Sex cord-stromal tumors of the ovary: Sertoli-stromal cell tumors
- 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 tumors are a heterogeneous group of benign or malignant neoplasms that develop from the stem cells that would typically furnish cells surrounding the oocytes, including the cells that produce ovarian hormones (the nongerm cell and nonepithelial components of the gonads) (figure 1) . Ovarian sex cord-stromal tumors are rare, comprising only 1.2 percent of all primary ovarian cancers .
In contrast with epithelial ovarian cancer, most patients with malignant sex cord-stromal tumors are diagnosed with early-stage disease; the tumors are generally considered to be low-grade malignant neoplasms.
Sertoli-Leydig cell tumors constitute less than 0.5 percent of ovarian neoplasms . They may behave in a benign or malignant fashion, which correlates with the degree of differentiation in an individual case. Approximately 75 percent occur in women under the age of 40 years (mean age at diagnosis is 25), but they occur in all age groups. The neoplasms are characterized by the presence of testicular structures that produce androgens. This can result in virilization, although not all of these neoplasms are functionally active.
Ovarian sex cord-stromal tumors of the Sertoli-stromal cell type are reviewed here. This category includes Sertoli-Leydig cell tumors (androblastomas) as well as pure Sertoli cell or Leydig cell tumors. An overview of sex cord-stromal tumors and other types of sex cord-stromal tumors of the ovary (Sertoli-stromal cell tumors and tumors with granulosa and Sertoli-Leydig elements), as well as epithelial ovarian cancer, are discussed separately. (See "Overview of sex cord-stromal tumors of the ovary" and "Sex cord-stromal tumors of the ovary: Granulosa-stromal cell tumors" and "Sex cord-stromal tumors of the ovary: Tumors with granulosa and Sertoli-Leydig elements" and "Epithelial carcinoma of the ovary, fallopian tube, and peritoneum: Histopathology" and "Epithelial carcinoma of the ovary, fallopian tube, and peritoneum: Clinical features and diagnosis".)
Two gene mutations associated with Sertoli-Leydig cell tumors have been reported, and both may be clinically relevant. FOXL2 is a somatic missense point mutation that was initially reported to be present in granulosa cell tumors of the ovary . Subsequently, the same group analyzed this marker in a larger series of sex cord-stromal ovarian tumors . The authors found that FOXL2 is a relatively sensitive and highly specific marker for this tumor type. FOXL2 immunostaining was present in almost all sex cord-stromal tumors with a FOXL2 mutation and in a majority of tumors without a mutation. Importantly, only 50 percent of Sertoli-Leydig cell tumors expressed FOXL2. However, together with alpha-inhibin and calretinin, FOXL2 forms an immunomarker panel that will aid in the diagnosis of a sex cord-stromal ovarian tumor.
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