• Presse Med · Sep 2023

    Review

    Lymphangioleiomyomatosis.

    • Davide Elia, Roberto Cassandro, Antonella Caminati, Francesca Luisi, and Sergio Harari.
    • UO di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, MultiMedica IRCCS, Via San Vittore 12, 20123 Milan, Italy.
    • Presse Med. 2023 Sep 1; 52 (3): 104173104173.

    AbstractLymphangioleiomyomatosis (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.Copyright © 2023 Elsevier Masson SAS. All rights reserved.

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