1. Primary cutaneous malignant perivascular epithelioid cell tumor: Case of a rare tumor with review of the literature
- Author
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Scott C. Bresler, Tyler D Menge, Douglas R. Fullen, Leela M Hamp, Daniel W Cole, Sruthi Renati, and Rajiv M. Patel
- Subjects
Adult ,Pathology ,medicine.medical_specialty ,Skin Neoplasms ,Histology ,Perivascular Epithelioid Cell Neoplasms ,Biopsy ,Dermatology ,Perivascular Epithelioid Cell ,Pathology and Forensic Medicine ,030207 dermatology & venereal diseases ,03 medical and health sciences ,0302 clinical medicine ,Atypia ,Humans ,Medicine ,Neoplasm ,Skin ,integumentary system ,biology ,business.industry ,CD68 ,Mesenchymal stem cell ,Margins of Excision ,medicine.disease ,Combined Modality Therapy ,Immunohistochemistry ,Actins ,Caldesmon ,Treatment Outcome ,030220 oncology & carcinogenesis ,biology.protein ,Calmodulin-Binding Proteins ,Female ,Radiotherapy, Adjuvant ,Dermatopathology ,business ,Follow-Up Studies - Abstract
Perivascular epithelioid cell tumors (PEComas) are mesenchymal neoplasms with characteristic epithelioid or spindled cytomorphology that typically grow around blood vessels. These tumors are phenotypically and immunohistochemically distinct, expressing markers of both melanocytic and smooth muscle differentiation. Herein, we describe a case of histopathologically malignant cutaneous PEComa without metastatic spread, with review of the pertinent literature. Telescoping punch biopsy demonstrated an epithelioid neoplasm with marked atypia, hypercellularity, and increased mitotic activity. Immunohistochemical stains for HMB-45, NK1-C3, PGP9.5, MiTF, CD10, and CD68 were positive within tumor cells. In addition, there was diffuse expression of caldesmon and focal cytoplasmic staining for smooth muscle actin on the excision specimen. The patient underwent treatment with surgical excision with adjuvant radiation and surveillance computed tomography (CT). The patient remains free of recurrence or metastatic disease after 10 months of follow-up. To our knowledge, this is only the third reported case of a malignant cutaneous PEComa reported in the literature to date.
- Published
- 2021
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