KEGG   DISEASE: 肝血管肉腫
エントリ  
H01557                                                             
名称    
肝血管肉腫
  上位グループ
固形腫瘍 [DS:H02421]
概要    
Angiosarcoma represents 1 to 2% of soft tissue tumors. It originates from endothelial cells of small blood vessels and may affect a variety of organs, including the retroperitoneum, skeletal muscle, subcutis, liver, heart and breast. Primary hepatic angiosarcomas (HAS) are much rare tumors, with worse prognosis compared with other angiosarcomas. HAS has been found to be caused by occupational exposure to vinyl chloride (VC) monomer since 1970, and the number of VC-associated HAS cases reported up to the end of 1998 was 197 worldwide. Exposure of VC connects with a series of mechanistic events leading to carcinogenic outcome; mutations of K-ras and p53 are also detected.
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
    悪性間葉系腫瘍
     2B56  血管肉腫, 原発巣
      H01557  肝血管肉腫
病因遺伝子 
TP53 (mutation) [HSA:7157] [KO:K04451]
KRAS-2 (mutation) [HSA:3845] [KO:K07827]
リンク   
ICD-11: 2B56.3
ICD-10: C22.3
MeSH: D006394
文献    
  著者
Chaudhary P, Bhadana U, Singh RA, Ahuja A
  タイトル
Primary hepatic angiosarcoma.
  雑誌
Eur J Surg Oncol 41:1137-43 (2015)
DOI:10.1016/j.ejso.2015.04.022
文献    
  著者
Zheng YW, Zhang XW, Zhang JL, Hui ZZ, Du WJ, Li RM, Ren XB
  タイトル
Primary hepatic angiosarcoma and potential treatment options.
  雑誌
J Gastroenterol Hepatol 29:906-11 (2014)
DOI:10.1111/jgh.12506
文献    
  著者
Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ
  タイトル
Angiosarcoma.
  雑誌
Lancet Oncol 11:983-91 (2010)
DOI:10.1016/S1470-2045(10)70023-1
文献    
  著者
Asmane I, Litique V, Heymann S, Marcellin L, Metivier AC, Duclos B, Bergerat JP, Kurtz JE
  タイトル
Adriamycin, cisplatin, ifosfamide and paclitaxel combination as front-line chemotherapy for locally advanced and metastatic angiosarcoma. Analysis of three case reports and review of the literature.
  雑誌
Anticancer Res 28:3041-5 (2008)
文献    
  著者
Wang ZB, Yuan J, Chen W, Wei LX
  タイトル
Transcription factor ERG is a specific and sensitive diagnostic marker for hepatic angiosarcoma.
  雑誌
World J Gastroenterol 20:3672-9 (2014)
DOI:10.3748/wjg.v20.i13.3672
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