Pulmonary lymphangioleiomyomatosis in tuberous sclerosis. A case report.

Pulmonary lymphangioleiomyomatosis (PLAM) is a rare disease that exclusively affects young women of reproductive age. It is characterized by widespread pulmonary proliferation of abnormal, "immature smooth muscle cells (lam cells) leading to cystic destruction of the lung parenchyma. Lam occurs frequently in the thoracic duct and in axial lymph nodes, mediastinal or retroperitoneal. It can occur either in association with tuberous sclerosis complex (TSC-LAM) or without TSC (sporadic LAM). A case of TSC-LAM is reported, and the histogenesis and the role of lymphangiogenesis in the progression of disease is discussed.