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Large Cell Calcifying Sertoli Cell Tumor

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Molecular Genetics: Large cell calcifying Sertoli cell tumors in the setting of Carney complex show germline mutations in the tumor suppressor gene PRKAR1A on chromosome 17q22-24 in 60-70% of patients. Some sporadic tumors show acquired mutations in the same location.

PRKAR1A codes for a critical component of type I protein kinase A - which is the main mediator of cAMP signaling in mammals.

In this image, the tumor cells have abundant eosinophilic cytoplasm. The nuclei lack pleomorphism and are round to oval with punctate nucleoli. Mitotic activity is not increased. Intratubular tumor is seen in about half of the cases.

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