Soft Tissue Perivascular Epithelioid Cell Tumors

Phoebe M Hammer; Serena Y Tan

doi:10.1016/j.path.2023.06.002

Soft Tissue Perivascular Epithelioid Cell Tumors

Surg Pathol Clin. 2024 Mar;17(1):105-118. doi: 10.1016/j.path.2023.06.002. Epub 2023 Aug 1.

Authors

Phoebe M Hammer¹, Serena Y Tan²

Affiliations

¹ Department of Pathology, Stanford University School of Medicine, 1291 Welch Road, Lane Building L235, Stanford, CA 94305, USA.
² Department of Pathology, Stanford University School of Medicine, 1291 Welch Road, Lane Building L235, Stanford, CA 94305, USA. Electronic address: serenayt@stanford.edu.

PMID: 38278600
DOI: 10.1016/j.path.2023.06.002

Abstract

Perivascular epithelioid cell tumors (PEComas) are a heterogenous group of mesenchymal neoplasms with a mixed myomelanocytic immunophenotype. PEComa-family tumors include angiomyolipoma, lymphangioleiomyomatosis, and a large category of rare neoplasms throughout the body that are now classified under the umbrella term "PEComa." This review focuses on recent advances in the clinicopathological and molecular features of PEComas, with an emphasis on PEComas that originate in soft tissue.

Keywords: Angiomyolipoma; PEComa; TFE3; TSC.

Publication types

Review

MeSH terms

Biomarkers, Tumor / genetics
Humans
Neoplasms, Connective and Soft Tissue*
Perivascular Epithelioid Cell Neoplasms* / diagnosis
Perivascular Epithelioid Cell Neoplasms* / genetics
Perivascular Epithelioid Cell Neoplasms* / pathology

Substances

Biomarkers, Tumor