結節性硬化症に腎，肝，大網の血管筋脂肪腫を合併した1例

Tuberous sclerosis associated with angiomyolipomas of kidneys,liver,and omentum:Report of a case

患者は結節性硬化症の25歳，女性。2003年3月頃より体重減少，食欲低下を認め，6月頃より乾性咳嗽が出現したため近医を受診した。CTにて腹腔内腫瘍および大量の胸腹水貯留を認め，当科を紹介された。画像精査で肝，腎のほか，大網に巨大な腫瘍を認めた。大網腫瘍を経皮的針生検したところHMB-45陽性であり，血管筋脂肪腫と診断した。さらに，胸水の性状は乳糜状であり，肺リンパ管筋腫症が疑われた。

We report a case angiomyolipomas of kidney,liver,and omentum associated with tuberous sclerosis. A 25-year-old female with tuberous sclerosis noticed appetite loss and body weight loss since March 2003 and complained of dry cough in May 2003. CT scan revealed a huge intraperitoneal tumor and massive pleural effusion and ascites. She was introduced to our hospital for further examination. CT scan,MRI,and angiography were performed,which suggested multiple angiomyolipoma in the kidneys,liver,and omentum. Biopsy of the omentum confirmed the diagnosis with HMB-45 positive cells. Furthermore,pleural effusion was chyliform,which suggested pulmonary lymphangioleiomyomatosis.