先天性眼白皮症に心臓粘液腫を併発した1症例

A case of cardiac myxoma associated with congenital occulocutaneous albinism

　原発性心臓腫瘍は心エコーなどの診断技術の向上により生前に発見されることが多くなりつつあるが，未だ比較的稀な疾患である。左心房粘液腫は30〜50％を占め原発性心臓腫瘍の中でもその発生頻度は高い^1）が，皮膚疾患との合併となると非常に稀である。これまでに，NAME （nevus, atrial myxomna, myxoid neurofibroma，and ephelides） syndrome・LAMB （lentigines, atrialmyxoma, mucocutaneous myxomas, and blue nevi）syndrome^2）が認められている。今回，我々は先天性眼白皮症に左心房粘液腫を併発した中年女性症例を経験したので若干の文献的考察を加えて報告する。

This case is a 43 year-old-woman of left atrial myxoma associated with oculocutaneous albinism, who was admitted to our hospital because of noc-turnal cough everyday. Electrocardiogram showed normal sinus rhythm and complete right bundle branch block. Chest X-ray disclosed slight enlarge-ment of left atrium. Echocardiography revealed the left atrial pedunculated tumor, arising from atrial septum, prolapsing into left ventricle during dias-tole, which was coincided with lucent tumor by levophase of pulmonary arteriography. Feeder ar-tery of this tumor was visualized by right coronary arteriography. Cardiac catheterization represented normal pressure of right-sided heart. Thus the diagnosis of left atrial myxoma was established.

At the operation, radical excision of the base of tumor and septum and patch closure was undergone. The gross morphology and microscopic findings were consistent with myxoma.