Carney syndrome is an autosomal dominant disorder involving a mutated protein kinase (PR.KARIA) that acts on a tumor suppressor gene on chromosome l 7q22-24. Cardiac myxomas (choice A) are present in 65% of patients and are often multiple and the most serious component of the disorder, causing death in 20% of patients. 25% of women with the syndrome have breast myxomas (choice B). Carney syndrome is associated with endocrine tumors involving the adrenal gland, pituitary, and testis. Both Sertoli cell tumors and steroid type tumors, including Leydig cell tumors ( choice D ), occur in the testis. An uncommon schwannoma variant ( choice E), psammomatous melanotic, is seen in the disorder. Carney syndrome is associated with skin pathology including myxomas and lentiginous and blue nevi, not spitz nevi (choice C). QCSP, Cardiac Myxoma and Carney Syndrome, p 9-10.
2. B. CALRETININ AND CD31
Myxomas are typically positive for the vascular markers CD31 and CD34, with 80% reactive for vimentin
and 20% reactive for cyrokeratin. The myxoma tumor cells stain variably for muscle markers including smooth muscle actin and will be positive for nonspecific histiocytic markers (lysozyme, a1 -antichymotrypsin, and a1 -antitrypsin), but negative for the specific histiocytic marker CD68. Recent studies have shown that the most helpful in1mtmohistochemical stain to differentiate myxomas from primary cardiac sarcomas is calretinin, which will be positive in the former and negative in the latter. Therefore the best answer is B.
QCSP, Cardiac Myxoma, p 9.
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