TY - JOUR
T1 - Growth hormone-producing pituitary macroadenoma, multiple epidermoid cysts, and right atrial myxoma
T2 - An unusual case of carney complex
AU - Iacobellis, Gianluca
PY - 2001/1/1
Y1 - 2001/1/1
N2 - Carney complex is a rare familial syndrome characterized by myxomas, endocrine tumors, schwannomas, and lentiginous pigmentation lesions. Endocrine overactivity includes Cushing disease, acromegaly, and thyroid and testis tumors. The syndrome is transmitted by an autosomal dominant trait. About 300 cases are known worldwide. The interesting finding in this patient is the presence of epidermoid cysts, which have not previously been reported as a feature of the Carney complex. Acromegaly as a component of Carney complex is not frequent, but it has been considered the only pituitary-related manifestation of the complex. Acromegaly and right atrial myxoma association is infrequent in this syndrome. The origin of Carney complex neoplasms, including pituitary adenoma and cardiac myxoma, remains unclear. A common neuroendocrine nature of Carney complex tumors is suggested. In addition, some similarities between multiple endocrine neoplasia type 1 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 myxoma and cutaneous lesions suggesting Carney complex in patients with acromegaly.
AB - Carney complex is a rare familial syndrome characterized by myxomas, endocrine tumors, schwannomas, and lentiginous pigmentation lesions. Endocrine overactivity includes Cushing disease, acromegaly, and thyroid and testis tumors. The syndrome is transmitted by an autosomal dominant trait. About 300 cases are known worldwide. The interesting finding in this patient is the presence of epidermoid cysts, which have not previously been reported as a feature of the Carney complex. Acromegaly as a component of Carney complex is not frequent, but it has been considered the only pituitary-related manifestation of the complex. Acromegaly and right atrial myxoma association is infrequent in this syndrome. The origin of Carney complex neoplasms, including pituitary adenoma and cardiac myxoma, remains unclear. A common neuroendocrine nature of Carney complex tumors is suggested. In addition, some similarities between multiple endocrine neoplasia type 1 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 myxoma and cutaneous lesions suggesting Carney complex in patients with acromegaly.
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U2 - 10.1097/00019616-200107000-00012
DO - 10.1097/00019616-200107000-00012
M3 - Article
AN - SCOPUS:0034888974
VL - 11
SP - 327
EP - 330
JO - Endocrinologist
JF - Endocrinologist
SN - 1051-2144
IS - 4
ER -