NYMC Faculty Publications
Lymphangioleiomyomatosis: A Metastatic Lung Disease
Author Type(s)
Faculty
DOI
10.1152/ajpcell.00202.2022
Journal Title
American Journal of Physiology. Cell Physiology
First Page
C320
Last Page
C326
Document Type
Article
Publication Date
2-1-2023
Department
Cell Biology and Anatomy
Abstract
Lymphangioleiomyomatosis (LAM) is a rare disease affecting women, caused by somatic mutations in the TSC1 or TSC2 genes, and driven by estrogen. Similar to many cancers, it is metastatic, primarily to the lung. Despite its monogenetic nature, like many cancers, LAM is a heterogeneous disease. The cellular constituents of LAM are very diverse, including mesenchymal, epithelial, endothelial, and immune cells. LAM is characterized by dysregulation of many cell signaling pathways, distinct populations of LAM cells, and a rich microenvironment, in which the immune system appears to play an important role. This review delineates the heterogeneity of LAM and focuses on the metastatic features of LAM, the deregulated signaling mechanisms and the tumor microenvironment. Understanding the tumor-host interaction in LAM may provide insights into the development of new therapeutic strategies, which could be combinatorial or superlative to Sirolimus, the current U.S. Food and Drug Administration-approved treatment.
Recommended Citation
Kundu, N., & Holz, M. K. (2023). Lymphangioleiomyomatosis: A Metastatic Lung Disease. American Journal of Physiology. Cell Physiology, 324 (2), C320-C326. https://doi.org/10.1152/ajpcell.00202.2022