Juvenile granulosa cell tumor of the ovary associated with tuberous sclerosis.
BACKGROUND
Tuberous sclerosis is a neurocutaneous syndrome characterized by benign tumors that can affect many organs. Juvenile granulosa cell tumors of the ovary are rare neoplasms that typically occur in the first three decades of life and have excellent prognosis for early-staged disease.


METHODS
We report the first case of an 8-year-old white female with tuberous sclerosis and juvenile granulosa cell tumor of the ovary. She presented with a 20 x 22 cm pelvic mass and received a right salpingo-oophorectomy. Three months later, she recurred and underwent a left salpingo-oophorectomy, lymphadenectomy, and omentectomy followed by four cycles of Bleomycin, Etoposide, and Cisplatin chemotherapy. She is currently free of disease 8 years after her recurrence.


CONCLUSIONS
Treatment options and a review of the literature pertaining to juvenile ovarian granulosa cell tumors and tuberous sclerosis are discussed.
Guo
Hongyan
H
Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Stanford University School of Medicine, 300 Pasteur Drive HH333, CA 94305, USA.
Keefe
Kristin A
KA
Kohler
Mathew F
MF
Chan
John K
JK
D002648:Child; D005260:Female; D006106:Granulosa Cell Tumor / Q000150:complications* / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D006801:Humans; D010051:Ovarian Neoplasms / Q000150:complications* / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D014402:Tuberous Sclerosis / Q000150:complications* / Q000188:drug therapy / Q000473:pathology / Q000601:surgery