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Large Cell Calcifying Sertoli Cell Tumor

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Introduction: Large cell calcifying Sertoli cell tumor (LCCSCT) is a unique variant of Sertoli cell tumor characterized by abundant eosinophilic cytoplasm in the tumor cells and stromal calcification. It may be sporadic (60% of cases) or occur as part of Carney complex (40%) which is an autosomal dominant condition characterized by skin pigmentation, endocrinopathy, and multiple neoplasia.

Clinical Features: The age range is teenage years to young adults (median age at diagnosis is 20 years). Patients with Carney complex are diagnosed at a younger age due to other manifestations. The tumors are usually bilateral and multifocal in Carney complex and almost always unilateral and unifocal in sporadic cases.

The patients usually present with a testicular mass. Some Carney complex-associated patients develop gynecomastia and isosexual pseudoprecocity. This is due to hormones produced by either tumor cells or by hyperplastic Leydig cells.

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