bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pulmonary Epithelioid Haemangioendothelioma Involving Pleura: A Case Report
Authors: Kadam maruthi V 
Published: October 2020

Abstract

Introduction/Objective
Pulmonary epithelioid haemangioendothelioma is a rare, low to intermediate grade tumor of endothelial origin. It is more common in middle aged women. In lung, more than 60 % of the cases present as bilateral multiple perivascular nodules on imaging raising the differential of metastasis or inflammatory cause. WWTR1- CAMTA1 fusion gene is characteristic for this entity. Evidence of pleural involvement carries a poor prognosis.

Methods
Here, we present a case of epithelioid haemangioendothelioma involving pleura in a 52-year-old woman with a long standing history of rheumatoid arthritis. Patient presented with recurrent pleural effusions and shortness of breath.

Results
A diagnosis of epithelioid haemangioendothelioma was made on her pleural decortication specimen that showed atypical vascular proliferation and confirmed by immunohistochemical staining. Subsequent right lung wedge resection performed later confirmed the presence of epithelioid haemangioendothelioma in lung parenchyma.

Conclusion
Pathologists should be aware of this rare condition that can clinically present in a very deceptive manner of recurrent effusion.
Submandibular Swelling: Initial Presentation of Metastatic Neuroblastoma
Authors: Ullah A , Mattox S N , Sliker T , Willhite D , Patel N , Doroodchi A , Hatley R , Velasquez Zarate L 
Published: October 2020

Abstract
Casestudy: Neuroblastoma is the most common solid tumor in the pediatric population. It is derived from neural crest cells and can arise from anywhere along the sympathetic chain. Fifty-to-sixty percent of patients with neuroblastoma present with disseminated disease. However, neuroblastoma rarely presents with metastasis to the submandibular gland or head and neck region. We present a case of a 3-year-old male with a one-month history of left submandibular swelling, lower back pain, fever, decreased urine output and constipation. An abdominal ultrasound demonstrated hydronephrosis. The ultrasound of the left mandible showed an echogenic mass with prominent Doppler flow. An excisional biopsy was performed on the left submandibular mass revealing small round blue cells with syncytial pattern, scant pink cytoplasm, and dark blue nuclei. Tumor cells were strongly positive for synaptophysin immunohistochemical stain. His urine vanillylmandelic acid and homovanillic acid were 121.7 and 79.3, respectively. Subsequent imaging revealed multifocal metastatic lesions involving the cervical, thoracic and lumbar vertebrae as well as the sacrum and bilateral iliac bones and posterior epidural soft tissue on MRI. While, CT scan demonstrated a right adrenal calcified mass that was presumed to be the primary malignancy site for his metastatic neuroblastoma. He was treated with chemotherapy and follow up was recommended. Although an initial presentation of neuroblastoma as a sub-mandibular mass is rare, diagnostic workup by way of radiography, bone marrow biopsy, and laboratory investigations is crucial in the accurate diagnosis, staging, and treatment of metastatic neuroblastoma.
A Rare Case Of CNS High-Grade Neuroepithelial Tumor With BCOR Alteration (HGNET-BCOR)
Authors: Al-Habib A , Bhattacharjee M 
Published: October 2020

Abstract

Introduction/Objective
HGNET-BCOR is a distinct molecular CNS tumor entity that was first identified by DNA methylation analysis in 2016. It is defined by an in-frame internal tandem duplication in exon 15 of the BCOR gene.
The BCOR mutation is not unique to this tumor entity and can be seen in other CNS tumors, as well as solid organ and hematologic malignancies. In this report, we describe the pathology in a 2-year-old girl with a posterior fossa brain tumor initially diagnosed as high-grade neuropeithelial tumor at an outside facility.

Methods
She underwent resection and adjuvant chemotherapy at the same institution. She presented later to our hospital complaining of fatigue and vomiting, and tumor recurrence on MRI. Microscopic examination of the recurrent tumor showed a relatively circumscribed lesion with atrophic cerebellar folia associated with some of the tumor fragments. Tumor cells were arranged in papillary, pseudopapillary, and rosetted patterns in a myxoid (Alcian-blue positive) background. The tumor showed regional necrosis and frequent mitoses (mitotic index ~14–15 /10 HPFs). An extensive panel of immunostains was performed.

Results
The tumor was diffusely and strongly positive only for vimentin and EGFR, with diffuse moderate staining for BCL-2, and patchy weak staining for S100 protein. There were rare, focal tumor cells with GFAP and neurofilament staining. The Ki67 labeling index was 80–90%. All other tested markers were negative. The primary tumor resection was later found to be positive for BCOR by IHC. Sequencing for exon 15 of the BCOR gene showed an in-frame internal tandem duplication.

Conclusion
These features are consistent with the diagnosis of HGNET-BCOR. The pathologic and clinical features for this rare tumor entity are not widely known. Further studies with additional patients are needed to expand our understanding of these rare tumors to develop new treatment approaches.
Massive Perivillous Fibrin Deposition and Chronic Histiocytic Intervillositis of a Placenta: Rare co-existence
Authors: Ozluk E , Cotelingam J , Ong M 
Published: October 2020

Abstract

Introduction/Objective
Massive perivillous fibrin deposition (MPFD) and chronic histiocytic intervillositis (CHI) of the placenta are both very rare entities individually. They both are associated with reccurent loss of the fetus.

Methods
Here we report a placenta with coexisting MPFD and CHI. Patient is a 24-year-old African American female who has a history of spontaneous abortion at the 10th week when she was 18. Her latest pregnancy is complicated by intrauterine growth retardation and she eventually losses the fetus at 20th week of gestation.

Results
Histopathology reveals extensive fibrin deposition of the perivillous area and the histiocytic infiltration of the intervillous spaces which is highlighted by CD68 staining.

Conclusion
This is the second literature report of a co-occurrence of MPFD and CHI to our knowledge. Pathologists must be familiar with these entities and inform the obstetricians because these placental lesions often result with recurrent loss of fetus.
An Early Hands-On Introduction To Grossing And Frozen Section Technique For First-Year Residents
Authors: Owczarczyk A B , Toth N , Skala S L 
Published: October 2020

Abstract

Introduction/Objective
First-year pathology residents begin their training with variable experience in anatomic pathology. At the University of Michigan, we have recently instated an introductory rotation (“Introduction to Surgical Pathology” [ISP]) all new residents experience prior to their core surgical pathology rotations.

Methods
For the first two weeks of ISP, residents learn to gross under the supervision of certified Pathologists’ Assistants. Residents gross biopsies to gain familiarity with dictation and grossing software prior to learning how to approach larger resections. Specific feedback about the content and organization of gross descriptions is provided by the rotation director, and residents are encouraged to review slides from complex specimens they grossed. After orientation, including a lab tour and simulated frozen sections, residents spend two weeks on-service learning to triage specimens (including breast resections and lymphoma work-ups), prepare and cut frozen sections, and participate in frozen reporting. We analyzed the effect of the experience on 8 residents with a 14-question (multiple choice and short answer) pre- and post-rotation quiz and through review of anonymous feedback.

Results
Overall, 7/8 residents improved on the post quiz, while one resident’s performance slightly decreased. Average scores improved by 5.1 (of 36) points or 14.2%. Scores markedly improved on questions related to cold ischemia time, lymphoma protocol, and frozen section documentation (1–4 residents correct pre- to all correct post- rotation). Residents had difficulty answering questions related to appropriate frozen section requests and mandatory specimen photography, but reported feeling somewhat confident about their ability to gross and to prepare frozen sections. Feedback revealed an appreciation of rotation organization, clear expectations, frozen section simulation, and rotation director availability for feedback and questions.

Conclusion
Overall, ISP is a subjectively and objectively valuable rotation. The quiz results and resident feedback have highlighted areas for future improvement, including more effective education about the role of frozen section in surgical management.
Objective Histologic Characterization of Trophoblastic Proliferations
Authors: Perez C , Worrell H , Lieberman R , Skala S L 
Published: October 2020

Abstract

Introduction/Objective
Placental site nodules (PSNs) are rare intermediate trophoblastic lesions characterized by hyalinized cellular aggregates that are small, well-circumscribed, non-necrotic, and paucicellular. PSNs are generally incidental in gynecologic specimens. ETTs are malignant proliferations of intermediate trophoblasts and are generally larger, with nuclear atypia, and higher mitotic rate. Atypical placental site nodules (APSNs) are histologically intermediate between PSN and ETT and not well characterized in the literature to date. There exists room for more objective characterization of PSN and APSN. We use objective measurements to characterize trophoblastic proliferations based on size, location, mitotic rate, number of trophoblasts per high-power field (t-HPF), ki-67 index, beta-hCG, time since last pregnancy, presence of calcification or necrosis, and clinical follow-up.

Methods
The surgical pathology database of a single large academic institution was searched for cases of “placental site nodule,” “atypical placental site nodule,” “epithelioid trophoblastic tumor,” and “placental site trophoblastic tumor”. Clinical and morphologic data were recorded for each case. Ki-67/cytokeratin AE1/AE3 dual immunohistochemical stains were performed to assess the proliferative index of the trophoblastic cells.

Results
70 cases of trophoblastic lesions (53 PSN, 4 APSN, 5 ETT, 8 PSTT) were identified. Results include: PSNs were on average 3.2 mm, 56.5 t-HPF, and 2.6% ki-67. APSNs 4.3 mm, 97.8 t-HPF, and 6.4% ki-67. ETTs 16.5 mm, 161.8 t-HPF, and 12.2% ki-67.

Conclusion
Objective measurements of trophoblastic lesions, as well as correlation with clinical data, may be useful for more accurate classification of these lesions, especially by those who encounter them rarely. To date, the clinical risk, optimal management, and risk for progression of APSNs are not well characterized and thus are worthy of additional study and description. For example, in our data, 14 cases originally signed out at PSN were at least 4 mm, which may re-classify them as APSN.
Aberrant Diffuse Intense CD10 Expression In P16 Positive Undifferentiated Endometrial Carcinoma: A Diagnostic Dilemma
Authors: Derakhshandeh R , Kallakury B 
Published: October 2020

Abstract

Introduction/Objective
High-grade endometrial neoplasms frequently pose a diagnostic challenge on purely histologic evaluation due to their ambiguous morphologic features. High grade endometrioid adenocarcinoma, serous carcinoma, undifferentiated carcinoma, and a minority of sarcoma cases can present with a solid growth pattern.

Methods
We present a 59-year-old patient who underwent a hysterectomy and bilateral salpingo-oophorectomy for a biopsy proven FIGO grade 2 endometrial carcinoma.

Results
Grossly, the entire endometrial cavity was involved by a hemorrhagic tumor. Microscopic examination showed the entire specimen to be replaced by predominantly non-cohesive diffuse sheets of mitotically active, pleomorphic cells without any glandular, papillary, serous or clear cell features.The differential diagnosis included undifferentiated carcinoma, serous carcinoma, carcinosarcoma, stromal sarcoma and other tumors including high- grade lymphoma. By immunohistochemistry, the tumor cells showed variable positivity for CK7, EMA, CK19, CK18, CK8, AE1/AE3, pan-keratin, CAM5.2, CD56, synaptophysin, p53, cyclinD1 and with a high Ki-67 proliferation index of 80%. The tumor was diffusely/intensely positive for CD10 and P16 while being negative for ER, PR, PAX8, HPV, mesenchymal and lymphoid markers.
While diffuse/intense CD10 positivity and ER/PR negativity raised a concern for stromal sarcoma component, the above phenotype confirmed an aggressive/high grade carcinoma. Diffuse/intense p53 and p16 expression raised a consideration of serous carcinoma, but morphology and absence of PAX-8 failed to support this diagnosis.
Undifferentiated carcinoma of the endometrium is a high-grade carcinoma which has been recognized as a distinct entity with diffuse p16 and p53 positivity in the absence of PAX-8 and hormone receptors. Positive vimentin, along with a negative HPV, confirmed the endometrial rather than cervical origin of this neoplasm.

Conclusion
By reporting this case, we draw attention to the previously unreported diffuse intense CD10 expression in undifferentiated carcinoma of endometrium to avoid misdiagnosis with considerations that include stromal sarcoma.
Myeloid Sarcoma Involving the Central Nervous System Without Evidence of Preexisting Hematologic Disease: A Case Report
Authors: Marshall C , Sandhu A D , Mohanty A 
Published: October 2020

Abstract

Introduction/Objective
Myeloid sarcoma within the central nervous system is a rare presentation of an infiltrative mass of immature myeloid cells. It is typically found in association with myeloid dysplastic syndrome, myeloproliferative disorder, or acute myeloid leukemia. Rarely, it can occur in the absence of these diseases.

Methods
We present the case of 68-year-old female with worsening nausea and headaches. Brain MRI showed diffuse pachymeningeal enhancement with numerous nodular enhancing extra-axial masses overlying both cerebral hemispheres.

Results
Open biopsy of these masses showed gelatinous, hemorrhagic, brown tissue measuring 3.0 x 2.0 x 0.7 cm. Histologically, the specimen shows a diffuse polymorphous atypical myeloid proliferation consisting of all three cell lineages. There are nodules composed predominantly of large blastoid cells with 1 to 2 prominent oblong nucleoli, consistent with erythroblasts and early erythroid precursors. In between the nodules there are myeloid cells in different stages of maturation, including numerous eosinophilic precursors. Scattered megakaryocytes are also identified.
Immunohistochemistry shows positivity for E-cadherin, CD235a and spectrin which highlight the immature erythroid cells. MPO, muramidase, and CD33 are positive in myeloid elements. Eosinophilic peroxidase highlights numerous eosinophilic precursors. CD34 is positive in the endothelial cells and very rare hematopoietic cells. CD20 and PAX 5 highlights B-cells, CD3 highlights T-cells, and CD4 highlights monocytic precursors, monocytes, histiocytes and a subset of T-cells.

Conclusion
Additional work up at the time of biopsy including FLOW cytometry and bone marrow biopsies showed no evidence of hematologic pathology. In summary the histologic and immunohistochemical findings demonstrate a tumor composed of proliferating immature myeloid cells most consistent with myeloid sarcoma without any evidence of hematological disease.
Key Differences in Final Pathology Compared to Pre-Operative Clinical Diagnoses in the Liver Transplant Population: Implications for Quality Improvement
Authors: Attaway C C , Furth E E , Tondon R , Fortuna D , Talwar A 
Published: October 2020

Abstract

Introduction/Objective
The pre-transplant clinical evaluation of patients with liver disease are critical components determining prognoses, eligibility and placement on the transplant waiting (eg. hepatocellular carcinoma (HCC) may decrease time on list), and surgical planning for the type of biliary anastomosis performed. For example, patients with Primary Sclerosing Cholangitis (PSC) are given direct liver to small bowel anastomosis, a type prone to ascending cholangitis, compared to duct to duct types in all other etiologies, and a clinical diagnoses of alcohol-induced cirrhosis impacts the patient’s and family’s psycho-social well-being. The goal of our study was to determine the frequency and types of differences in the clinical versus liver explant pathology diagnoses.

Methods
A search for “liver explant” for all in-house cases between 7/15/2019 to 7/15/2018 and 1/1/2010 to 12/31/2010 identified 105 and 108 cases, respectively. For each case, pre-operative clinical diagnoses determined by the electronic medical record was compared to pathologic diagnosis. Cases with non-concordance were divided into two groups: 1. Concordant etiology but incidental malignancy identified in explant 2. Non-concordant etiology.

Results
In the 2018–2019 group, of the 105 liver explants, 6 had concordant etiology but previously unknown HCC and 9 cases had non-concordant diagnoses. Similarly, the 2010 group, of the 108 liver explants, 6 had consistent etiology but previously unknown HCC and 8 cases non-concordant diagnoses. Of the total 17 non-concordant cases, 4 carried a pre-transplant diagnosis of PSC and were reversed to autoimmune hepatitis (2), chronic rejection, and primary biliary cholangitis on explant pathology and 3 carried a diagnosis of alcohol-induced cirrhosis and were reversed to portal tract venopathy, viral hepatitis, and alpha-1 antitrypsin.

Conclusion
There are clinically significant discordances between pre-transplant and explant diagnoses with 6% incidental malignancies and 8% etiology changes. Of particular importance for anastomoses complications and psycho-social issues are patients with a pre-operative diagnoses of PSC or alcohol whose diagnosis was changed by pathology explant assessment, respectfully. Strategies to refine and improve pre-operative liver disease diagnoses particularly with regard to biliary and alcohol diagnoses will be explored.
Mixed Acinar-Neuroendocrine Carcinoma of the Stomach: A Case Report and Review of the Literature
Authors: Coppola D , Hough B , Saller J 
Published: October 2020

Abstract

Introduction/Objective
Mixed Acinar Neuroendocrine Carcinoma (MACNEC) is rare and usually occurrs in the head of the pancreas. This is an epithelial neoplasm of the pancreas exhibiting both acinar and neuroendocrine differentiation. Each component must represent at least 30% of the tumor. Extrapancreatic acinar cell carcinoma arising in the stomach are also rare and represent &lt;1% of all gastric neoplasms. Here we report a case of MACNEC arising in the stomach of a 60 yo female and we review the literature.

Methods
The specimen was formalin-fixed and paraffin embedded. 4-microns sections of the tumor block were stained for hematoxylin and eosin and immunostained for CAM5.2, Chromogranin, synaptophysin, trypsin, chymotrypsin, BCL10, HepPar1 and AFP using a Ventana Discovery XT automated system (Ventana Medical Systems, Tucson, Ariz) as per manufacturer’s protocol with proprietary reagents.

Results
The patient presented with heartburn and weight loss. An upper endoscopy revealed an ulcer along the stomach greater curvature. Endoscopic ultrasound revelaed an hypoechoic round mass involving the submucosa. Imaging showed no lesions in the pancreas. A stomach wedge resection was performed: a 1.2 x 0.6 x 0.5 cm. submucosal tan-brown mass was found. Microscopically the tumor had alternated solid/acinar areas with cords/ribbons neuroendocrine-like areas. Both components were immunohistochemically positive for keratin (CAM 5.2), chromogranin (diffuse), and synaptophysin (patchy). The trypsin, chymotrypsin and BCL10 immunostains were patchy and stained 30 to 40% of the tumor. The immunostains for HepPar-1 and AFP were negative. These results support the diagnosis of MACNEC. Pancreatic heterotopia was not identified in the tissue examined. The margins of resection were free of tumor. The tumor was staged as T1NXMX. The patient has been under close surveillance for 3 years and she has no radiographic evidence of tumor recurrence or metastases.

Conclusion
We found only 6 prior cases of MACNEC originating in the stomach. They had similar histopathologic and immunohistochemical features. In the absence of a pancreatic lesions, in this patient the tumor may have originated from ectopic pancreatic tissue or from a pluripotent stem cell in the stomach.
Partial Nephrectomy For a Presumed Single Renal Mass Revealing Multiple Tumor Histologies: A Series of 4 Patients
Authors: Arora S , Rogers C G , Arora K , Abou Shaar R , Kezlarian B , Modi M , Gupta N , Williamson S R 
Published: October 2020

Abstract

Introduction/Objective
Renal mass biopsy is known to have a low but unavoidable diagnostic error rate. However, the occurrence of multiple adjacent masses mimicking one mass clinically has been minimally studied.

Methods
We report a series of four patients who were radiologically presumed to have a single renal mass and treated with partial nephrectomy, yet who were found to have multiple demarcated renal cell carcinoma histologies at pathologic evaluation.

Results
All were men aged 63–70 years. Grossly, tumors were red brown with scant, bright yellow foci in one of them. Dominant tumors followed by smaller tumors were: patient 1 - clear cell renal cell carcinoma (5.0 cm), clear cell papillary renal cell carcinoma (0.5 cm), and papillary adenoma (0.6 cm); patient 2 - clear cell renal cell carcinoma (1.5 cm) and clear cell papillary renal cell carcinoma (0.5 cm); patient 3 - papillary renal cell carcinoma (5.0 cm) and eosinophilic variant of chromophobe renal cell carcinoma (1.0 cm); patient 4 - chromophobe renal cell carcinoma (4.0 cm) and clear cell papillary renal cell carcinoma (0.6 cm). Immunohistochemical studies for cytokeratin 7, carbonic anhydrase IX, high molecular weight cytokeratin, CD10, and alpha-methyl acyl-CoA racemase (AMACR) confirmed the separate components in all.

Conclusion
This series adds to the spectrum of causes that may contribute to discordant results of renal mass biopsy and resection specimens. Secondary smaller tumors appear to be predominantly nonaggressive histologies, enriched for clear cell papillary renal cell carcinoma. Pathologists and urologists should be aware of this occurrence when considering the role of renal mass biopsy and interpreting the results.