長期経過観察した結節性硬化症に伴った腎血管筋脂肪腫自然破裂の1例

Abstract

46歳女.妊娠時にエコーにより両側腎血管筋脂肪腫(AML)を指摘されていたが, 加療せず外来により経過観察をしていた.右腎AML破裂により腫傷塞栓術を施行した.左腎AMLに対しては特に塞栓術など行わず, 外来経過観察をしていた.約10年間無症状で経過していたが, 左側腹部痛及び腹部膨満感を主訴に受診し, 左AML破裂の疑いで緊急入院した.輸血等の対症療法を行ったが, 高度の貧血の進行を認めた.既に止血はされていたものと考えたが再出血予防の意味も含め, 腫瘍を支配する動脈を金属コイルを用いて超選択的に栓塞した.DICを併発した左AML破裂の診断のもと腫瘍塞栓術を行った.DICに対してはメシル酸ガベキサートの点滴静注を行った.塞栓術後, 症状, 検査所見は速やかに改善した.術後造影CTでは腫瘍は造影されず, 塞栓術は有効であったと思われた

We present a case of spontaneous rupture of bilateral renal angiomyolipoma (AML) with tuberous sclerosis. A 46-year-old woman was admitted with sudden onset of severe left flank pain. She had been diagnosed to have bilateral AML with tuberous sclerosis 15 years earlier. Four years after the initial diagnosis, spontaneous rupture of right renal AML occurred and right renal embolization of the right renal artery was performed. The treatment for the left renal AML was not performed. Eleven years later in 2000, spontaneous rupture of contralateral renal AML occurred and left renal embolization of the left renal artery was performed. We evaluated the efficacy of selective arterial embolization in right AML and the change of the tumor size during a 10-year follow up after embolization. The right AML had decreased 86% in 11 years. Selective arterial embolization is an effective and safe treatment for AML. We evaluated the natural history of left AML and calculated the doubling time to be about 1, 370 days for the first period of 4 years and about 2, 075 days for the second period of 11 years. Although the growth change was very slow, we should observe the tumors carefully on computed tomography or ultrasound to prevent life-threatening hemorrhage.