Ito Fusahiko, Tanaka H, Oi K, Arai H, Sunamori M
Department of Cardiothoracic Surgery, Tokyo Medical and Dental University Graduate School of Medicine, Tokyo, Japan.
Kyobu Geka. 2006 Dec;59(13):1159-62.
Carney complex is a rare syndrome which includes cardiac myxoma, hyperactive endocrine neoplasm, spotty pigmented skin, and extracardiac myxomatous tumors. We report a case of a 26-year-old woman with Carney complex in whom recurrent multiple cardiac myxomas were resected 4 years after the first operation for left atrial (LA) myxoma. She had a history of left adrenalectomy in 1997 for Cushing syndrome due to primary pigmented nodular adrenocortical disease (PPNAD). In February 2001, she was diagnosed with Carney complex because of evidence of LA myxoma, her spotty pigmented skin lesions, her past history and family history of cardiac myxoma in her mother. Then, LA myxoma was successfully resected through the superior trans-septal approach and has been followed-up by ultrasound cardiography (UCG) every 6-month after discharge. In January 2005, UCG revealed 2 masses in the LA and the right ventricle outflow tract. The 2nd surgery was performed in February 2005. We found the 3rd myxoma during surgery, resembling a flat polyp in the LA just at the inflow of the right upper pulmonary vein. All 3 myxomas were successfully resected. Sixteen months after the 2nd operation, she has been doing well without any sign of recurrence of myxoma.
卡尼综合征是一种罕见的综合征，包括心脏黏液瘤、内分泌活性过高的肿瘤、斑点状色素沉着皮肤和心脏外黏液瘤样肿瘤。我们报告一例26岁患有卡尼综合征的女性病例，该患者在首次因左心房黏液瘤手术后4年，再次切除了多发复发性心脏黏液瘤。她曾于1997年因原发性色素沉着性结节性肾上腺皮质疾病（PPNAD）导致的库欣综合征接受左肾上腺切除术。2001年2月，由于存在左心房黏液瘤、斑点状色素沉着皮肤病变、既往史以及其母亲有心脏黏液瘤家族史，她被诊断为卡尼综合征。随后，通过经房间隔上入路成功切除左心房黏液瘤，出院后每6个月进行超声心动图（UCG）随访。2005年1月，UCG显示左心房和右心室流出道有2个肿块。2005年2月进行了第二次手术。术中我们发现了第三个黏液瘤，位于左心房，恰似右上肺静脉入口处的扁平息肉样。所有3个黏液瘤均被成功切除。第二次手术后16个月，她情况良好，无黏液瘤复发迹象。
