Perivascular epithelioid cell tumor (PEComa) of the uterus with aggressive behavior at presentation

Hematol Oncol Stem Cell Ther. 2009;2(3):426-30. doi: 10.1016/s1658-3876(09)50013-1.


Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells (PECs). Both benign and malignant tumors have been identified, but the criteria for diagnosis of malignancy have not been fully established due to the rarity of the tumor. We report on a case of uterine PEComa in a 33-year old woman with lymph node metastasis at presentation. The tumor had the characteristic histologic features of PEComa with cytologic atypia, mitotic activity of 2/10 high power field (HPF), and necrosis; it exhibited immunopositivity for HMB-45, calponin and desmin and was negative for melan-A. The patient received neoadjuvant chemotherapy, debulking surgery and adjuvant chemotherapy. No evidence of recurrence or metastasis was apparent 8 months after surgery.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Female
  • Humans
  • Lymphatic Metastasis
  • Neoadjuvant Therapy
  • Perivascular Epithelioid Cell Neoplasms / drug therapy
  • Perivascular Epithelioid Cell Neoplasms / pathology*
  • Perivascular Epithelioid Cell Neoplasms / surgery
  • Treatment Outcome
  • Uterine Neoplasms / drug therapy
  • Uterine Neoplasms / pathology*
  • Uterine Neoplasms / surgery