Fertility-sparing surgery for advanced juvenile granulosa cell tumor of the ovary

Juvenile granulosa cell tumors (JGCT) of the ovary are rare. They usually present in children and adolescents. About 90% are diagnosed in early stage (FIGO I) with a favorable prognosis. More advanced stages (FIGO II-IV) usually have a poor clinical outcome.

We report a case of long-term survival of a teenager with Stage III JGCT treated with aggressive debulking and thorough staging, but conservative surgery relative to the uterus, contralateral uninvolved ovary, and fallopian tube, plus combination chemotherapy. Her tumor recurred twice, 18 months and 17 years later, for which she had 2 additional surgeries and more chemotherapy. Our patient achieved 2 pregnancies and had 3 children.

With fertility sparing surgery, patients may be able to achieve pregnancies and children.