The association of pulmonary lymphangioleiomyomatosis with renal and hepatic angiomyolipomas in a prepubertal girl: a previously unreported entity.

Pulmonary lymphangioleiomyomatosis (PLAM) is a rare, diffuse progressive interstitial lung disease that affects females of childbearing age and is characterized by diffuse proliferation of abnormal smooth muscle fibers predominantly developing in the lung and leading to cystic destruction. A prepubertal 13-year-old girl with PLAM associated with renal and hepatic angiomyolipomas who was treated by nephroureterectomy and thoracoscopic pleurodesis is presented. To the best of our knowledge, this is the first reported case of PLAM associated with renal and hepatic angiomyolipomas at the prepubertal age. After evaluating the clinicopathologic features of this rare entity, the authors conclude that PLAM should be considered in the differential diagnosis of cystic pulmonary pathologies in children, particularly teenagers. The most important clinical aid is to bear this rare entity in mind when a child presents with renal and/or hepatic angiomyolipomas. Thoracoscopic pleurodesis is the most effective treatment modality for recurrent pneumothoraces. (c) 2005 S. Karger AG, Basel.