Lymphangioleiomyomatosis of the Pulmonary Body: Pathogenesis, Pathomorphology, Problems of Treatment

Aim. To summarize modern literature data on pathophysiological mechanisms, morphological changes in lung lymphangioleiomyomatosis and to analyze our own clinical case. Key points. More than 120 sources of literature on this topic were studied, the sources that most fully corresponded to the aims of the article were selected for citation. Databases searched: PubMed, Scopus, Google Scholar, CyberLeninka, eLIBRARY.RU; keywords used: lymphangioleiomyomatosis, LAM, sirolimus, mTOR inhibitor. The depth of the search was 2004–2024. Current data on the peculiarities of pathogenesis, pathomorphologic manifestations and approaches to the treatment of LAM were analyzed. The case history of the patient who underwent treatment in Novgorod regional hospital with the diagnosis of lymphangioleiomyomatosis of the lungs was analyzed. Progressive course of lymphangioleiomyomatosis was observed, despite 3-year administration of mTOR blocker. Conclusion. Lymphangioleiomyomatosis is a rare disease affecting women caused by somatic mutations in TSC1 or TSC2 genes. The basic pathogenetic drug in the treatment of lymphangioleiomyomatosis to date is an mTOR inhibitor. However, lymphangioleiomyomatosis is characterized by dysregulation of many cellular signaling pathways. Their study and understanding of tumor-host interactions in lymphangioleiomyomatosis may lead to the development of new drugs and therapeutic strategies, including combination therapies superior to sirolimus. Keywords: pulmonary lymphangioleiomyomatosis, computed tomography, biopsy, mTOR inhibitors, sirolimus.