结节性硬化症患者中，巨大血管平滑肌脂肪瘤伴显著肺部病变，提示淋巴管平滑肌瘤病。
Giant angiomyolipoma associated with marked pulmonary lesions suggesting lymphangioleiomyomatosis in a patient with tuberous sclerosis.
作者信息
Tüzel E, Kirkali Z, Mungan U, Cüler C, Sade M
机构信息
Dokuz Eylül University, School of Medicine, Department of Urology, Inciralti, Izmir, Turkey.
出版信息
Int Urol Nephrol. 2000;32(2):219-22. doi: 10.1023/a:1007183001681.
The association between Tuberous Sclerosis (TS) and Angiomyolipoma (AML) is well known. A patient with TS and giant AML mimicking Renal Cell Carcinoma (RCC), measuring 29 x 18 x 11 cm, weighing 4700 gr is presented. Imaging studies revealed coexistent pulmonary lymphangioleiomyomatosis and concurrent renal and pulmonary involvement is extremely rare in patients in TS. We believe that the growth potential of this hamartomatous lesion may reach to a life threatening size.
结节性硬化症（TS）与肾血管平滑肌脂肪瘤（AML）之间的关联已广为人知。本文报告了一名患有TS且有巨大AML（其大小为29×18×11厘米，重4700克，酷似肾细胞癌（RCC））的患者。影像学研究显示同时存在肺淋巴管平滑肌瘤病，而TS患者出现肾脏和肺部同时受累的情况极为罕见。我们认为这种错构瘤性病变的生长潜能可能会达到危及生命的大小。
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