肝・腎血管筋脂肪腫とリンパ脈管筋腫症を合併した結節性硬化症

Tuberous sclerosis complex with lympangioleiomyomatosis and angiomyolipomas of the kidney and liver：a case report

症例は26歳，女性。結節性硬化症，両側腎血管筋脂肪腫で経過観察されていた。同時期に両側の腎血管筋脂肪腫の破裂を認め，それぞれ，血管塞栓術を施行した。その後，さらに気胸を認め，リンパ脈管筋腫症も合併していると診断された。

We herein report a case of tuberous sclerosis complex with lympangioleiomyomatosis and angiomyolipomas of the kidney and liver. A 26-year-old woman visited our hospital because of the renal tumor. Abdominal CT revealed bilateral angiomyolipomas and cranial CT revealed calcification of the cerebral ventricles. She had no symptom of tuberous sclerosis. Six years later, she developed right flank pain and was diagnosed as right renal ruptute, for which selective transarterial embolotherapy was performed In the same year, she received selective transarterial embolotherapy because of the left kidney rupture. In the following year, she had attacks of the pneumothorax. Chest CT revealed cystic formations throughout the lungs, consistent with lympangioleiomyomatosis.