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Pulmonary lymphangioleiomyomatosis and multiple hepatic angiomyolipomas in a man

Identifieur interne : 003327 ( Istex/Corpus ); précédent : 003326; suivant : 003328

Pulmonary lymphangioleiomyomatosis and multiple hepatic angiomyolipomas in a man

Auteurs : Na Rae Kim ; Man Pyo Chung ; Cheol Keun Park ; Kyung Soo Lee ; Joungho Han

Source :

RBID : ISTEX:C68BFD359CFDCE2B21F44D368A82BC920D8B2E59

English descriptors

Abstract

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.

Url:
DOI: 10.1046/j.1320-5463.2003.01460.x

Links to Exploration step

ISTEX:C68BFD359CFDCE2B21F44D368A82BC920D8B2E59

Le document en format XML

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<title type="main">Pulmonary lymphangioleiomyomatosis and multiple hepatic angiomyolipomas in a man</title>
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<p>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.</p>
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