Abbreviations and AcronymsEMA = epithelial membrane antigen GCT = granulosa cell tumor PgR = progesterone receptors SMA = smooth muscle actin ABSTRACT A primary extragonadal granulosa cell tumor (GCT) is very rare. It is believed to arise from the ectopic gonadal tissue along the embryonal route of the genital ridge. We encountered one such tumor in a 45-year-old female who presented with a large intraabdominal mass. She had an abdominal hysterectomy with unilateral salpingooophorectomy 10 years previously for dysfunctional uterine bleeding. She underwent exploratory laparotomy and excision of the mass. The histologic features failed to differentiate between a carcinoid and granulosa celltumor. Immunohistochemistry showed that the neoplastic cells expressed calretinin, smooth muscle actin, melan-A, inhibin-alpha, and progesterone receptors and were negative for desmin, CD10, cytokeratin, epithelial membrane antigen, synaptophysin, or chromogranin-A. An English literature search revealed only 4 similar cases, primarily arising from the retroperitoneum. Immunohistochemistry helps to differentiate GCTs from other tumors.
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