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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

French recommendations for the diagnosis and management of lymphangioleiomyomatosis.
Cottin V, Blanchard E, Kerjouan M, Lazor R, Reynaud-Gaubert M, Taille C, Uzunhan Y, Wemeau L, Andrejak C, Baud D, Bonniaud P, Brillet PY, Calender A, Chalabreysse L, Court-Fortune I, Desbaillets NP, Ferretti G, Guillemot A, Hardelin L, Kambouchner M, Leclerc V, Lederlin M, Malinge MC, Mancel A, Marchand-Adam S, Maury JM, Naccache JM, Nasser M, Nunes H, Pagnoux G, Prévot G, Rousset-Jablonski C, Rouviere O, Si-Mohamed S, Touraine R, Traclet J, Turquier S, Vagnarelli S, Ahmad K; OrphaLung network
Respir Med Res 2023 Jun;83:101010. Epub 2023 Mar 24 doi: 10.1016/j.resmer.2023.101010. PMID: 37087906
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
Recent advances in the pathobiology and clinical management of lymphangioleiomyomatosis.
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

Recent clinical studies

Diagnosis

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

Supplemental Content

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