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J Oncol Pract. 2016 Oct;12(10):940-946.

Ovarian Sex Cord-Stromal Tumors.

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Children's Hospitals and Clinics of Minnesota, Minneapolis, MN; Massachusetts General Hospital, Harvard Medical School; Dana-Farber Cancer Center/Children's Cancer Care, Boston, MA; Levine Cancer Institute, Carolinas Medical Center, Charlotte, NC; The University of Texas MD Anderson Cancer Center, Houston, TX; Washington University Medical Center, St Louis, MO; Children's National Medical Center and Center for Genetic Medicine Research, Children's Research Institute, Washington, DC; and Clinic of Pediatrics, Dortmund, Germany.


Ovarian sex cord-stromal tumors are clinically significant heterogeneous tumors that include several pathologic types. These tumors are often found in adolescents and young adults and can present with hormonal manifestations as well as signs and symptoms of a pelvic mass. Serum tumor markers may assist in preoperative diagnosis and surveillance. Several subtypes are associated with genetic predisposition, including those observed in patients with Peutz-Jegher syndrome. Recent studies have elucidated the relationship between Sertoli-Leydig cell tumors and DICER1 mutations. When classified as International Federation of Gynecology and Obstetrics stage Ia, most subtypes may be treated with surgery alone. Higher stage or recurrent tumors have variable prognoses that range from a usually rapid course in poorly differentiated Sertoli-Leydig cell tumor to an often prolonged course in adult granulosa cell tumors. New understanding of the molecular pathogenesis of these tumors may pave the way for novel therapeutics.

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