ABSTRACT
The authors report on a premenopausal female hemodialysis patient with relapsing pneumothorax, in whom the diagnosis of pulmonary lymphangioleiomyomatosis (LAM) was made. Ten years earlier, she had retroperitoneal bleeding from a kidney tumor corresponding to an angiomyolipoma (AML). The association between renal AML and pulmonary LAM is reviewed. Renal AML represents the most frequent extrapulmonary manifestation of pulmonary LAM. It is found in 32% to 60 % of cases in which a systematic search with abdominal computed tomography (CT) scan is done. The latter approach is advised to help avoid complications caused by renal AML. Therapeutic recommendations for renal AML are based on tumor size or presence of symptoms. Conversely, premenopausal women presenting with AML should be investigated for associated pulmonary LAM with high-resolution CT scan. Because LAM is very likely estrogen dependent, one of the several proposed antiestrogen therapies should be considered. Finally, there is significant overlap between renal AML, pulmonary LAM, and tuberous sclerosis. The latter should therefore be actively searched for in case of either AML or LAM.
