Primary Sclerosing Epithelioid Fibrosarcoma of the Kidney: A Case Report and Review of the Literature

2021 ◽  
pp. 106689692110651
Author(s):  
Yan Peng ◽  
Tongbing Chen ◽  
Dachuan Zhang ◽  
Chao Wu ◽  
Ting Lei ◽  
...  
Keyword(s):  
Clear Cell Sarcoma ◽  
Epithelioid Cell ◽  
Fish Analysis ◽  
Immunohistochemical Expression ◽  
Neoplastic Cells ◽  
Sclerosing Epithelioid Fibrosarcoma ◽  
Perivascular Epithelioid Cell ◽  
Ewsr1 Gene ◽  
Fibrous Tumor

Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of fibrosarcoma. We report one case of primary kidney SEF occurring in a 38-year-old man. Microscopically, epithelioid neoplastic cells are mainly arranged in cords and nests embedded in the dense sclerosing stroma. Diffuse immunohistochemical staining for MUC4 in neoplastic cells and the presence of the EWSR1 gene split by fluorescence in situ hybridization (FISH) analysis confirmed the histological diagnosis. Primary kidney SEF is extremely rare, the differential diagnosis strategy broadly includes a series of tumors with epithelioid morphology and sclerosing matrix, mainly including sclerosing variants of clear cell sarcoma of the kidney (CCSK), renal synovial sarcoma (SS), renal solitary fibrous tumor (SFT), metanephric stromal tumor (MST), sclerosing perivascular epithelioid cell tumor (PEComa), and carcinomas, and immunohistochemical expression of MUC4 and evidence of the EWSR1 gene split are helpful in making a definite diagnosis.

scholarly journals The clinicopathological features of epithelioid undifferentiated sarcoma with TFE3 amplification: one case report and literature review

2020 ◽  
Author(s):  
Yuejiao Lang ◽  
Xiaojuan Li ◽  
Shaoyu Chen ◽  
Pei Xiang ◽  
Anjia Han
Keyword(s):  
Epithelioid Cell ◽  
Genetic Alteration ◽  
Lymphocytic Leukemia ◽  
Undifferentiated Sarcoma ◽  
Vascular Growth ◽  
Perivascular Epithelioid Cell ◽  
History Of ◽  
Palpable Nodule ◽  
Mitotic Figures

Abstract Background: Strong nuclear expression of TFE3 protein resulting from gene fusion has been reported in some neoplasms. TFE3 amplification has been proven to be a novel mechanism leading to increased protein level only in several cases of perivascular epithelioid cell tumor and renal cell carcinoma. Such rare genetic alteration might be associated with poor prognosis or aggressive course. Herein, we first reported a case of undifferentiated sarcoma with epithelioid features harboring TFE3 amplification. Case presentation: A 66-year-old woman with a history of chronic lymphocytic leukemia and chemotherapy presented with a 4 cm palpable nodule in the left lower leg. Magnetic resonance imaging revealed an oval juxtacortical lesion to the anterolateral left tibia. Microscopically, the large epithelioid cells with marked pleomorphism and the small round cells intermingled with each other in a diffuse sheet or a hemangiopericytoma-like vascular growth pattern. Myxoid stromal change was evident focally, imparting a hypocellular appearance. Atypical mitotic figures and lymph node metastasis were identified while tumor necrosis was absent. Immunohistochemically, the tumor was positive for vimentin, TFE3, CD68 and CD34. TFE3 gene amplification was identified by fluorescence in situ hybridization. Surgical resection was performed. The patient was alive without recurrence 8 month after surgery.Conclusions: The present case might represent a novel entity. Our report expands the scope of tumors carrying TFE3 amplification, and raises more attention to this rare genetic alteration and its association with potential aggressive behavior of the tumor.

Gastric Perivascular Epithelioid Cell Tumor (PEComa)

2019 ◽  
Vol 152 (2) ◽  
pp. 221-229 ◽  
Author(s):  
Jinghong Xu ◽  
Yu Yan ◽  
Xueping Xiang ◽  
Peter Jiang ◽  
Xiangrong Hu ◽  
...  
Keyword(s):  
Gene Rearrangement ◽  
Smooth Muscle Actin ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Perivascular Epithelioid Cell Tumor ◽  
Rare Neoplasm ◽  
Perivascular Epithelioid Cell ◽  
Molecular Features ◽  
First Case

Abstract Objectives To review the clinicopathologic, immunophenotypic, and molecular features of gastric perivascular epithelioid cell tumor (PEComa). Methods We identified two new cases of gastric PEComa and summarized the clinical and pathologic characteristics of this rare neoplasm. Results The first case was a 48-year-old woman who was treated with an endoscopic submucosal dissection (ESD), and the second case was a 64-year-old man who received a distal gastrectomy. Microscopic examination showed one tumor was composed of purely epithelioid cells, while the other was composed of epithelioid and spindle cells. Both tumors were immunoreactive for melanocytic markers (HMB45 and Melan-A), smooth muscle actin, and vimentin. No TFE3 gene rearrangement was identified by fluorescence in situ hybridization in either case. Conclusions Gastric PEComa is an exceedingly rare neoplasm, with only seven other reported cases to date. We are the first to report the results of molecular assays for the TFE3 gene rearrangement associated with gastric PEComa.

scholarly journals CD10-Positive Cutaneous PEComa: An Extremely Rare Skin Tumour

2020 ◽  
Vol 12 (3) ◽  
pp. 192-198
Author(s):  
Simon Ueberschaar ◽  
Matthias Goebeler ◽  
Hermann Kneitz
Keyword(s):  
Clear Cell ◽  
Subcutaneous Tissue ◽  
Epithelioid Cell ◽  
Tumour Cells ◽  
Cell Tumour ◽  
Surrounding Tissue ◽  
Neoplastic Cells ◽  
Cell Renal Cell Carcinoma ◽  
Perivascular Epithelioid Cell ◽  
Clear Cytoplasm

We here present the case of a 67-year-old woman with a history of a slowly progressive, polypous nodule on her left wrist. The lesion was excised, and the histological analysis revealed a clear cell tumour that was relatively sharply demarked from the surrounding tissue extending into the subcutaneous tissue. The tumour showed a characteristic trabecular pattern in which the tumour cells were arranged around numerous vessels. The neoplastic cells had a predominantly epithelioid shape, granular eosinophilic to clear cytoplasm and prominent centrally located nucleoli. The histological differential diagnosis included a metastatic clear-cell renal cell carcinoma and a primary cutaneous perivascular epithelioid cell tumour (PEComa). Immunohistochemically, the tumour cells revealed homogenous expression of HMB-45, MiTF and CD10, whereas MART-1 and S100 were negative. Antibodies against actin marked the trabecularly arranged vessels, and the neoplastic cells yielded a patchy positivity against actin and desmin. Additional immunohistochemical stains against pan-cytokeratin, CAIX, PAX-8 and EMA were negative. Based on the morphologic and immunophenotypic findings, the histological diagnosis of a CD10-positive cutaneous PEComa was made.

Sign in / Sign up

Export Citation Format

Share Document