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- Title
Transcription Factor E3 Protein-Positive Perivascular Epithelioid Cell Tumor of the Appendix Presenting as Acute Appendicitis.
- Authors
Matkowskyj, Kristina A.; Rao, Sambasiva; Raparia, Kirtee
- Abstract
N Perivascular epithelioid cell tumors (PEComas) are a group of mesenchymal tumors that coexpress melanocytic and smooth muscle markers; their exact origin remains unknown. This group includes renal angiomyolipoma, clear cell sugar tumor, and lymphangioleiomyomatosis, although the term perivascular epithelioid cell tumors is currently used for lesions that exhibit a similar morphologic andimmunohistochemical profile throughout the human body.Recently, a distinct subset of PEComas has been shown to harbor transcription factor E3 gene (TFE3) fusions. We report, for the first time, a unique case of TFE3-positive PEComa presenting as acute appendicitis in a 24-year-old woman. Microscopically, the tumor was composed of benign-appearing epithelioid cells with clear and eosinophiliccytoplasm, and arranged in nested and alveolar patterns. Immunohistochemical studies showed diffuse strong positivity for neuron-specific enolase, TFE3, and progesterone receptor and focal strong positivity for human melanoma black-45 (HMB-45) and melanocytedifferentiation antigen (Melan-A) in the tumor cells.Although rare, PEComa should be included in the differential diagnosis of mesenchymal tumors of the appendix.(Arch Pathol Lab Med. 2013;137:434-437; doi: 10.5858/arpa.2012-0103-CR)
- Subjects
APPENDICITIS; APPENDIX (Anatomy); DIFFERENTIAL diagnosis; GENES; IMMUNOHISTOCHEMISTRY; CONNECTIVE tissue tumors; GENETICS
- Publication
Archives of Pathology & Laboratory Medicine, 2013, Vol 137, Issue 3, p434
- ISSN
0003-9985
- Publication type
Article
- DOI
10.5858/arpa.2012-0103-CR