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Lung lymphangioleiomyomatosis

MedGen UID:
91161
•Concept ID:
C0349649
•
Neoplastic Process
Synonyms: LAM; Lung Lymphangioleiomyomatosis; lung lymphangioleiomyomatosis; Lung Lymphangiomyomatosis; lung lymphangiomyomatosis; Lymphangioleiomyomatosis; Pulmonary lymphangioleiomyomatosis; pulmonary lymphangioleiomyomatosis; pulmonary lymphangiomyomatosis
SNOMED CT: Pulmonary lymphangioleiomyomatosis (277844007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0006277
Orphanet: ORPHA538

Definition

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

PubMed

Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management.
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
Diagnosis and treatment of cystic lung disease.
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
Treatment of lymphangioleiomyomatosis and Camões.
Rufino R
J Bras Pneumol 2015 Jul-Aug;41(4):295-6. doi: 10.1590/S1806-37132015000400001. PMID: 26398747Free PMC Article

Recent clinical studies

Etiology

Hepatic perivascular epithelioid cell tumors: Benign, malignant, and uncertain malignant potential.
Amante MF
World J Gastroenterol 2024 May 14;30(18):2374-2378. doi: 10.3748/wjg.v30.i18.2374. PMID: 38764772Free PMC Article

Diagnosis

Hepatic perivascular epithelioid cell tumors: Benign, malignant, and uncertain malignant potential.
Amante MF
World J Gastroenterol 2024 May 14;30(18):2374-2378. doi: 10.3748/wjg.v30.i18.2374. PMID: 38764772Free PMC Article
Malignant perivascular epithelioid cell tumor of the gallbladder: a case report and review of literature.
Zhao L, Anders KH
Arch Pathol Lab Med 2014 Sep;138(9):1238-41. doi: 10.5858/arpa.2013-0092-CR. PMID: 25171708
Primary perivascular epithelioid cell tumor of the bladder.
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

Prognosis

Hepatic perivascular epithelioid cell tumors: Benign, malignant, and uncertain malignant potential.
Amante MF
World J Gastroenterol 2024 May 14;30(18):2374-2378. doi: 10.3748/wjg.v30.i18.2374. PMID: 38764772Free PMC Article

Clinical prediction guides

Hepatic perivascular epithelioid cell tumors: Benign, malignant, and uncertain malignant potential.
Amante MF
World J Gastroenterol 2024 May 14;30(18):2374-2378. doi: 10.3748/wjg.v30.i18.2374. PMID: 38764772Free PMC Article

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