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D efinition — A malignant rhabdoid tumor associated with hypercalcemia and mutations and inactivation in the SMARCA4 gene. Clinical Features Epidemiology Presents between childhood and menopause, predominating in the late teens and early twenties. Rarely familial, but kindreds with mutations…

D efinition — Variant of sex cord–stromal tumor of the ovary. Clinical Features Epidemiology Uncommon; accounts for less than 2% of all sex cord–stromal tumors. Predominant in the second and third decades. You’re Reading a Preview Become a Clinical Tree…

D efinition — A benign steroid-producing tumor of the ovary seen in pregnancy. Clinical Features Epidemiology Rare. History of multiple pregnancies is common. More common in black women. You’re Reading a Preview Become a Clinical Tree membership for Full access…

D efinition — A benign, steroid-producing tumor of the ovary. Clinical Features Epidemiology Rare overall, but accounts for about one fourth of ovarian steroid cell tumors. Occurs in women in their 50s and 60s, as with all ovarian steroid cell…

D efinition — A steroid cell tumor of the ovary arising in hilus cells and characterized by the presence of crystalloids of Reinke. Clinical Features Epidemiology Uncommon overall, but represents about one fifth of all ovarian steroid cell tumors. Patient…

PITFALL D efinition — A sex cord stromal tumor with testicular epithelial (Sertoli) and interstitial (Leydig) cell differentiation and a retiform architecture. Clinical Features Epidemiology These are rare variants of Sertoli-Leydig cell tumors (SLCTs), comprising less than 1:2000 ovarian neoplasms.…

D efinition — A sex cord–stromal tumor with testicular epithelial (Sertoli) and interstitial (Leydig) cell differentiation. Clinical Features Epidemiology These are rare tumors, comprising 1 : 2000 ovarian neoplasms. Usually seen in the second to fourth decades of life, but can occur…

PITFALL D efinition — A sex cord–stromal tumor seen in the first decades of life that closely resembles the developing follicle. Clinical Features Epidemiology Granulosa cell tumors (GCTs) comprise less than 2% of ovarian tumors. Juvenile GCTs comprise less than…

PITFALL D efinition — Diagnostically challenging variant patterns include those with nuclear atypia, cystic architecture, thecoma-like, luteinized, and sertoliform. Clinical Features Epidemiology Uncommon. The granulosa cell tumor (GCT) comprises less than 2% of ovarian tumors. Over 90% will be adult…

D efinition — An ovarian steroid-producing sex cord–stromal tumor composed of cells resembling the granulosa cells of the developing follicle. Clinical Features Epidemiology Uncommon. Perimenopausal or postmenopausal women. Mean age at presentation is in the mid-50s. You’re Reading a Preview…