Introduction

Sex cord-stromal tumors account for 5.7 to 17% of malignant tumors in a series of ovarian neoplasms in children[1][2]. They consist of a variety of tumor types, the most common of which are granulosa-theca cell tumors and Sertoli-Leydig cell tumors. These tumors arise from uncommitted mesenchymal stem cells that reside below the surface epithelium of the urogenital ridge[3][4]. Sex cord-stromal tumors are referred to as functioning ovarian tumors as they have systemic hormonal effects and therefore typically present with signs of hormonal production. Before nine years of age, most sex cord-stromal tumors are feminizing. After nine years of age, there is a predominance of virilizing neoplasms[5]. A subset of sex cord-stromal tumors are associated with genetic aberrations such as a DICER-1 mutation, and an underlying cancer predisposition syndrome should always be considered[6][7][8].

see also Ovarian Tumors

Content in these topics is referenced in SCORE Ovarian and Adnexal Problems overview