U.S. flag

An official website of the United States government


Send to:

Choose Destination

Lung lymphangioleiomyomatosis

MedGen UID:
Concept ID:
Neoplastic Process
Synonyms: LAM; Lung Lymphangioleiomyomatosis; lung lymphangioleiomyomatosis; Lung Lymphangiomyomatosis; lung lymphangiomyomatosis; lymphangioleiomyomatosis; Lymphangioleiomyomatosis; Pulmonary lymphangioleiomyomatosis; pulmonary lymphangioleiomyomatosis; pulmonary lymphangiomyomatosis
SNOMED CT: Pulmonary lymphangioleiomyomatosis (277844007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
Concept ID:
Source: Orphanet
clinical entity without genetic inheritance.
Monarch Initiative: MONDO:0006277
Orphanet: ORPHA538


A rare, multiple cystic lung disease characterized by progressive cystic destruction of the lung and lymphatic abnormalities, frequently associated with renal angiomyolipomas (AMLs). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLung lymphangioleiomyomatosis

Professional guidelines


McCarthy C, Gupta N, Johnson SR, Yu JJ, McCormack FX
Lancet Respir Med 2021 Nov;9(11):1313-1327. Epub 2021 Aug 27 doi: 10.1016/S2213-2600(21)00228-9. PMID: 34461049
Harari S, Spagnolo P, Cocconcelli E, Luisi F, Cottin V
Curr Opin Pulm Med 2018 Sep;24(5):469-476. doi: 10.1097/MCP.0000000000000502. PMID: 29927757
Park S, Lee EJ
Korean J Intern Med 2017 Mar;32(2):229-238. Epub 2017 Feb 28 doi: 10.3904/kjim.2016.242. PMID: 28264540Free PMC Article

Recent clinical studies


Zhao L, Anders KH
Arch Pathol Lab Med 2014 Sep;138(9):1238-41. doi: 10.5858/arpa.2013-0092-CR. PMID: 25171708
Huang Y, Lu G, Quan J, Sun H, Li H, Hu H, Guo J
Ann Diagn Pathol 2011 Dec;15(6):427-30. Epub 2010 Oct 30 doi: 10.1016/j.anndiagpath.2010.07.006. PMID: 21036638

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...