Primary ovarian rhabdomyosarcoma in children.

Although rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children, primary rhabdomyosarcomas of the ovary are extremely rare, with only eight well-documented pediatric cases previously reported in the literature. We present two additional cases: an alveolar RMS originating in the right ovary with metastatic spread to the splenic flexure of the colon and to both lungs in a 13-year-old African American girl, and an embryonal RMS arising in the right ovary of a 6-year-old Caucasian girl with pre-operative intra-abdominal rupture and a malignant right pleural effusion. Both patients had complete resection of their primary tumors and received chemotherapy including vincristine, doxorubicin and cyclophosphamide with good response to therapy. Both are alive 8 and 9 months post-operatively.