Perivascular epithelioid cell tumor (PEComa) of the uterus with aggressive behavior at presentation.
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.
Changhua Christian Hospital, Changhua, Taiwan, Province of China.
Jing-Lan
JL
Liu
Yueh-Min
YM
Lin
Ming-Chieh
MC
Lin
Kun-Tu
KT
Yeh
Jui-Chang
JC
Hsu
Chih-Jung
CJ
Chin
D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008207:Lymphatic Metastasis; D020360:Neoadjuvant Therapy; D054973:Perivascular Epithelioid Cell Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D016896:Treatment Outcome; D014594:Uterine Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery