PEComas: A review of imaging and clinical features

Adam Kinzel; Mark McArthur; Lori Mankowski Gettle; Ely Felker; Maitraya Patel

doi:10.1016/j.clinimag.2024.110332

PEComas: A review of imaging and clinical features

Clin Imaging. 2024 Dec:116:110332. doi: 10.1016/j.clinimag.2024.110332. Epub 2024 Oct 16.

Authors

Adam Kinzel¹, Mark McArthur², Lori Mankowski Gettle³, Ely Felker⁴, Maitraya Patel⁴

Affiliations

¹ Charlotte Radiology, 8514 McAlpine Park Dr., Suite 100, Charlotte, NC 28211, USA.
² University of California, Los Angeles, David Geffen School of Medicine at UCLA, Department of Radiological Sciences, 757 Westwood Plaza, Suite 1621, Los Angeles, CA 90095-7532, USA. Electronic address: mmcarthur@mednet.ucla.edu.
³ Richard L. Roudebush VA Medical Center, 1481 W. 10(th) St., Indianapolis, IN 46202, USA.
⁴ University of California, Los Angeles, David Geffen School of Medicine at UCLA, Department of Radiological Sciences, 757 Westwood Plaza, Suite 1621, Los Angeles, CA 90095-7532, USA.

PMID: 39442258
DOI: 10.1016/j.clinimag.2024.110332

Abstract

Perivascular epithelioid cell tumors (PEComas) are a group of rare mesenchymal tumors, which demonstrate varied imaging appearances and treatment options. These tumors may arise de novo or in the setting of systemic disorders, such as tuberous sclerosis. Some PEComas are benign and easily resected while others may represent systemic or metastatic disease with limited therapeutic options. The purpose of this review is to introduce the topic of perivascular epithelioid cell tumors and the most common tumors within the PEComa family as well as discuss the epidemiology, morphology, radiographic appearance, and treatment options of these rare tumors.

Keywords: Angiomyolioma (AML); Lymphangioleiomyomatosis (LAM); PEComa; Tuberous sclerosis (TS).

Publication types

Review

MeSH terms

Diagnosis, Differential
Female
Humans
Magnetic Resonance Imaging / methods
Perivascular Epithelioid Cell Neoplasms* / diagnostic imaging
Tomography, X-Ray Computed / methods