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lymphangioleiomyomatosis

^ http://purl.obolibrary.org/obo/MONDO_0011705

A multifocal neoplasm with perivascular epithelioid cell differentiation affecting almost exclusively females of child-bearing age. It is characterized by the presence of smooth muscle and epithelioid cells and by the proliferation of lymphatic vessels. Sites of involvement include the lungs, mediastinum, and the retroperitoneum. It usually presents with chylous pleural effusion or ascites. [ NCIT:C3725 ]

Term info

database cross reference
  • MESH:D018192 (https://orcid.org/0000-0003-1967-3726)
  • ICD10CM:J84.81 (MONDO:equivalentTo)
  • NCIT:C3725 (MONDO:equivalentTo)
  • OMIM:606690 (MONDO:equivalentTo)
  • ICDO:9174/1 (NCIT:C3725)
Subsets

gard_rare, ordo_disease

abbreviation
LAM [ Orphanet:538 MONDO:Lexical OMIM:606690 ]

IAO 0000233

https://github.com/monarch-initiative/mondo/issues/4016, https://github.com/monarch-initiative/mondo/issues/5537, https://github.com/monarch-initiative/mondo/issues/4521

definition

A multifocal neoplasm with perivascular epithelioid cell differentiation affecting almost exclusively females of child-bearing age. It is characterized by the presence of smooth muscle and epithelioid cells and by the proliferation of lymphatic vessels. Sites of involvement include the lungs, mediastinum, and the retroperitoneum. It usually presents with chylous pleural effusion or ascites.

exactMatch

http://identifiers.org/mesh/D018192, https://omim.org/entry/606690, http://purl.bioontology.org/ontology/ICD10CM/J84.81, http://purl.obolibrary.org/obo/NCIT_C3725

has exact synonym

lymphangiomyomatosis, lymphangioleiomyomatosis, lymphangioleiomyomatosis, somatic

has narrow synonym

pulmonary lymphangioleiomyomatosis, lung lymphangioleiomyomatosis

has related synonym

LAM, lymphangio-myomatosis

id

MONDO:0011705

seeAlso

https://rarediseases.info.nih.gov/diseases/3319/lymphangioleiomyomatosis