Spontaneous pneumothorax occurs less in females than in males, in contrast to the relatively more commonly and incidentally found renal angiomyolipoma (AML). We report a renal AML in a 23-year-old female patient, which presented as right palpable abdominal mass. This renal tumor was removed owing to enlargement and internal hemorrhage and AML was proved by pathological examination. Three years later, she experienced a life-threatening spontaneous tension pneumothorax and pulmonary lymphangioleiomyomatosis (LAM), which was diagnosed by chest computed tomography. Clinically, she did not have tuberous sclerosis. Pulmonary LAM and renal AML are related lesions and both of these proliferative lesions occur in sporadic patients without family history and at much higher frequency in patients with tuberous sclerosis. Because of the nonspecific symptoms and high rate of complications, we should consider the possibility of LAM in patients diagnosed with AML. The association between renal AML and pulmonary LAM is reviewed.
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