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Overview of sex cord-stromal tumors of the ovary

David M Gershenson, MD
Section Editors
Barbara Goff, MD
Rochelle L Garcia, MD
Don S Dizon, MD, FACP
Deputy Editors
Sandy J Falk, MD, FACOG
Sadhna R Vora, MD


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) [1]. Malignant ovarian sex cord-stromal neoplasms are rare, comprising only 1.2 percent of all primary ovarian cancers (malignant neoplasms) [2].

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 [2]. 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:

(See "Sex cord-stromal tumors of the ovary: Granulosa-stromal cell tumors".)

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Literature review current through: Nov 2017. | This topic last updated: Aug 22, 2017.
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