Primary ovarian fibrosarcoma – An unusual tumor in an adolescent: Case report and review of the pediatric literature

Abstract Background Primary ovarian fibrosarcomas are rare and usually observed in perimenopausal and postmenopausal women. Up-to-date, there are only 3 reports of ovarian fibrosarcoma in childhood and adolescence in English literature. In this report, we aimed to present the first pediatric case with advanced staged primary ovarian fibrosarcoma and to compare with previous cases. Case A 14-year-old teenage girl was admitted due to a giant abdominal mass. Imaging techniques revealed a giant heterogeneous and vascular ovarian mass. Salpingo-oophorectomy along with staging procedures were performed. Pathologic examination confirmed grade III ovarian fibrosarcoma with severe nuclear atypia and high mitotic count. Due to pulmonary metastasis, she received four courses of adjuvant combination chemotherapy (Ewing’s protocole). The patient stood the treatment well and is disease-free in two years of follow-up. Conclusion Fibrosarcoma of the ovary is an uncommon neoplasm with poor prognosis. To our knowledge, the presented patient is the first pediatric case with advanced staged primary ovarian fibrosarcoma to be treated with surgery and adjuvant chemotherapy.