Malignant perivascular epithelioid cell tumor of the oropharynx with strong TFE3 expression mimicking alveolar soft part sarcoma: a case report and review of the literature
| Autor: | Adel K. El-Naggar, Songlin Zhang, Diana Bell, Kunal S. Jain, Karan Saluja, Jaiyeola Thomas, Victor G. Prieto, Erich M. Sturgis |
|---|---|
| Rok vydání: | 2018 |
| Předmět: |
Adult
0301 basic medicine Pathology medicine.medical_specialty Perivascular Epithelioid Cell Neoplasms TFE3 Perivascular Epithelioid Cell Pathology and Forensic Medicine Diagnosis Differential 03 medical and health sciences 0302 clinical medicine Predictive Value of Tests Alveolar soft part sarcoma Biomarkers Tumor Humans Medicine In Situ Hybridization Fluorescence Gene Rearrangement Basic Helix-Loop-Helix Leucine Zipper Transcription Factors business.industry Gene rearrangement medicine.disease Immunohistochemistry Oropharyngeal Neoplasms Sarcoma Alveolar Soft Part 030104 developmental biology Oropharyngeal Neoplasm 030220 oncology & carcinogenesis Female Differential diagnosis Tomography X-Ray Computed business |
| Zdroj: | Human Pathology. 76:149-155 |
| ISSN: | 0046-8177 |
| DOI: | 10.1016/j.humpath.2017.11.016 |
| Popis: | Perivascular epithelioid cell tumors (PEComas) in the head and neck region are rare, with 26 cases described in literature. These distinct mesenchymal tumors normally express both myoid and melanocytic markers. We here report an interesting and challenging case of malignant PEComa that showed transcription factor E3 (TFE3) protein expression and rearrangement, paucity of muscle and melanocytic marker expression, and morphologically mimicked alveolar soft part sarcoma. Awareness of this morphologic pitfall and recognition of TFE3 gene-rearranged PEComa, as a distinct subtype of PEComa, is essential to avoid misdiagnosis. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full Text from ScienceDirect