KEGG   DISEASE: Salivary gland cancerHelp
H01508                      Disease                                

Salivary gland cancer
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.
Human diseases [BR:br08402]
  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
hsa04330 Notch signaling pathway   
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]
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
PMID:22842859 (description, gene, marker, drug, tumor type)
Ettl T, Schwarz-Furlan S, Gosau M, Reichert TE
Salivary gland carcinomas.
Oral Maxillofac Surg 16:267-83 (2012)
PMID:26928905 (description, gene, tumor type)
Yin LX, Ha PK
Genetic alterations in salivary gland cancers.
Cancer 122:1822-31 (2016)
Stenman G
Fusion oncogenes in salivary gland tumors: molecular and clinical consequences.
Head Neck Pathol 7 Suppl 1:S12-9 (2013)
PMID:26470903 (gene, marker, tumor type)
Goyal G, Mehdi SA, Ganti AK
Salivary Gland Cancers: Biology and Systemic Therapy.
Oncology (Williston Park) 29:773-80 (2015)
PMID:22687949 (description, marker, drug, tumor type)
Adelstein DJ, Koyfman SA, El-Naggar AK, Hanna EY
Biology and management of salivary gland cancers.
Semin Radiat Oncol 22:245-53 (2012)
PMID:19939701 (marker, drug, tumor type)
Guzzo M, Locati LD, Prott FJ, Gatta G, McGurk M, Licitra L
Major and minor salivary gland tumors.
Crit Rev Oncol Hematol 74:134-48 (2010)

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