Growth hormone-producing pituitary macroadenoma, multiple epidermoid cysts, and right atrial myxoma: An unusual case of Carney complex

Carney complex is a rare familial syndrome characterized by myxomas, endocr ine tumors, schwannomas, and lentiginous pigmentation lesions. Endocrine ov eractivity includes Cushing disease, acromegaly, and thyroid and testis tum ors. The syndrome is transmitted by an autosomal dominant trait. About 300 cases are known worldwide. The interesting finding in this patient is the p resence of epidermoid cysts, which have not previously been reported as a f eature of the Carney complex. Acromegaly as a component of Carney complex i s not frequent, but it has been considered the only pituitary. related mani festation of the complex. Acromegaly and right atrial myxoma association is infrequent in this syndrome. The origin of Carney complex neoplasms, inclu ding pituitary ade, noma and cardiac myxoma, remains unclear. A common neur oendocrine nature of Carney complex tumors is suggested. In addition, some similarities between multiple endocrine neoplasia type I and Carney complex have been found. We reported a case of a man with acromegaly, right atrial myxoma, lentigines, multiple cystic skin lesions, and thyroid nodules. We discovered a myxoma in this patient only after the diagnosis of acromegaly; it was asymptomatic. It is important to look for the presence of cardiac m yxoma and cutaneous lesions suggesting Carney complex in patients with acro megaly.