Document Detail

Targeted approaches toward understanding and treating pulmonary lymphangioleiomyomatosis (LAM).
MedLine Citation:
PMID:  23184699     Owner:  NLM     Status:  MEDLINE    
Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease found almost exclusively in women that is characterized by neoplastic growth of atypical smooth muscle-like cells in the lung, destruction of lung parenchyma, and obstruction of lymphatics. These processes lead to the formation of lung cysts, rupture of which results in spontaneous pneumothorax. Progression of LAM often results in loss of pulmonary function and death. LAM affects predominantly women of childbearing age and is exacerbated by pregnancy. The only proven treatment for LAM is lung transplantation, and even then LAM cells will often return to the transplanted lung. However, methodical and targeted approaches toward understanding LAM pathophysiology have led to the discovery of new potential therapeutic avenues. For example, the mutational inactivation of tumor suppressor complex genes tuberous sclerosis complex 1 or tuberous sclerosis complex 2 has been shown to be present in lung LAM cells. These mutations occur sporadically or in association with inherited hamartoma syndrome tuberous sclerosis (TSC). Since TSC genes function as negative regulators of the mammalian target of rapamycin, a major controller of cell growth, metabolism, and survival, rapamycin analogs have recently been used to treat LAM patients with promising results. Similarly, studies focusing on the importance of estrogen in LAM progression have suggested that anti-estrogen therapy might prove to be an alternative means of treating LAM. This minireview summarizes recent progress in understanding LAM pathophysiology, including the latest preclinical and clinical studies, and insights regarding the role of hormones in LAM.
Stephen R Hammes; Vera P Krymskaya
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.; Review     Date:  2012-11-27
Journal Detail:
Title:  Hormones & cancer     Volume:  4     ISSN:  1868-8500     ISO Abbreviation:  Horm Cancer     Publication Date:  2013 Apr 
Date Detail:
Created Date:  2013-02-25     Completed Date:  2013-09-03     Revised Date:  2014-04-02    
Medline Journal Info:
Nlm Unique ID:  101518427     Medline TA:  Horm Cancer     Country:  United States    
Other Details:
Languages:  eng     Pagination:  70-7     Citation Subset:  IM    
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MeSH Terms
Cell Proliferation
Disease Progression
Lung / cytology,  physiopathology*
Lung Transplantation
Lymphangioleiomyomatosis* / complications,  physiopathology,  therapy
Molecular Targeted Therapy*
Pneumothorax / complications,  physiopathology*
Rare Diseases
Tumor Suppressor Proteins / genetics*
Grant Support
Reg. No./Substance:
0/Tumor Suppressor Proteins; 0/tuberous sclerosis complex 1 protein

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