Large cell calcifying sertoli cell tumor (LCCSCT) is a subtype of Sertoli cell tumor with unique clinical associations. It is associated with Peutz-Jeghers Syndrome and is a component of Carney’s Complex. The tumor cells are arranged in cords, nests, and tubules separated by fibrous stroma containing foci of calcification.
CARNEY’S COMPLEX: A syndrome characterized by: lentigines of face; myxomas of skin, soft tissue, cardiac myxomas; blue nevi of skin; primary pigmented adrenocortical disease; pituitary adenomas; pigmented psammomatous schwannomas.
Molecular Genetics of Carney’s Complex:Genetic analysis has mapped Carney’s complex to 2p16 and 17q22-24. The 17q region harbors the tumor suppressor gene Protein kinase A - a vital component of many cellular signaling systems. Mutations in a subunit of protein kinase A have been found in many patients with Carney’s complex and in some sporadic endocrine tumors. Sandrini F, Stratakis C. Mol Genet Metab 2003 Feb; 78(2)83-92.return to Sex Cord & Stromal Tumors