Giant renal angiomyolipomatosis in association with pulmonary lymphangiomyomatosis.

摘要

A 51-year-old woman was admitted to hospital with a 2-year history of intermittent exertional dyspnoea. High-resolution computed tomography (CT) of the lungs showed bilateral diffuse cystic changes with a maximal diameter of 15 mm (Fig. 1). Abdominal CT showed bilateral renal fat-density masses (massive in the right kidney) consistent with angiomyolipomas (Fig. 2), and a non-obstructing calculus in the right renal pelvis. Pulmonary function tests show decreased forced expiratory volume in 1 s. These findings were consistent with giant renal angiomyolipomas in association with pulmonary lymphangioleiomyomatosis (LAM), and raised the possibility of tuberous sclerosis. Family history and clinical signs of tuberous sclerosis were absent and cerebral magnetic resonance imaging was normal.