Cases Journal


Open Access Case Report

Tuberous sclerosis with pulmonary lymphangioleiomyomatosis and renal angiomyolipomas. Computed tomographic findings: a case report

Flávia G Vianna, Edson Marchiori*, Gláucia Zanetti, Claudia M Mano, Branca Sarcinelli-Luz, Juliana F Carvalho, Carla Assed, Isabella G Santos, Alair ASMD Santos and Alberto D Vianna

Author Affiliations

Department of Radiology, Faculty of Medicine, Fluminense Federal University, Rio de Janeiro, Brazil

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Cases Journal 2009, 2:9124 doi:10.1186/1757-1626-2-9124

Published: 1 December 2009

Abstract

The authors describe a case of a 31-year-old female with tuberous sclerosis, a genetic, rare, variably expressed disease. Clinical symptoms were chest pain, and progressive dyspnea. Computed tomography scan of the chest showed bilateral, diffuse, small thin-walled cysts scattered throughout the lungs characteristic for pulmonary lymphangioleiomyomatosis. Computed tomography scan of the abdomen revealed enlarged, heterogeneous kidneys, with low density tumors corresponding to angiomyolipomas. Pulmonary lymphangioleiomyomatosis and bilateral renal angiomyolipomas are some presentations of tuberous sclerosis and the coexistence of both conditions may cause devastating morbidity and mortality.