Natural History of Primary Retroperitoneal Extra-Visceral Perivascular Epithelioid Cell Tumors (PEC): A Study from Transatlantic and Australasian Retroperitoneal Sarcoma Working Group (TARPSWG).

ABSTRACT:

Background

Perivascular epithelioid cell tumors (PEComa) are a rare family of mesenchymal tumors that include several subtypes. There are very limited data describing the natural history of patients with extra-visceral retroperitoneal PEComas of the retroperitoneum. The aim of this study is to describe the clinical features, treatment patterns, outcomes, and diagnostic challenges of primary extra-visceral retroperitoneal or abdominopelvic PEComa over the past decade.

Patients and methods

This is a retrospective analysis of all extra-visceral, non-renal, retroperitoneal, or abdominopelvic PEComas treated at participating centers over the past 10 years.

Results

A total of 77 patients from 13 centers were included. The median age at diagnosis was 56 years (range 18-81 ye

SUBMITTER: Mor E 

PROVIDER: S-EPMC12454588 | biostudies-literature | 2025 Oct

REPOSITORIES: biostudies-literature