KEGG   DISEASE: Pancreatic neuroendocrine tumor
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 the digestive system
   H00045  Pancreatic neuroendocrine tumor
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Malignant neoplasms, except primary neoplasms 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 digestive organs
     2C10  Malignant neoplasm of pancreas
      H00045  Pancreatic neuroendocrine tumor
Tumor markers [br08442.html]
 H00045
Related
pathway
hsa05200  Pathways in cancer
Gene
MEN1 [HSA:4221] [KO:K14970]
DAXX [HSA:1616] [KO:K02308]
ATRX [HSA:546] [KO:K10779]
Drug
Lanreotide acetate [DR:D04666]
Streptozocin [DR:D05932]
Everolimus [DR:D02714]
Sunitinib malate [DR:D06402]
Lutetium (177Lu) oxodotreotide [DR:D11033] (somatostatin receptor-positive)
Other DBs
ICD-11: 2C10.1
ICD-10: C25.4
MeSH: D018273
Reference
  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)
DOI:10.1016/j.critrevonc.2016.03.013
Reference
  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)
DOI:10.1146/annurev-med-061813-012908
Reference
PMID:25619712 (MEN1, DAXX_ATRX)
  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)
DOI:10.1002/jhbp.210
LinkDB

» Japanese version

DBGET integrated database retrieval system