Fibrosarcoma of ovary. A new component in the nevoid basal-cell carcinoma syndrome

An 8-year-old child with nevoid basal-cell carcinoma syndrome who developed abdominal pain underwent exploratory laparotomy. Both ovaries were enlarged and replaced by fibroblastic proliferations having cellular foci with high mitotic indices (greater than 4 mitoses/10 high-power fields) diagnostic of fibrosarcoma. Two years following salpingo-oophorectomy, a metastasis was excised from one adnexa. Further recurrence or distant metastasis was not evident after 6 more years of follow-up. The association of fibrosarcoma of the ovary in a patient with nevoid basal-cell carcinoma further expands the multifarious nature of this syndrome.