KEGG   DISEASE: Pancreatic neuroendocrine tumorHelp
Entry
H00045                      Disease                                

Name
Pancreatic neuroendocrine tumor
Description
Pancreatic neuroendocrine tumors (PNETs), also known as islet cell tumors, are rare neoplasms that arise in the endocrine tissues of the pancreas. They may occur sporadically or in association with a genetic syndrome, such as multiple endocrine neoplasia type 1 (MEN1), Von Hippel-Landau (VHL) syndrome, neurofibromatosis type 1, or tuberous sclerosis. The most frequent genetic alterations in PNET occur in MEN1 (Multiple Endocrine Neoplasia-1 Gene), DAXX/ATRX (Death-Domain Associated Protein/Mental Retardation Syndrome X-Linked Genes) and the mTOR pathway (Mammalian Target of Rapamycin). A germline mutation in the MEN1 tumor suppressor gene causes MEN1, the above-mentioned autosomal dominant hereditary syndrome. Mutations of DAXX and ATRX are common and related to altered telomeres but not to prognosis.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of endocrine organs
   H00045  Pancreatic neuroendocrine tumor
Human diseases in ICD-10 classification [BR:br08403]
 2. Neoplasms (C00-D48)
  C15-C26  Malignant neoplasms of digestive organs
   C25  Malignant neoplasm of pancreas
    H00045  Pancreatic neuroendocrine tumor
Tumor markers [br08442.html]
 Commonly used tumor markers
  H00045
BRITE hierarchy
Gene
MEN1 (mutation) [HSA:4221] [KO:K14970]
DAXX (mutation) [HSA:1616] [KO:K02308]
ATRX (mutation) [HSA:546] [KO:K10779]
Marker
Chromogranin A (CgA) [HSA:1113] [KO:K19990]
Drug
Everolimus [DR:D02714]
Bevacizumab [DR:D06409]
Temsirolimus [DR:D06068]
Sunitinib [DR:D08552]
Streptozotocin [DR:D05932]
Temozolomide [DR:D06067]
Comment
ICD-O-3: 8151/3 Insulinoma, malignant (C25._)
ICD-O-3: 8153/3 Gastrinoma, malignant
ICD-O-3: 8155/3 Vipoma, malignant
ICD-O-3: 8152/3 Glucagonoma, malignant (C25._)
ICD-O-3: 8156/3 Somatostatinoma, malignant
Other DBs
ICD-10: 
MeSH: 
Reference
PMID:27021395 (description, gene, drug)
  Authors
Viudez A, De Jesus-Acosta A, Carvalho FL, Vera R, Martin-Algarra S, Ramirez N
  Title
Pancreatic neuroendocrine tumors: Challenges in an underestimated disease.
  Journal
Crit Rev Oncol Hematol 101:193-206 (2016)
Reference
PMID:25619712 (gene, drug)
  Authors
Capurso G, Archibugi L, Delle Fave G
  Title
Molecular pathogenesis and targeted therapy of sporadic pancreatic neuroendocrine tumors.
  Journal
J Hepatobiliary Pancreat Sci 22:594-601 (2015)
Reference
PMID:25341008 (gene, drug)
  Authors
Halperin DM, Kulke MH, Yao JC
  Title
A tale of two tumors: treating pancreatic and extrapancreatic neuroendocrine tumors.
  Journal
Annu Rev Med 66:1-16 (2015)
Reference
PMID:24828360 (description)
  Authors
Rustagi T, Farrell JJ
  Title
Endoscopic diagnosis and treatment of pancreatic neuroendocrine tumors.
  Journal
J Clin Gastroenterol 48:837-44 (2014)
Reference
PMID:24987821 (gene, ICD-O-3)
  Authors
Karakaxas D, Gazouli M, Liakakos T, Vaiopoulou A, Apessou D, Papaparaskeva K, Patapis P, Dervenis C
  Title
Pancreatic neuroendocrine tumors: current opinions on a rare, but potentially curable neoplasm.
  Journal
Eur J Gastroenterol Hepatol 26:826-35 (2014)
Reference
PMID:23237225 (ICD-O-3)
  Authors
Ro C, Chai W, Yu VE, Yu R
  Title
Pancreatic neuroendocrine tumors: biology, diagnosis,and treatment.
  Journal
Chin J Cancer 32:312-24 (2013)
Reference
PMID:22586144 (description, gene)
  Authors
Capurso G, Festa S, Valente R, Piciucchi M, Panzuto F, Jensen RT, Delle Fave G
  Title
Molecular pathology and genetics of pancreatic endocrine tumours.
  Journal
J Mol Endocrinol 49:R37-50 (2012)
Reference
PMID:25689919 (description)
  Authors
Grozinsky-Glasberg S, Mazeh H, Gross DJ
  Title
Clinical features of pancreatic neuroendocrine tumors.
  Journal
J Hepatobiliary Pancreat Sci 22:578-85 (2015)

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