Hepatic perivascular epithelioid cell tumor: five case reports and literature review

Asian J Surg. 2015 Jan;38(1):58-63. doi: 10.1016/j.asjsur.2012.06.010. Epub 2012 Aug 19.

Abstract

Perivascular epithelioid cell tumor (PEComa) is a rare tumor. Here, we present data regarding clinical presentations, diagnoses, management, and prognosis of five cases of hepatic PEComa between January 2002 and December 2008. Ultrasonography showed hyperechoic masses in all patients. Precontrast computed tomography (CT) showed that all lesions scanned were heterogeneous in density and were heterogeneously enhanced in arterial phase images. In two cases, magnetic resonance imaging showed hypointensity on T1-weighted images and hyperintensity on T2-weighted images. In enhanced scanning, lesions showed asymmetrical enhancement during arterial phase imaging. All tumors were composed of varying proportions of smooth muscle, adipose tissue, and thick-walled blood vessels, and showed positive immunohistochemical staining for Human Melanoma Black-45. All patients underwent hepatectomy, and there was no evidence of recurrence or metastasis during the follow-up period.

Keywords: Human Melanoma Black-45; hepatectomy; liver; perivascular epithelioid cell tumor.

Publication types

  • Review

MeSH terms

  • Adult
  • Biomarkers, Tumor / analysis
  • Female
  • Hepatectomy*
  • Humans
  • Image Enhancement
  • Liver / pathology
  • Liver Neoplasms / diagnosis*
  • Liver Neoplasms / pathology
  • Liver Neoplasms / surgery*
  • Male
  • Melanoma-Specific Antigens / analysis
  • Middle Aged
  • Multimodal Imaging
  • Perivascular Epithelioid Cell Neoplasms / diagnosis*
  • Perivascular Epithelioid Cell Neoplasms / pathology
  • Perivascular Epithelioid Cell Neoplasms / surgery*
  • Rare Diseases
  • Retrospective Studies
  • gp100 Melanoma Antigen

Substances

  • Biomarkers, Tumor
  • Melanoma-Specific Antigens
  • PMEL protein, human
  • gp100 Melanoma Antigen