Bcl-2 expression in a primary leiomyosarcoma of the ovary: a case report.

Abstract

Primary ovarian leiomyosarcoma is an extremely rare malignant smooth-muscle neoplasm with fewer than 30 documented cases worldwide. Immunohistochemically, ovarian leiomyosarcomas are characterized by the expression of alpha-smooth muscle actin (alpha-SMA). Until recently, no report investigated the role of bcl-2 expression in primary ovarian leiomyosarcoma. We report the management and an immunohistochemical analysis of bcl-2 in a patient with primary leiomyosarcoma of the ovary. The patient, a 71-year-old woman, presented with a history of lower abdominal pain and a palpable mass in the lower abdomen. Surgical exploration revealed a large left adnexal mass that had developed from the left ovary and infiltrated one part of the omentum adherent to the left adnexa. Microscopically the left adnexal tumor consisted of spindle cells arranged in smooth-muscle bundles and fascicles. The tumor was characterized by high cellularity and nuclear polymorphism, as well as patchy necrosis and large areas of hemorrhage. Mitotic activity exceeded 5 mitoses per 10 high-power fields with many atypical mitoses. The tumor appeared encapsulated, and the capsule was partially disrupted by tumor cells. Immunohistochemically, the tumor showed a strong staining reaction for muscle actin, alpha-SMA and neuron-specific enolase, and also a weak reaction for vimentin. Strong staining of bcl-2 was detected, with more than 80% of sarcoma cells positive. According to the FIGO staging system for ovarian cancer, the tumor was classified as a primary ovarian leiomyosarcoma stage III C, and the patient was treated with an adjuvant chemotherapy regimen consisting of cisplatin and ifosfamide. Two days after the third cycle of cisplatin/ifosfamid she developed an apoplexy spontaneously and chemotherapy was therefore discontinued. The patient remained without evidence of disease until she died as a result of intercurrent pneumonia 14 months after initial surgery. Beside the routine histological and immunohistochemical characteristics of primary ovarian leiomyosarcoma, strong staining for bcl-2 was detected. In order to understand more about the nature and the behaviour of this highly malignant neoplasm and to be able to improve the treatment, the prognostic value of bcl-2 has to be investigated in additional studies.