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Published on: August 18, 2022
PEComas: a review with emphasis on cutaneous lesions
Sarah N Walsh1, Omar P Sangüeza
1Cutaneous Pathology, WCP Laboratories, Inc, 2326 Millpark Dr., St. Louis, MO 63043, USA. sarahnwalsh@aol.com
Seminars in Diagnostic Pathology
|January 2, 2010
Summary
Perivascular epithelioid cell (PEC) tumors, or PEComas, share similarities with angiomyolipoma, clear cell sugar tumor, and lymphangioleiomyoma. Cutaneous PEComas, rare skin tumors, typically present in adult females and are often benign.
Area of Science:
- Pathology
- Oncology
- Dermatology
Background:
- Angiomyolipoma (AML), clear cell sugar tumor (CCST), and pulmonary lymphangioleiomyoma (LAM) are linked by histologic and immunophenotypic similarities, often associated with tuberous sclerosis complex (TSC).
- These tumors share a common cell type, the perivascular epithelioid cell (PEC), which forms PEComas in various locations.
- PEComas outside of AML, CCST, and LAM are typically found in middle-aged adult females and are not associated with TSC.
Purpose of the Study:
- To describe the characteristics of primary cutaneous PEComas.
- To highlight the unique features of PEComas arising in the skin compared to other sites.
- To review the current understanding of PEComa behavior and treatment.
Main Methods:
- Histologic and immunophenotypic analysis of PEComas.
- Review of reported cases of cutaneous PEComas.
- Comparison of cutaneous PEComas with PEComas at other sites.
Main Results:
- PEComas are composed of nests and fascicles of epithelioid or spindled cells arranged around blood vessels.
- These cells characteristically express melanocytic (HMB45) and myogenic (actin) markers.
- Primary cutaneous PEComas predominantly affect adult females, often presenting as painless extremity masses, with desmin being the most common myogenic marker. Most cases are benign, but malignant potential exists.
Conclusions:
- Cutaneous PEComas are a distinct entity within the PEComa family, with specific clinical and immunohistochemical features.
- While generally benign, the possibility of malignancy in cutaneous PEComas warrants consideration.
- Surgical excision remains the primary treatment for cutaneous PEComas.
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