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PEComa-its clinical features, histopathology, and current therapy.
Yuya Izubuchi1, Takaaki Tanaka1
1Department of Orthopaedics and Rehabilitaion Medicine, Unit of Surgery, Division of Medicine, Faculty of Medical Sciences, University of Fukui 23-3, Matsuokashimoaizuki, Eiheiji-cho, Yoshida-gun, Fukui 910-1193, Japan.
Japanese Journal of Clinical Oncology
|May 8, 2025
Summary
Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. This review covers PEComa clinical features, molecular biology, and treatment, including TFE3 gene rearrangements and mTOR inhibitors.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Perivascular epithelioid cell tumors (PEComas) are a rare group of mesenchymal neoplasms.
- This tumor family includes angiomyolipoma, lymphangioleiomyomatosis, and clear cell
- sugar
- tumors.
- PEComas can occur in various organs and predominantly affect women, typically presenting in young to middle-aged individuals.
Purpose of the Study:
- To comprehensively review the clinical characteristics, molecular biology, and treatment modalities for PEComas.
- To highlight the diagnostic significance of TFE3 gene rearrangements in a subset of PEComas.
- To discuss current therapeutic strategies, including surgery and emerging treatments like mTOR inhibitors.
Main Methods:
- Literature review of clinical features, molecular pathology, and treatment outcomes for PEComas.
- Analysis of immunohistochemical markers (HMB45, melan-A, α-SMA, desmin, caldesmon) and genetic alterations (TFE3 rearrangement, TSC mutations).
- Evaluation of treatment efficacy based on case reports and clinical trials.
Main Results:
- PEComas exhibit diverse clinical presentations and can follow a benign or malignant course.
- Immunohistochemistry typically shows melanocytic and muscle markers; TFE3 gene rearrangement defines a distinct subtype.
- TFE3-rearranged PEComas are associated with younger age, lack of TSC mutation, specific morphology, and strong TFE3 expression.
Conclusions:
- PEComas are a heterogeneous group requiring accurate diagnosis through clinical, immunohistochemical, and molecular findings.
- Surgery remains the primary curative treatment, with mTOR inhibitors showing promise in clinical trials.
- Further research is needed to fully elucidate the role of radiation therapy in PEComa management.
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