Overview of sex cord-stromal tumors of the ovary
- David M Gershenson, MD
David M Gershenson, MD
- J Taylor Wharton MD Distinguished Chair in Gynecologic Oncology
- Professor of Gynecologic Oncology
- The University of Texas MD Anderson Cancer Center
- Section Editors
- Barbara Goff, MD
Barbara Goff, MD
- Section Editor — Gynecologic Oncology
- Professor of Gynecologic Oncology
- University of Washington
- Rochelle L Garcia, MD
Rochelle L Garcia, MD
- Section Editor — Obstetric and Gynecologic Pathology
- Professor of Pathology
- Adjunct Professor of Obstetrics & Gynecology
- University of Washington Medical Center
- Don S Dizon, MD, FACP
Don S Dizon, MD, FACP
- Section Editor – Gynecologic Oncology
- Clinical Co-Director, Gynecologic Oncology
- Founder and Director, The Oncology Sexual Health Clinic
- Massachusetts General Hospital Cancer Center
- Associate Professor of Medicine
- Harvard Medical School
- Deputy Editors
- Sandy J Falk, MD, FACOG
Sandy J Falk, MD, FACOG
- Director, Editorial Relations — UpToDate
- Deputy Editor — Obstetrics, Gynecology and Women's Health
- Instructor of Obstetrics, Gynecology and Reproductive Biology, Part-time
- Harvard Medical School
- Sadhna R Vora, MD
Sadhna R Vora, MD
- Deputy Editor — Oncology
- Instructor in Medicine
- Harvard Medical School
Ovarian sex cord-stromal neoplasms are a heterogeneous group of benign or malignant neoplasms that develop from the dividing cell population that would normally give rise to cells surrounding the oocytes, including the cells that produce ovarian hormones (the nongerm cell and nonepithelial components of the gonads) (figure 1 and table 1) . Malignant ovarian sex cord-stromal neoplasms are rare, comprising only 1.2 percent of all primary ovarian cancers (malignant neoplasms) .
In contrast with epithelial ovarian cancer, most patients with malignant sex cord-stromal neoplasms are diagnosed with early-stage disease; even malignant neoplasms are generally considered to be low grade . Lymph node metastases are rare from these neoplasms [3-5].
Some sex cord-stromal neoplasms produce sex steroid hormones, including estrogen and androgens [6,7]. These may result in symptoms of estrogen excess or other effects, and measurement of these and other tumor markers may play a role in diagnosis.
General principles of ovarian sex cord-stromal neoplasms are reviewed here. Histologic subtypes are discussed separately:
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- EPIDEMIOLOGY AND RISK FACTORS
- Cell population
- Patterns of spread
- CLINICAL FEATURES
- Clinical presentation
- Physical examination
- Tumor markers
- Imaging studies
- Endometrial sampling
- Staging and surgical treatment
- - Preoperative preparation and evaluation
- - Staging system and procedure
- - Fertility preservation
- Postoperative treatment
- POSTTREATMENT SURVEILLANCE
- HORMONE THERAPY FOLLOWING TREATMENT
- APPROACH TO RECURRENT DISEASE
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS