Cystadenocarcinoma of the ovary in a 4-year-old: benign transformation during therapy.

Ovarian tumors comprise only 1% of all tumors in females below the age of 17 years. Of these, the epithelial tumors are the least common, being particularly rare before puberty and then increasing in frequency with advancing age after puberty. The youngest girl previously reported in the literature with an epithelial ovarian tumor was a 9-year-old child with an ovarian papillary cystadenocarcinoma. This is the only case thus far reported of a malignant ovarian epithelial tumor occurring before puberty. Only histologically benign appearing glands were detected in the resected tumor masses, on a second-look laparotomy following the use of multiagent chemotherapy.