Carney's Complex with Familial Atrial Myxoma.
- Author:
Ji Yong PARK
;
Han Dong YOO
;
Nack In KIM
- Publication Type:Original Article
- MeSH:
Diagnosis;
Female;
Humans;
Mothers;
Myxoma*;
Neurilemmoma;
Nipples;
Peripheral Nervous System Neoplasms;
Pigmentation;
Siblings;
Skin;
Young Adult
- From:Korean Journal of Dermatology
1998;36(5):942-945
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
The Carneys complex is a multisystemic tumorous disorder that features myxoma(heart, skin, and breast), spotty skin pigmentation(cutaneous and mucocutaneous), endocrine tumors(adrenal, testicular, and pituitary) and peripheral nerve tumors. The criteria far diagnosis of the complex is the presence of two or more of the above conditions. The condition is transmitted as an autosomal dominant trait. The most serious components of the syndrome are cardiac myxoma and psammomatous melanotic schwannoma. The cutaneous manifestations are a major clue in the disorder. We report a case of Carneys complex in a 19-year-old woman. She had spotty pigmentation on her face, left atrial myxoma, and myxoma on her right nipple. Her mother and sister had left atrial myxoma.
