Neoplasms of the perivascular epithelioid cell involving the abdomen and the pelvis: Cross-sectional imaging findings

Srinivasa R. Prasad, Dushyant V. Sahani, Mari Mino-Kenudson, Vamsi R. Narra, Peter A. Humphrey, Christine O. Menias, Kedar N. Chintapalli

Research output: Contribution to journalReview articlepeer-review

37 Scopus citations

Abstract

Neoplasms of the perivascular epithelioid cell (PEComas) represent a recently described heterogeneous group of mesenchymal tumors characterized by the presence of specific histological, immunohistochemical, and ultrastructural findings. The PEComas encompass a family of neoplasms that include angiomyolipomas, clear cell sugar tumors, and lymphangioleiomyomatosis. The PEComas demonstrate a wide spectrum of clinicobiological behavior and imaging findings. Perivascular epithelioid cell, as the name implies, is a unique cell that is characterized by perivascular distribution and epithelioid morphology. Perivascular epithelioid cell consistently shows immunoreactivity to melanocytic and smooth muscle markers including HMB-45 and actin. Abdominopelvic PEComas are found at a variety of somatic and visceral locations including kidney, liver, pancreas, gastrointestinal tract, genitourinary tract, peritoneum, and retroperitoneum. A subset of patients with abdominopelvic PEComas manifests tuberous sclerosis complex. In this paper, we review the histological spectrum and discuss the imaging findings of the PEComas that involve the abdomen and pelvis.

Original languageEnglish (US)
Pages (from-to)688-696
Number of pages9
JournalJournal of Computer Assisted Tomography
Volume31
Issue number5
DOIs
StatePublished - Sep 1 2007

Keywords

  • Abdomen
  • CT scan
  • Magnetic resonance imaging
  • PEComa
  • Pelvis

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging

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