Eleven Cardiac Operations for Recurrent Cardiac Myxomas with Typical Endocrinal Tumors in a Family : Carney Complex

Sung Joon Han, Hao Jun Cui, Roknuggaman, Hyun Jin Cho, Min-Woong Kang, Jae Hyeon Yu, Shinkwang Kang, Myung Hoon Na

Department of Thoracic and Cardiovascular Surgery, Chungnam National University Hospital, Chungnam National University College of Medicine, Daejeon, Korea

목적 : Intracardiac myxoma is categorized by sporadic cardiac myxoma and familial cardiac myxoma. Carney complex is familial cardiac myxoma, is inherited as an autosomal dominant trait, and characterized by cardiac myxoma, skin pigmentation and endocrine abnormalities. We report a case of familial cardiac myxoma, in mother, two sons and daughter. They have various endocrinologic disorders and recurrent cardiac myxomas. The specific relation between genotype and clinical status was not found.

방법 : Clinical data was obtained from medical records retrospectively. Genetic mutation analysis was performed in all patients with Carney complex.

결과 : Eleven times of cardiac operation for recurrent myxomas had been performed. There are three times operations in the mother, seven times in the two sons and once in the daughter. There were nine endocrinologic disorders diagnosed, paraganglioma, brain bone tumor, schwannoma, peripheral nerve tumor, adrenal gland tumor, testis tumor, skin myxoma and pituitary adenoma in this family. Neither mutations of PRKAR1A(protein kinase, cAMP-dependent, regulatory, type I, alpha), Carney complex 1 and 2 could not be found in this cases in genetic analysis.

결론 : We report a typical family of Carney complex characterized with recurrent cardiac myxomas for eleven cardiac operations and diverse endocrinologic disorders.

책임저자: Myung Hoon Na

Department of Thoracic and Cardiovascular Surgery, Chungnam National University Hospital, Chungnam National University College of Medicine, Daejeon, Korea

발표자: Sung Joon Han, E-mail : hansungjoon@cnuh.co.kr