Summary: A disease characterized by the progressive invasion of SMOOTH MUSCLE CELLS into the LYMPHATIC VESSELS, and the BLOOD VESSELS. The majority of the cases occur in the LUNGS of women of child-bearing age, eventually blocking the flow of air, blood, and lymph. The common symptom is shortness of breath (DYSPNEA).
Johnson S, Cordier J, Lazor R, Cottin V, Costabel U, Harari S, et al
. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 2010;35:14-26 pubmed publisher
Cudzilo C, Szczesniak R, Brody A, Rattan M, Krueger D, Bissler J, et al
. Lymphangioleiomyomatosis screening in women with tuberous sclerosis. Chest. 2013;144:578-585 pubmed publisher
b>Lymphangioleiomyomatosis (LAM) occurs in at least 40% of women with tuberous sclerosis complex (TSC), as diagnosed based on chest CT scan findings. Early identification may inform lifestyle choices and treatment decisions...
Gopinath D, Attarbashi S, Reid F, Seif M. Extrapulmonary lymphangioleiomyomatosis complicated by vesicovaginal fistula. J Obstet Gynaecol. 2013;33:910-2 pubmed publisher
Nishino K, Yoshimi K, Shibuya T, Hayashi T, Mitani K, Kobayashi E, et al
. Protein-losing Enteropathy Caused by Intestinal or Colonic Lymphangiectasia Complicated by Sporadic Lymphangioleiomyomatosis: A Report of Two Cases. Intern Med. 2017;56:943-948 pubmed publisher
This report describes two patients with sporadic lymphangioleiomyomatosis complicated by protein-losing enteropathy (PLE). Imaging studies indicated retroperitoneal lymphangioleiomyomas and abnormalities of the adjacent digestive tract...
Dongre A, Clements D, Fisher A, Johnson S. Cathepsin K in Lymphangioleiomyomatosis: LAM Cell-Fibroblast Interactions Enhance Protease Activity by Extracellular Acidification. Am J Pathol. 2017;187:1750-1762 pubmed publisher
b>Lymphangioleiomyomatosis (LAM) is a rare disease in which LAM cells and fibroblasts form lung nodules and it is hypothesized that LAM nodule-derived proteases cause cyst formation and tissue damage...
Avila N, Dwyer A, Moss J. Active Surveillance of Nonfatty Renal Masses in Patients With Lymphangioleiomyomatosis: Use of CT Features and Patterns of Growth to Differentiate Angiomyolipoma From Renal Cancer. AJR Am J Roentgenol. 2017;209:611-619 pubmed publisher
..our experience with active surveillance of nonfatty renal masses in a large cohort of patients with lymphangioleiomyomatosis (LAM), correlate their CT features and patterns of growth with histopathology results, and provide ..
Bissler J, Kingswood J, Radzikowska E, Zonnenberg B, Belousova E, Frost M, et al
. Everolimus long-term use in patients with tuberous sclerosis complex: Four-year update of the EXIST-2 study. PLoS ONE. 2017;12:e0180939 pubmed publisher
..everolimus in patients with renal angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis. Following favorable results from the double-blind core phase of EXIST-2 (NCT00790400), patients were ..
Julian L, Delaney S, Wang Y, Goldberg A, Dore C, Yockell Lelièvre J, et al
. Human Pluripotent Stem Cell-Derived TSC2-Haploinsufficient Smooth Muscle Cells Recapitulate Features of Lymphangioleiomyomatosis. Cancer Res. 2017;77:5491-5502 pubmed publisher
b>Lymphangioleiomyomatosis (LAM) is a progressive destructive neoplasm of the lung associated with inactivating mutations in the TSC1 or TSC2 tumor suppressor genes...