KEGG   DISEASE: 唾液腺癌
エントリ  
H01508                                                             
名称    
唾液腺癌
  上位グループ
頭頸部癌 [DS:H02420]
固形腫瘍 [DS:H02421]
概要    
Salivary gland carcinomas are rare tumours representing about 0.5% of all malignancies and less than 5 % of all head and neck cancers. These are a heterogeneous group of tumors that include 24 histologically distinct tumor types. Mucoepidermoid carcinoma (MEC) is the most common malignant salivary gland tumor in adults and children. The most common genetic alteration in MECs is a unique translocation t(11;19)(q21;p13) producing the MECT1-MAML2 fusion protein. This fusion transcript can activate transcription of targets in the Notch pathway. Epidermal growth factor receptor (EGFR) and human epidermal growth factor receptor 2 (HER2) overexpression is present in a minority of MECs and is associated with higher rate of metastasis and worse overall survival. Recent studies have evaluated the epidermal growth factor receptor family including EGFR and HER2 as potential therapeutic targets.
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
    口唇, 口腔, 咽頭の悪性腫瘍
     2B68  顎下腺または舌下腺の悪性腫瘍
      H01508  唾液腺癌
病因遺伝子 
CRTC1-MAML2 (translocation) [HSA:23373 84441] [KO:K15309 K06061]
MYB-NF1B (translocation) [HSA:4602] [KO:K09420]
ETV6-NTRK3 (translocation) [HSA:4916] [KO:K05101]
EGFR (overexpression) [HSA:1956] [KO:K04361]
HER2 (overexpression) [HSA:2064] [KO:K05083]
CDKN2A (deletion) [HSA:1029] [KO:K06621]
治療薬   
トラスツズマブ [DR:D03257] (HER2陽性)
リンク   
ICD-11: 2B68
MeSH: D012468
文献    
  著者
Ettl T, Schwarz-Furlan S, Gosau M, Reichert TE
  タイトル
Salivary gland carcinomas.
  雑誌
Oral Maxillofac Surg 16:267-83 (2012)
DOI:10.1007/s10006-012-0350-9
文献    
  著者
Yin LX, Ha PK
  タイトル
Genetic alterations in salivary gland cancers.
  雑誌
Cancer 122:1822-31 (2016)
DOI:10.1002/cncr.29890
文献    
  著者
Stenman G
  タイトル
Fusion oncogenes in salivary gland tumors: molecular and clinical consequences.
  雑誌
Head Neck Pathol 7 Suppl 1:S12-9 (2013)
DOI:10.1007/s12105-013-0462-z
文献    
  著者
Goyal G, Mehdi SA, Ganti AK
  タイトル
Salivary Gland Cancers: Biology and Systemic Therapy.
  雑誌
Oncology (Williston Park) 29:773-80 (2015)
文献    
  著者
Adelstein DJ, Koyfman SA, El-Naggar AK, Hanna EY
  タイトル
Biology and management of salivary gland cancers.
  雑誌
Semin Radiat Oncol 22:245-53 (2012)
DOI:10.1016/j.semradonc.2012.03.009
文献    
  著者
Guzzo M, Locati LD, Prott FJ, Gatta G, McGurk M, Licitra L
  タイトル
Major and minor salivary gland tumors.
  雑誌
Crit Rev Oncol Hematol 74:134-48 (2010)
DOI:10.1016/j.critrevonc.2009.10.004
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