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Pulmonary lymphangioleiomyomatosis and multiple hepatic angiomyolipomas in a man

2003; Wiley; Volume: 53; Issue: 4 Linguagem: Inglês

10.1046/j.1320-5463.2003.01460.x

1440-1827

Na Rae Kim, Man Pyo Chung, Cheol Keun Park, Kyung Soo Lee, Joung‐Ho Han,

Eosinophilic Disorders and Syndromes

Pulmonary lymphangioleiomyomatosis (LAM) is an uncommon disease that to this point has been known to occur exclusively in reproductive women. To our knowledge, only one case of pulmonary LAM that was proven pathologically has been reported in a genotypical and phenotypical man. Multiple occurrence of hepatic angiomyolipomas is also rare, and only six cases have been found in the literature. Here, we report a biological and phenotypical man who had pulmonary LAM and multiple hepatic angiomyolipomas, leading to a presumptive diagnosis of tuberous sclerosis. This unsual presentation further broadens the wide spectrum of various clinicopathological aspects of pulmonary lymphangioleiomyomatosis and hepatic angiomyolipoma. Here, we emphasize that multiple hepatic angiomyolipomas should be distinguished from hepatic tumors, particularly in an endemic area for hepatocellular carcinoma. Further, pulmonary lymphangioleiomyomatosis can be a cause of cystic pulmonary disease even in a man.