Abstract

Epithelial ovarian malignancies are uncommon prior to the menarche. Mucinous cystadenocarcinomas are rare in children and tend to present with advanced disease. A case is described of malignant mucinous cystadenocarcinoma of the ovary occurring in a premenarchal girl resulting in intestinal obstruction. There was a fatal outcome despite multimodal therapy, which included ablative surgery. Familial recurrence of site-specific ovarian cancer requires counselling and follow-up of the female siblings of children with aggressive tumors.

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