KEGG   DISEASE: メルケル細胞癌
エントリ  
H01555                                                             
名称    
メルケル細胞癌
概要    
Merkel cell carcinoma (MCC) is a cutaneous neuroendocrine malignancy that exhibits clinically aggressive features and is associated with a poor prognosis. Ultraviolet (UV) radiation may be a cause, and corresponds with the sites of these tumours, often on sun-exposed skin, mostly (94%) in the White population. UV light may exert local immunomodulating action as it decreases amount of epidermal T cells and Langerhans cells, inducing hapten tolerance. Additionally, UV- specific mutations in TP53 and Harvey (Ha)-RAS genes are present in some MCC cell lines. Recently, the polyomavirus group has been linked with MCC. The MCC polyomavirus (MCPyV) has been isolated from MCC tissue and thought to be present in up to 80% of MCC. Tumorigenesis likely is caused by a number of sequential steps from viral integration into host DNA, mutagenic events, and specific immune responses.
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
    皮膚の悪性腫瘍
     2C34  皮膚の神経内分泌癌
      H01555  メルケル細胞癌
病因遺伝子 
TP53 (mutation) [HSA:7157] [KO:K04451]
Ha-RAS (mutation) [HSA:3265] [KO:K02833]
病原体   
Merkel cell polyomavirus (MCPyV) [GN:T40051]
治療薬   
アベルマブ [DR:D10817]
リンク   
ICD-11: 2C34
MeSH: D015266
文献    
  著者
Czapiewski P, Biernat W
  タイトル
Merkel cell carcinoma - recent advances in the biology, diagnostics and treatment.
  雑誌
Int J Biochem Cell Biol 53:536-46 (2014)
DOI:10.1016/j.biocel.2014.04.023
文献    
  著者
Prewett SL, Ajithkumar T
  タイトル
Merkel Cell Carcinoma: Current Management and Controversies.
  雑誌
Clin Oncol (R Coll Radiol) 27:436-44 (2015)
DOI:10.1016/j.clon.2015.04.007
文献    
  著者
Miles BA, Goldenberg D
  タイトル
Merkel cell carcinoma: Do you know your guidelines?
  雑誌
Head Neck 38:647-52 (2016)
DOI:10.1002/hed.24359
文献    
  著者
Schrama D, Ugurel S, Becker JC
  タイトル
Merkel cell carcinoma: recent insights and new treatment options.
  雑誌
Curr Opin Oncol 24:141-9 (2012)
DOI:10.1097/CCO.0b013e32834fc9fe
文献    
  著者
Desch L, Kunstfeld R
  タイトル
Merkel cell carcinoma: chemotherapy and emerging new therapeutic options.
  雑誌
J Skin Cancer 2013:327150 (2013)
DOI:10.1155/2013/327150
文献    
  著者
Oram CW, Bartus CL, Purcell SM
  タイトル
Merkel cell carcinoma: a review.
  雑誌
Cutis 97:290-5 (2016)
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