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Angiomyolipoma/PEComa: the past, the present…and back to the future
Anna Caliò1, Stefano Marletta2,3, Guido Martignoni1,4
1Department of Diagnostics and Public Health, Section of Pathology, University of Verona.
Current Opinion in Urology
|December 11, 2025
Summary
Renal angiomyolipoma, a PEComa family neoplasm, is now understood as having clonal origin and malignant potential. New markers and molecular insights, including the cGAS-STING-TFEB pathway, are advancing diagnosis and understanding.
Area of Science:
- Nephrology
- Oncology
- Pathology
Background:
- Renal angiomyolipoma (AML) is a mesenchymal neoplasm belonging to the perivascular epithelioid cell tumor (PEComa) family.
- Historically classified as a hamartoma, recent insights reveal its neoplastic nature with clonal origins.
Purpose of the Study:
- To provide a comprehensive update on renal angiomyolipoma (AML).
- To review its classification, pathology, and molecular insights.
- To emphasize its relevance in current diagnostic and pathogenetic contexts.
Main Methods:
- Review of historical classifications and evolving pathological understanding.
- Analysis of recent molecular studies and immunohistochemical advancements.
- Synthesis of emerging evidence on molecular drivers and pathways.
Main Results:
- AML/PEComa is recognized as a neoplasm with malignant potential, particularly epithelioid subtypes.
- New immunohistochemical markers (GPNMB, STING, TRIM63) aid differential diagnosis.
- Frequent TSC1/TSC2 mutations and mTOR pathway dysregulation are identified.
- Emerging evidence points to noncanonical TFEB activation via the cGAS-STING pathway.
Conclusions:
- Accurate diagnosis and risk stratification require understanding AML/PEComa histological subtypes and molecular drivers.
- The cGAS-STING-TFEB axis may explain the unique immunophenotype and observed autophagy.
- Further research is needed to validate the proposed pathophysiological mechanisms involving STING.
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