Pathomorphological Characteristic of Pulmonary Lymphangioleiomyomatosis

Abstract

Lymphangioleiomyomatosis (LAM) is a polysystemic disease based on the formation of granulomas prone to degenerate into cysts in the lungs, abdominal and pelvic organs. Lung involvement in LAM is accompanied by respiratory dysfunction and can be fatal. WHO classifies this disease into a group of mesenchymal tumors and considers it as a low-grade destructive metastasizing neoplasm. LAM is a rare disease and earlier it was only detected in women, but nowadays cases of pulmonary LAM in men (include teenagers) have been described. The etiology of LAM and the inducing factors are still unknown. Objective – to collect and systematize data on pathomorphological characteristic of changes in lung tissue in LAM to evaluate the completeness and sufficiency of the available data. Material and methods. There were analyzed 912 articles and monographs (including duplicates) from PubMed, eLibrary, and Cyberleninka databases over a fifty-year period from 1973 to August of 2023, with an emphasis on more relevant publications and articles in highly rated scientific journals that include information on the review topic. Eventually 55 original sources were selected. Results. The article presents summary data on macroscopic and microscopic picture of pulmonary LAM, data on morphological and antigenic characterization of cells forming the basis of granulomas and their surroundings, the results of experiments demonstrating dynamics of pathological changes in LAM. Conclusion. Despite the relatively large amount of data about morphological characteristics that is enough for diagnosis, he main issue is still open: what is the origin of LAM cells? A systematic pathomorphology analyze can help us to find an answer for this question.