Small cell ovarian carcinoma: Long term survival in juvenile case with poor prognostic features

BACKGROUND: Ovarian small cell carcinoma is a rare, aggressive neoplasm that occurs in young women and has a poor long-term prognosis. Treatment involves surgical resection and chemotherapy. The required radicality of surgery is uncertain, balancing cytoreduction with fertility preservation. Various chemotherapy regimens are utilized due to confusion regarding the neoplasm's lineage. Case We describe an adolescent with small cell carcinoma, hypercalcemic type, stage IA. Surgery included left salpingo-oopherectomy, left pelvic/paraaortic lymphadenectomy, omentectomy and peritoneal biopsies. She received four cycles of bleomycin, etoposide and cisplatin, similar to high-risk germ cell cancers. She has received no further therapy and is eleven years from diagnosis without evidence of disease. CONCLUSION: This is the first long-term juvenile survivor managed with both fertility-sparing surgery and BEP (bleomycin, etoposide, cisplatin).