Perivascular epithelioid cell tumor [PEComa] of the uterus with aggressive behavior at presentation

ABSTRACT

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