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Sex cord-stromal tumors of the ovary: Tumors with granulosa and Sertoli-Leydig elements

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

INTRODUCTION

Ovarian sex cord-stromal tumors are a heterogeneous group of benign or malignant neoplasms that develop from the dividing cells that normally produce cells that support and surround the oocytes, including the cells that produce ovarian hormones (the nongerm cell and nonepithelial components of the gonads) (figure 1) [1]. Ovarian sex cord-stromal tumors are rare, comprising only 1.2 percent of all primary ovarian cancers [2].

In contrast with epithelial ovarian cancer, most patients with malignant sex cord-stromal tumors are diagnosed with early-stage disease; the neoplasms are generally considered to be low-grade malignant neoplasms.

Diagnosis of sex-cord stromal tumors is made by histology at time of surgical excision. Staging for malignant tumors is the same as for other primary ovarian carcinomas (table 1).

Ovarian sex cord-stromal tumors with both granulosa and Sertoli-Leydig cell elements (sex cord tumor with annular tubules and other tumors) are reviewed here. An overview of sex cord-stromal tumors and discussions about 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, can be found 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: Sertoli-stromal cell tumors" 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".)

SEX CORD TUMOR WITH ANNULAR TUBULES

These are unique types of sex cord-stromal tumors that are characterized by simple and complex annular (ring-shaped) tubules; they may be benign or malignant. They are thought to represent an intermediate between Sertoli cell and granulosa cell tumors. In one institutional series, sex cord tumors with annular tubules (SCTAT) accounted for 6 percent of all ovarian sex cord-stromal tumors [3].

  

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Literature review current through: Nov 2016. | This topic last updated: Mon Sep 26 00:00:00 GMT+00:00 2016.
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