Juvenile granulosa cell tumor of the ovary associated with tuberous sclerosis

Gynecologic Oncology
Hongyan GuoJohn K Chan

Abstract

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. 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. Treatment options and a review of the literature pertaining to juvenile ovarian granulosa cell tumors and tuberous sclerosis are discussed.

References

Mar 27, 2001·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·M W JohnsonH V Vinters
Jan 10, 2002·International Journal of Gynecological Pathology : Official Journal of the International Society of Gynecological Pathologists·Ann E AndersonRobert H Young
May 7, 2002·Journal of Pediatric Hematology/oncology·Anat Erdreich-EpsteinJudith G Villablanca
Aug 8, 2002·Klinische Pädiatrie·D T SchneiderU Göbel
Apr 11, 2003·The Journal of Urology·Thomas S Lendvay, Fray F Marshall

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Citations

Jun 19, 2013·Indian Journal of Endocrinology and Metabolism·Nisha Nigil HaroonPreeti Dabadghao
Feb 16, 2021·European Journal of Surgical Oncology : the Journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology·Alice BergaminiGiorgia Mangili

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