KEGG   DISEASE: 悪性胸膜中皮腫
エントリ  
H00015                                                             
名称    
悪性胸膜中皮腫
概要    
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.
カテゴリ  
がん
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 02 腫瘍
  悪性腫瘍, ただしリンパ, 造血, 中枢神経系または関連組織の原発腫瘍を除く
   悪性腫瘍, 原発性と確定または推定されるもの, 明示された部位のもの, ただしリンパ, 造血, 中枢神経系, 関連組織を除く
    中耳, 呼吸器または胸腔内臓器の悪性腫瘍
     2C26  胸膜の悪性腫瘍
      H00015  悪性胸膜中皮腫
病因遺伝子 
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]
病原体   
Simian virus 40 (SV40) [GN:T40086]
治療薬   
ペメトレキセドナトリウム水和物 [DR:D06503]
ペメトレキセドナトリウムヘミペンタ水和物 [DR:D10596]
ニボルマブ [DR:D10316]
ペムブロリズマブ [DR:D10574]
イピリムマブ [DR:D04603]
シスプラチン [DR:D00275]
リンク   
ICD-11: 2C26
MeSH: D008654
文献    
  著者
Whitson BA, Kratzke RA.
  タイトル
Molecular pathways in malignant pleural mesothelioma.
  雑誌
Cancer Lett 239:183-9 (2006)
DOI:10.1016/j.canlet.2005.08.010
文献    
  著者
Jaurand MC, Fleury-Feith J.
  タイトル
Pathogenesis of malignant pleural mesothelioma.
  雑誌
Respirology 10:2-8 (2005)
DOI:10.1111/j.1440-1843.2005.00694.x
文献    
  著者
Luch A.
  タイトル
Nature and nurture - lessons from chemical carcinogenesis.
  雑誌
Nat Rev Cancer 5:113-25 (2005)
DOI:10.1038/nrc1546
文献    
  著者
O'Reilly KM, Mclaughlin AM, Beckett WS, Sime PJ.
  タイトル
Asbestos-related lung disease.
  雑誌
Am Fam Physician 75:683-8 (2007)
文献    
  著者
Sterman DH, Albelda SM.
  タイトル
Advances in the diagnosis, evaluation, and management of malignant pleural mesothelioma.
  雑誌
Respirology 10:266-83 (2005)
DOI:10.1111/j.1440-1843.2005.00714.x
文献    
  著者
Ordonez NG.
  タイトル
The immunohistochemical diagnosis of mesothelioma: a comparative study of epithelioid mesothelioma and lung adenocarcinoma.
  雑誌
Am J Surg Pathol 27:1031-51 (2003)
DOI:10.1097/00000478-200308000-00001
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