Journal
JOURNAL OF CLINICAL INVESTIGATION
Volume 122, Issue 11, Pages 3807-3816Publisher
AMER SOC CLINICAL INVESTIGATION INC
DOI: 10.1172/JCI58709
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Funding
- Adler Foundation
- LAM Foundation
- LAM Treatment Alliance
- Tuberous Sclerosis Alliance
- Department of Defense Tuberous Sclerosis Complex Research Program
- National Institute of Diabetes and Digestive and Kidney Diseases
- Veteran's Administration
- National Heart, Lung and Blood Institute
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Lymphangioleiomyomatosis (LAM) is a rare progressive lung disease of women. LAM is caused by mutations in the tuberous sclerosis genes, resulting in activation of the mTOR complex 1 signaling network. Over the past 11 years, there has been remarkable progress in the understanding of LAM and rapid translation of this knowledge to an effective therapy. LAM pathogenic mechanisms mirror those of many forms of human cancer, including mutation, metabolic reprogramming, inappropriate growth and survival, metastasis via blood and lymphatic circulation, infiltration/invasion, sex steroid sensitivity, and local and remote tissue destruction. However, the smooth muscle cell that metastasizes, infiltrates, and destroys the lung in LAM arises from an unknown source and has an innocent histological appearance, with little evidence of proliferation. Thus, LAM is as an elegant, monogenic model of neoplasia, defying categorization as either benign or malignant.
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