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.
D000290:Adnexa Uteri; D001478:Basal Cell Nevus Syndrome / Q000150:complications*; D001706:Biopsy; D002280:Basal Cell Carcinoma / Q000150:complications*; D002648:Child; D005260:Female; D005350:Fibroma / Q000150:complications; D005354:Fibrosarcoma / Q000150:complications* / Q000473:pathology / Q000556:secondary / Q000648:ultrastructure; D005833:Genital Neoplasms, Female / Q000556:secondary / Q000648:ultrastructure; D006801:Humans; D010051:Ovarian Neoplasms / Q000150:complications* / Q000473:pathology / Q000648:ultrastructure