KEGG   DISEASE: Salivary gland cancerHelp
Entry
H01508                      Disease                                

Name
Salivary gland cancer
Description
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.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Head and neck cancers
   H01508  Salivary gland cancer
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Malignant neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues
   Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues
    Malignant neoplasms of lip, oral cavity or pharynx
     2B68  Malignant neoplasms of submandibular or sublingual glands
      H01508  Salivary gland cancer
BRITE hierarchy
Pathway
hsa04330 Notch signaling pathway   
Gene
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]
Comment
ICD-O-3: 8430/3 mucoepidermoid carcinoma (MEC)
ICD-O-3: 8550/3 acinic cell carcinoma
ICD-O-3: 8200/3 adenoid cystic carcinoma
ICD-O-3: 8140/3 adenocarcinoma not otherwise specified (NOS)
ICD-O-3: 8525/3 polynorphous low grade adenocarcinoma
ICD-O-3: 8940/0 pleomorphic adenoma
ICD-O-3: 8500/3 salivary duct carcinoma
ICD-O-3: 8070/3 squamous cell carcinoma
Other DBs
ICD-11: 2B68
ICD-10: C08.9
MeSH: D012468
Reference
PMID:22842859 (description, gene, marker, drug, tumor type)
  Authors
Ettl T, Schwarz-Furlan S, Gosau M, Reichert TE
  Title
Salivary gland carcinomas.
  Journal
Oral Maxillofac Surg 16:267-83 (2012)
DOI:10.1007/s10006-012-0350-9
Reference
PMID:26928905 (description, gene, tumor type)
  Authors
Yin LX, Ha PK
  Title
Genetic alterations in salivary gland cancers.
  Journal
Cancer 122:1822-31 (2016)
DOI:10.1002/cncr.29890
Reference
  Authors
Stenman G
  Title
Fusion oncogenes in salivary gland tumors: molecular and clinical consequences.
  Journal
Head Neck Pathol 7 Suppl 1:S12-9 (2013)
DOI:10.1007/s12105-013-0462-z
Reference
PMID:26470903 (gene, marker, tumor type)
  Authors
Goyal G, Mehdi SA, Ganti AK
  Title
Salivary Gland Cancers: Biology and Systemic Therapy.
  Journal
Oncology (Williston Park) 29:773-80 (2015)
Reference
PMID:22687949 (description, marker, drug, tumor type)
  Authors
Adelstein DJ, Koyfman SA, El-Naggar AK, Hanna EY
  Title
Biology and management of salivary gland cancers.
  Journal
Semin Radiat Oncol 22:245-53 (2012)
DOI:10.1016/j.semradonc.2012.03.009
Reference
PMID:19939701 (marker, drug, tumor type)
  Authors
Guzzo M, Locati LD, Prott FJ, Gatta G, McGurk M, Licitra L
  Title
Major and minor salivary gland tumors.
  Journal
Crit Rev Oncol Hematol 74:134-48 (2010)
DOI:10.1016/j.critrevonc.2009.10.004

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