KEGG   DISEASE: 血管肉腫
エントリ  
H01666                                                             
名称    
血管肉腫
  上位グループ
固形腫瘍 [DS:H02421]
軟部肉腫 [DS:H02427]
概要    
Angiosarcomas are rare, generally aggressive, malignant mesenchymal tumours of vascular origin and constitute less than 1% of all sarcomas. Their most frequent site of origin is the head and neck, especially the scalp, of elderly men. Angiosarcomas are likely derived from vascular endothelial cells, possibly resulting from aberrant angiogenesis. Studies have shown relatively increased expression of vascular endothelial growth factor (VEGF) and its receptors (VEGFr1-3) in human angiosarcoma. The most common gene mutation in angiosarcomas is KRAS as well as other RAS mutations. Activation of the RAS pathway is often tumorigenic. Although immortalised murine endothelial cells form benign haemangiomas in vivo, the addition of activated HRAS produces rapidly growing, poorly differentiated angiosarcomas.
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
      H01666  血管肉腫
病因遺伝子 
KRAS (mutation) [HSA:3845] [KO:K07827]
HRAS (mutation) [HSA:3265] [KO:K02833]
NRAS (mutation) [HSA:4893] [KO:K07828]
治療薬   
ビンクリスチン硫酸塩 [DR:D02197]
パクリタキセル [DR:D00491]
テセロイキン [DR:D02749]
リンク   
ICD-11: 2B56
ICD-10: C44.9 C22.3
MeSH: D006394
文献    
  著者
Tanioka M, Ikoma A, Morita K, Fujii H, Toda KI, Takahashi K, Tanaka T, Nishigori C, Jin G, Higashi S, Toyokuni S, Miyachi Y
  タイトル
Angiosarcoma of the scalp: absence of vascular endothelial cadherin in primary and metastatic lesions.
  雑誌
Br J Dermatol 144:380-3 (2001)
DOI:10.1046/j.1365-2133.2001.04032.x
文献    
  著者
Letsa I, Benson C, Al-Muderis O, Judson I
  タイトル
Angiosarcoma of the face and scalp: effective systemic treatment in the older patient.
  雑誌
J Geriatr Oncol 5:276-80 (2014)
DOI:10.1016/j.jgo.2014.02.004
文献    
  著者
Patel SH, Hayden RE, Hinni ML, Wong WW, Foote RL, Milani S, Wu Q, Ko SJ, Halyard MY
  タイトル
Angiosarcoma of the scalp and face: the Mayo Clinic experience.
  雑誌
JAMA Otolaryngol Head Neck Surg 141:335-40 (2015)
DOI:10.1001/jamaoto.2014.3584
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
Huntington JT, Jones C, Liebner DA, Chen JL, Pollock RE
  タイトル
Angiosarcoma: A rare malignancy with protean clinical presentations.
  雑誌
J Surg Oncol 111:941-50 (2015)
DOI:10.1002/jso.23918
文献    
  著者
Antonescu C
  タイトル
Malignant vascular tumors--an update.
  雑誌
Mod Pathol 27 Suppl 1:S30-8 (2014)
DOI:10.1038/modpathol.2013.176
文献    
  著者
Sullivan HC, Edgar MA, Cohen C, Kovach CK, HooKim K, Reid MD
  タイトル
The utility of ERG, CD31 and CD34 in the cytological diagnosis of angiosarcoma: an analysis of 25 cases.
  雑誌
J Clin Pathol 68:44-50 (2015)
DOI:10.1136/jclinpath-2014-202629
文献    
  著者
Ohguri T, Imada H, Nomoto S, Yahara K, Hisaoka M, Hashimoto H, Tokura Y, Nakamura K, Shioyama Y, Honda H, Terashima H, Moroi Y, Furue M, Korogi Y
  タイトル
Angiosarcoma of the scalp treated with curative radiotherapy plus recombinant interleukin-2 immunotherapy.
  雑誌
Int J Radiat Oncol Biol Phys 61:1446-53 (2005)
DOI:10.1016/j.ijrobp.2004.08.008
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