PEComa (Perivascular Epithelioid Cell Neoplasm)

Presentation :

Pathophysiology :

• A family of rare soft tissue tumors that typically form around small blood vessels (perivascular spaces):
  • Angiomyolipoma (AML)
  • Lymphangioleiomyomatosis (LAM)
  • Clear cell “sugar” tumor of the lung (CCST)
  • Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres (CCMT)

Diagnostic Testing:

Treatment :

Prognosis:

References:

• Hornick, J. L., and C. D. M. Fletcher. "PEComa: what do we know so far?." Histopathology 48.1 (2006): 75-82.
• Bourgmayer, Agathe, Simon Nannini, Paul Bonjean, Jean-Emmanuel Kurtz, Gabriel G. Malouf, and Justine Gantzer. "Natural history and treatment strategies of advanced PEComas: a systematic review." Cancers 13, no. 20 (2021): 5227.