Advanced PEComa

About PEComa​

  • PEComas are a rare subset of soft tissue sarcomas recently recognized as a distinct entity by the World Health Organization in 2002 and are composed of histologically and immunohistochemically distinctive epithelioid cells.
  • PEComas appear to arise most commonly at visceral (especially gastrointestinal and uterine), retroperitoneal, and abdominopelvic sites.
  • Most PEComas are benign, but a subset are known as advanced malignant PEComas [1].
  • The prognosis for this patient subset is poor, with a median survival estimated to be 12-17 months following diagnosis of advanced disease [2].
  • Overactivation of the mTOR pathway has been reported in malignant PEComa, and mTOR inhibitors have shown anecdotal efficacy in this indication in case reports or retrospective studies [1].

1. Wagner AJ et al. J Clin Oncol. 2010;28(5):835-840.

2. Bleeker JS et al. Sarcoma. 2012;2012:541626.