KEGG   DISEASE: Malignant pleural mesotheliomaHelp
Entry
H00015                      Disease                                

Name
Malignant pleural mesothelioma
Description
Malignant mesothelioma (MM) is a rare but very aggressive tumor that arises from mesothelial cells lining the pleural, peritoneal and pericardial cavities. Malignant pleural mesothelioma (MPM) is the most common type, accounting for about 70% of all MM cases. Past asbestos exposure represents the major risk factor for MPM, as the link between asbestos fibres and MPM has been largely proved by epidemiological and experimental studies. Recently, simian virus 40 (SV40) has been implicated in the aetiology of MPM. The accumulation of numerous clonal chromosomal deletions in most MMs suggests a multistep process of tumorigenesis, characterized by the loss and/or inactivation of multiple tumor suppressor genes (TSGs). Cytogenetic and loss of heterozygosity (LOH) analyses of MMs have demonstrated frequent deletions of specific sites within chromosome arms 1p, 3p, 6q, 9p, 13q, 15q, and 22q. Furthermore, TSGs within two of these regions, i.e., p16/CDKN2A-p14ARF at 9p21 and NF2 at 22q12, are frequently altered in MMs. Mutations of the p53 gene (TP53) are occasionally observed in MMs.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of the lung and pleura
   H00015  Malignant pleural mesothelioma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Malignant neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues
   Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues
    Malignant neoplasms of middle ear, respiratory or intrathoracic organs
     2C26  Malignant neoplasms of the pleura
      H00015  Malignant pleural mesothelioma
BRITE hierarchy
Gene
p53 (inactivation) [HSA:7157] [KO:K04451]
p16/CDKN2A (deletion) [HSA:1029] [KO:K06621]
p15/CDKN2B (deletion) [HSA:1030] [KO:K04685]
NF2 (mutation) [HSA:4771] [KO:K16684]
PDGF-A (overexpression) [HSA:5154] [KO:K04359]
PDGF-B (overexpression) [HSA:5155] [KO:K17386]
IGF-1 (expression) [HSA:3479] [KO:K05459]
IGF-R1 (expression) [HSA:3480] [KO:K05087]
Carcinogen
Asbestos [CPD:C16442]
Pathogen
Simian virus 40 (SV40) [GN:T40086]
Drug
Pemetrexed sodium hydrate [DR:D06503]
Comment
ICD-O: 9050/3, Tumor type: Diffuse malignant mesothelioma
ICD-O: 9050/3, Tumor type: Localized malignant mesothelioma
Other DBs
ICD-11: 2C26
ICD-10: C45
MeSH: D008654
Reference
PMID:16216411 (gene, tumor type, carcinogen)
  Authors
Whitson BA, Kratzke RA.
  Title
Molecular pathways in malignant pleural mesothelioma.
  Journal
Cancer Lett 239:183-9 (2006)
DOI:10.1016/j.canlet.2005.08.010
Reference
PMID:15691231 (gene, carcinogen)
  Authors
Jaurand MC, Fleury-Feith J.
  Title
Pathogenesis of malignant pleural mesothelioma.
  Journal
Respirology 10:2-8 (2005)
DOI:10.1111/j.1440-1843.2005.00694.x
Reference
PMID:15660110 (carcinogen)
  Authors
Luch A.
  Title
Nature and nurture - lessons from chemical carcinogenesis.
  Journal
Nat Rev Cancer 5:113-25 (2005)
DOI:10.1038/nrc1546
Reference
PMID:17375514 (carcinogen)
  Authors
O'Reilly KM, Mclaughlin AM, Beckett WS, Sime PJ.
  Title
Asbestos-related lung disease.
  Journal
Am Fam Physician 75:683-8 (2007)
Reference
PMID:15955137 (marker)
  Authors
Sterman DH, Albelda SM.
  Title
Advances in the diagnosis, evaluation, and management of malignant pleural mesothelioma.
  Journal
Respirology 10:266-83 (2005)
DOI:10.1111/j.1440-1843.2005.00714.x
Reference
PMID:12883236 (marker)
  Authors
Ordonez NG.
  Title
The immunohistochemical diagnosis of mesothelioma: a comparative study of epithelioid mesothelioma and lung adenocarcinoma.
  Journal
Am J Surg Pathol 27:1031-51 (2003)

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