Wolffian tumor of the ovary with a prominent spindle cell component: report of a case with brief discussion of unusual problems in differential diagnosis, and literature review.

An 87-year-old woman underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy for endometrioid adenocarcinoma. At operation, a 4.5-cm partially solid and partially cystic right ovarian mass was identified. Frozen section showed a cytologically bland spindle cell proliferation reminiscent of cellular fibroma. Extensive sampling of the ovarian mass revealed a focus with the classic sieve-like pattern of a Wolffian adnexal tumor that merged with the fibroma-like appearance. The uterus showed well-differentiated, superficially invasive endometrioid adenocarcinoma arising in a background of atypical complex hyperplasia. Given the recent reports implying a therapeutic value for Gleevec (Novartis, Stein, Switzerland) (STI-571) in the treatment of Wolffian tumor of the ovary, paraffin immunostain for CD117 (c-kit) was performed that yielded negative results. Without further therapy, the patient was alive without disease 7 months after surgery. This case demonstrates the heterogeneity of Wolffian tumor of the ovary and shows how crucial sampling is in arriving at the correct diagnosis.