Spontaneous Rupture of Renal Angiomyolipoma in a Female Tuberous Sclerosis Patient with Pulmonary Lymphangioleiomyomatosis / 대한비뇨기과학회지

ABSTRACT

Pulmonary lymphangioleiomyomatosis (LAM) is uncommon debilitating disease, predominantly affecting women of childbearing age. Renal angiomyolipoma (AML) is the most common renal lesion in patients with tuberous sclerosis (TS), but, even without TS, is reported in up to 60% of the patients with LAM, which is frequently diagnosed several years before the LAM itself. However, it is extremely rare for renal AML and pulmonary LAM to simultaneously present in tuberous sclerosis complex. Herein, a case of spontaneous rupture of a renal AML, accompanied by pulmonary LAM, in a reproductive female with TS, managed by selective transcatheter arterial embolization (TAE), is reported.