子宮に血管筋脂肪腫をきたし急激な転帰をたどった結節性硬化症の1例

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We report here a patient suffering from the tuberous sclerosis complex with an angiomyolipoma of the uterus. In July 1997, a 31-year-old woman,gravida 0, para 0, visited our hospital because of severe left flank pain. She had a characteristic sebaceous adenoma on her face and a subungual fibroma. A left renal angiomyolipoma and intraventricular calcification were recognized by CT and MRI. A diagnosis of tuberous sclerosis complex was therefore made and left nephrectomy was performed. In March 2000, she again visited our hospital complaining of severe lower abdominal pain. MRI showed a giant tumor of the uterine corpus. Total hysterectomy, bilateral salpingectomy and right oophorectomy were performed. The histopathological diagnosis was angiomyolipoma, but fatty and vascular components were absent. In July 2001, she developed respiratory insufficiency due to pulmonary lymphangiomyomatosis and died of septic shock. No autopsy was performed.
社団法人日本産科婦人科学会の論文
2002-11-01