D efinition — Ovarian involvement is a rare metastatic presentation of this tumor. Clinical Features Epidemiology Most common mesenchymal tumor of the gastrointestinal tract. Driven by oncogenic KIT or PDGFRA mutations. Most common is an in-frame mutation of exon 11…
D efinition — An uncommon spindled mesenchymal neoplasm of variable behavior with a NAB2-STAT6 fusion gene arising in the submesothelial mesenchyme. Clinical Features Epidemiology Uncommon; originally described in pleura, but now reported in multiple soft tissue sites including the pelvis.…
D efinition — A sex cord stromal tumor with annular tubules and features of both granulosa and Sertoli cell tumors. Clinical Features Epidemiology These are rare tumors, comprising about 6% of sex cord stromal tumors and less than 1% of…
D efinition — A malignant small cell ovarian tumor with features of pulmonary neuroendocrine carcinomas. Clinical Features Epidemiology Mean age of 48 years, which is over 20 years older than the small cell carcinoma of hypercalcemic type (SCCHT). Not associated…
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.…