCL-NOS in a composite lymphoma can be challenging. In the absence of flow cytometry, a composite lymphoma can potentially be misdiagnosed, leading to under-treatment. Repeat testing can be helpful in case of any doubt. Although EBV-positive PTCL with cytotoxic profile and EBV-positive LPD has been reported, the combination of these two lymphomas together is exceedingly rare.
Acute myeloid leukemia with monocytic differentiation (myeloid sarcoma) masquerading as bone-soft tissue malignancy
Authors: Abdo M E , Al-kharouf I , Hamza A , Meyer A R , Kelting S
Published: November 2023

Introduction/Objective

Myeloid sarcoma is an extramedullary solid neoplasm composed of myeloid precursors with or without maturation. This tumor usually occurs simultaneously or following the onset of acute leukemia. Rarely, it can be the first manifestation of acute myeloid leukemia. We report a case of this rare tumor presenting histologically as a bone sarcoma with features of extraskeletal myxoid chondrosarcoma and myoepithelial carcinoma

Methods/Case Report

A 76-year-old female was referred to our institution for evaluation of a right foot mass associated with pain and swelling. MRI showed a 2.4 x 1.5x 1.2 cm soft tissue mass along the lateral margin of proximal and mid diaphysis of first metatarsal. The clinical and radiologic differential diagnosis was primary bone-soft tissue malignancy, or infection. Histologically, the biopsies showed sheets and cords of cells in a myxoid background. Cytologically the cells were plasmacytoid, mononuclear, mitotically active with immature chromatin, prominent nucleoli, and moderate to abundant cytoplasm. This morphologic appearance elicited the differential diagnosis of extraskeletal myxoid chondrosarcoma, myoepithelial carcinoma, and plasmacytoma. An initial panel of immunohistochemical stains showed the lesional cells to be negative for S-100, SOX-10, pancytokeratin, desmin, p63, EMA, CAM5.2, and CD138 but positive for CD43. Additional immunohistochemistry showed diffuse reactivity for CD33, and lysozyme; CD163 was focally positive, and the cells were negative for CD20, CD3, CD30, CD99, MUM1, CD138, and CD34. A diagnosis of extramedullary acute myeloid leukemia with monocytic differentiation (myeloid sarcoma) was rendered. Subsequently, patient underwent bone marrow biopsy which showed involvement by acute myeloid leukemia with monocytic differentiation.

Results (if a Case Study enter NA)

NA

Conclusion

Myeloid sarcoma is a rare tumor and presentation as an isolated metatarsal mass is highly unusual and exceedingly rare. This can mislead clinicians, radiologists, and pathologists. Pathologists need to keep hematopoietic tumors in their differential diagnosis even when the anatomic sites as well as the morphology are not typical of hematopoietic malignancies.
A 47-Year-Old Woman With a Large Unusual Presacral Mass
Authors: Mayorga M R , Guerry-Force M L
Published: November 2023

Introduction/Objective

Tumors developing in the presacral space are rare, having been reported in 1 of every 40,000 admissions. Most presacral tumors are congenital defects arising from vestiges of embryonal tissue like neuroectoderm, notochord, and hindgut. For this reason, a wide array of benign and malignant presentations can be expected. Preoperative diagnosis is often challenging due to the risk of seeding malignant cells and the high risk of infection.

Methods/Case Report

A 47-year-old woman presented to our institution with complaints of abdominal pressure and pelvic heaviness for about a year, dyspareunia, epigastric pain, diarrhea, and early satiety with an unintentional weight loss of 30 lbs. On physical examination, a large firm, tender growth posterior to the rectum was palpated, deviating both the rectum and the vagina. An MRI confirmed a 9.3 cm complex mass. Due to the location of the lesion and some of the patient’s symptoms, malignancy could not be ruled out. The MRI was useful for planning the perineal resection as the mass was located below S3. However, the accuracy of MRI for specific histological tumor types has been reported to be only 28% therefore the true nature of the lesion could not be fully assessed. Complete resection was then recommended and performed. The mass was entered during dissection and copious thick consistency contents were encountered. Histopathology revealed a cyst lined by squamous epithelium and laminated keratin debris with the presence of chronic inflammatory cells compatible with an epidermoid cyst. The patient made an uneventful recovery and was discharged home.

Results (if a Case Study enter NA)

NA

Conclusion

In cases where a presacral mass of cystic nature is found, especially in middle-aged women, epidermoid cysts should be one of the diagnoses to consider. Imaging is a good aid for surgical intervention but has a very limited ability to identify the mass thus making the post-operative histopathologic diagnosis crucial.
“Case of the Week: The Value and Impact of Sharing Pathology Cases Online”
Authors: Crowe C , Buckelew H , Netto G
Published: November 2023

Introduction/Objective

Social media has become a driving force in the field of pathology in a variety of ways, including aiding in recruitment efforts for residents, fellows, and faculty members; promoting both individual practitioners and institutional programs; sparking discussion; creating and growing community; and sharing resources. Large teaching hospitals see an expansive number of cases, some of which are rare or have unusual features. The sharing of unique pathology cases through social media has become a robust online conversation, resulting in connections to pathologists worldwide through shared educational resources, and as examples to potential trainees and faculty of the wide variety of cases the institution sees.

Methods/Case Report

In 2017, under guidance of our department chair, we began posting one case per week to our website and newly created Twitter account. These posts featured recent, in-house cases contributed by faculty and trainees with slide images and de-identified case descriptions. This content began garnering a following as we shared an increasing number of more unusual cases. In 2018, our department added communications team who standardized the weekly cases and worked with resident leaders to organize and plan a schedule to collect cases from department members. The team posts these cases every Monday on the department website and on our Twitter account, alongside a poll for users to vote on their suspected diagnosis. Each Friday, the team posts the final answer with an educational explanation of the case.

Results (if a Case Study enter NA)

NA

Conclusion

Our weekly cases have become the most popular content shared across our department’s social media channels, garnering hundreds of votes on Twitter, and now Instagram story polls, every week. Building on its popularity, in 2022 we added a second case of the week, specific to cytopathology, shared each Wednesday. We archive the cases on our website, and they are being accessed as a learning tool.