Living Reference Work Entry

Handbook of Gynecology

pp 1-25

Date: Latest Version

Diagnosis and Management of Nonepithelial Ovarian Cancer

  • Erin A. BlakeAffiliated withDepartment of Obstetrics and Gynecology, University of Colorado
  • , Saketh GuntupalliAffiliated withDepartment of Obstetrics and Gynecology Division of Gynecologic Oncology, University of Colorado
  • , Koji MatsuoAffiliated withDepartment of Obstetrics and Gynecology Division of Gynecologic Oncology, University of Southern California Email author 


Nonepithelial ovarian cancers represent a small fraction of ovarian cancers. Malignancies in this category include sex cord-stromal tumors (SCST) and ovarian malignant germ cell tumors (OMGCT), and each of these classifications encompasses multiple histologic subtypes. The most common SCST include granulosa cell and Sertoli-Leydig cell tumors. Dysgerminomas, yolk sac tumors, and immature teratomas are the most frequently encountered OMGCT. The prognosis for these tumors is good; however, the survival outcome is dependent on factors such as tumor subtype and stage at diagnosis. Most patients with nonepithelial ovarian cancers present with low stage disease due to symptoms that occur early in the disease process. Surgery is the mainstay of treatment for all nonepithelial ovarian cancers. If desired, fertility sparing surgery is typically an appropriate management option for both SCST and OMGCT. Postoperative adjuvant chemotherapy is dependent on disease type and stage; however, due to the exquisite chemosensitivity of malignant germ cell tumors of the ovary, platinum-based combination chemotherapy is used for almost all cases. The most commonly used initial regimen for both SCST and OMGCT is combination of bleomycin, etoposide, and cisplatin. Surveillance for recurrent disease is mandated for all SCST and OMGCT, even those that present in early stages.


Nonepithelial ovarian cancer Sex cord-stromal tumors Malignant germ cell tumors of the ovary Granulosa Sertoli-Leydig Fibroma-thecoma Gynandroblastoma Dysgerminoma Yolk sac Teratoma