General
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• 5% of ovarian neoplasms, 7% of malignant ovarian neoplasms
• Derived from ovarian stroma, itself derived from sex cords of embryonic gonad (predecessor of Sertoli, Leydig, granulosa and theca cells)
• Theca cells produce estrogens; Leydig cells produce androgens
• Morphology and clinically evident hormonal activity may go together, but classify based on morphology

Positive stains
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• Alpha-inhibin, mullerian inhibiting substance, alpha-inhibin (> 95%), calretinin (more sensitive, less specific than inhibin, Am J Surg Pathol 2002;26:1477), CD99 (Arch Pathol Lab Med 2000;124:563), MART1 / MelanA and WT-1

Negative stains
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• EMA, PLAP and CEA
• Note: anti-Mullerian hormone staining is present in only a small percentage of granulosa or Sertoli tumor cells, but is specific for these tumors (Hum Pathol 2000;31:1202), particularly compared to endometrioid tumors (Hum Pathol 1998;29:840)
• Strong alpha-inhibin staining is relatively specific for ovarian sex-cord stromal proliferations, although luteinized cells from other tumors are also immunoreactiveb (Hum Pathol 1997;28:1387, Am J Surg Pathol 1997;21:583)

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