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Large Cell Calcifying Sertoli Cell Tumor

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About 40% of large cell calcifying Sertoli cell tumors (LCCSCT) are associated with Carney complex.

CARNEY COMPLEX: A syndrome characterized by: lentigines of face; myxomas of heart, skin, soft tissue and elsewhere; myxoid fibroadenomas of breast; blue nevi of skin; primary pigmented adrenocortical disease with Cushing syndrome; growth hormone-producing pituitary adenomas; and pigmented psammomatous schwannomas.

A diagnosis of LCCST should prompt a workup to rule out the diagnosis of Carney complex because of life-threatening complications of cardiac myxomas that may be present.

Carney complex should not be confused with Carney triad which consists of a sporadic association of pulmonary chondromas, gastrointestinal stromal tumor, and paraganglioma.

Gross Pathology: The size ranges from 1 to 15 cm. The mean sizes for benign and malignant tumors are 1.4 cm and 5.4 cm respectively. Carney complex-associated tumors are usually small, bilateral, and multifocal and behave in a benign fashion. The cut surface is >tan-white with a gritty texture due to calcifications. Malignant tumors frequently show hemorrhage and necrosis.

The image shows psammomatous calcification surrounded by small nests and cords of tumor cells in a fibromyxoid stroma.

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