{"title":"Lymphangioleiomyomatosis","authors":"Davide Elia , Roberto Cassandro , Antonella Caminati , Francesca Luisi , Sergio Harari","doi":"10.1016/j.lpm.2023.104173","DOIUrl":null,"url":null,"abstract":"<div><p><span><span>Lymphangioleiomyomatosis (LAM) is an ultra-rare, slowly progressive neoplastic cystic disease, belonging to the group of PEComas. It can occur sporadically or associated to </span>tuberous sclerosis complex disease and affects mainly women in child-birth age. Dyspnoea is the most frequent symptom referred to the time of diagnosis, however </span>spontaneous pneumothorax<span> may be a typical presentation associated to extrathoracic manifestations, such as renal angiomyolipomas<span>. In the last decade, important advances in understanding molecular mechanisms underlying the LAM pathogenesis have been reached. It has allowed to obtain improvements in the research of novel biomarkers, treatment and a better management of the disease.</span></span></p></div>","PeriodicalId":20530,"journal":{"name":"Presse Medicale","volume":"52 3","pages":"Article 104173"},"PeriodicalIF":3.2000,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Presse Medicale","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0755498223000106","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
引用次数: 0
Abstract
Lymphangioleiomyomatosis (LAM) is an ultra-rare, slowly progressive neoplastic cystic disease, belonging to the group of PEComas. It can occur sporadically or associated to tuberous sclerosis complex disease and affects mainly women in child-birth age. Dyspnoea is the most frequent symptom referred to the time of diagnosis, however spontaneous pneumothorax may be a typical presentation associated to extrathoracic manifestations, such as renal angiomyolipomas. In the last decade, important advances in understanding molecular mechanisms underlying the LAM pathogenesis have been reached. It has allowed to obtain improvements in the research of novel biomarkers, treatment and a better management of the disease.
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