Primitive neuroectodermal tumor of the uterus

Background.Primitive peripheral neuroectodermal tumors (PNETs) of the uterus are rare. Recent data have demonstrated improved response rates with adjuvant chemotherapy.Case.A 26-year-old, gravida 2, para 1, Filipina female underwent an emergent cesarean section for fetal indications. Intraoperative findings were remarkable for a soft tissue mass in the lower uterine segment. Histologic features, immunohistochemical findings, and chromosomal analysis were consistent with a PNET. The patient underwent radical surgery, adjuvant chemotherapy with vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide (VAC/IE), and whole pelvic radiation therapy.Conclusion.Only 14 case reports of primitive neuroectodermal tumors of the uterus have been published in the English literature to date. No definitive conclusions concerning the therapeutic management and prognosis have been ascertained.