Abdominal perivascular epithelioid cell tumor (PEComa) without visible fat: a clinicopathologic and radiological analysis of 16 cases

Radiol Med. 2021 Feb;126(2):189-199. doi: 10.1007/s11547-020-01241-8. Epub 2020 Jun 19.

Abstract

Objective: To retrospectively review the clinicopathological features and computed tomography (CT) and magnetic resonance imaging (MRI) findings of abdominal perivascular epithelioid cell tumor without visible fat (PEComawvf).

Materials and methods: Sixteen patients with surgically and pathologically confirmed perivascular epithelioid cell tumor without visible fat were enrolled. Their clinicopathological data and imaging findings were retrospectively reviewed. The CT and MRI features, including location, size, shape, margin, density, calcification, cystic necrosis and enhancement pattern, were analyzed.

Results: There were 4 males and 12 females (median age, 46 years; range, 21-65 years) in this study. All 16 patients were diagnostic asymptomatic unenhanced CT or MRI and revealed a well-defined (n = 13), oval (n = 10), mass with heterogeneous (n = 6) or homogeneous density/signal intensity (n = 7), calcification and hemorrhage was no found in any cases. On enhanced CT/MRI, markedly enhancement patterns (n = 14) were observed. The "peripheral enhancement" sign was observed in 13 cases. One in 16 cases recurrence was discovered during the follow-up period.

Conclusions: Dynamic CT, MRI and pathology of PEComawvf had some characteristics of non-aggressive pattern of performance, and MRI would provide beneficial detection of microscopic fat. Enhanced imaging showed PEComawvf is characterized by a "peripheral enhancement" with a marked enhancement pattern. Knowing these characteristics could contribute to improving the understanding abdominal PEComawvf and related palliative care.

Keywords: Abdominal tumor; Computed tomography; Imaging features; Magnetic resonance imaging; PEComa.

MeSH terms

  • Abdomen / diagnostic imaging*
  • Abdomen / pathology
  • Adult
  • Aged
  • Female
  • Humans
  • Magnetic Resonance Imaging / methods*
  • Male
  • Middle Aged
  • Perivascular Epithelioid Cell Neoplasms / diagnostic imaging*
  • Perivascular Epithelioid Cell Neoplasms / pathology
  • Retrospective Studies
  • Tomography, X-Ray Computed / methods*