Another example of an atrial myxoma. The tumor is lobulated and polypoid with soft gelatinous appearance. Note the white stalk - the point of attachment of the tumor to the atrial septum. When myxomas occur in the familial setting, extra-cardiac manifestations are seen in about 20% of cases. Together, they make up the Carney Complex and consist of: 1) Primary pigmented nodular adrenocortical dysplasia resulting in Cushing syndrome; 2) Lentigines, ephelides, and blue nevi of the skin and mucosae; 3) Myxomas of skin, heart, breast (myxoid mammary fibroadenomas), and other locations;4) Other tumors, including psammomatous melanotic schwannoma, large cell calcifying Sertoli cell tumor, and growth hormone-producing pituitary adenoma. About 7% of cardiac myxomas are associated with Carney Complex. The etiology of Carney Complex has been localized to protein kinase A regulatory subunit gene PRKAR1A on chromosome 17q23-24. Germline inactivating mutations in this gene have been found in about 70% of individuals with Carney Complex. Image Copyright: pathorama.ch.