TY - CHAP M1 - Book, Section TI - Pulmonary Lymphangioleiomyomatosis A1 - Taveira-DaSilva, Angelo M. A1 - King, Talmadge E. A1 - Moss, Joel A2 - Grippi, Michael A. A2 - Elias, Jack A. A2 - Fishman, Jay A. A2 - Kotloff, Robert M. A2 - Pack, Allan I. A2 - Senior, Robert M. A2 - Siegel, Mark D. PY - 2015 T2 - Fishman's Pulmonary Diseases and Disorders, 5e AB - Lymphangioleiomyomatosis (LAM) is a multisystem disorder, predominantly affecting women, which is characterized by cystic lung lesions, abdominal angiomyolipomas (AML) and lymphatic abnormalities, for example, lymphatic tumors, chylous effusions.1–5 These pathologic features are caused by the proliferation of a neoplastic smooth muscle–like LAM cell that also has characteristics of melanocytes.6 Inherited and sporadic forms of LAM have been described. Sporadic LAM is caused by somatic mutations in an unknown susceptible cell of the tuberous sclerosis complex (TSC) 2 (TSC2) gene.7,8 LAM also occurs in TSC, an autosomal dominant disorder resulting from germline mutations in the TSC1 or TSC2 genes that is characterized by widespread hamartomas in several organs including the brain, heart, skin, kidney, eyes, lung, and liver, and occurs in 1 of 6000 live births.9 SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accessmedicine.mhmedical.com/content.aspx?aid=1122361404 ER -