H00045 | |
H number | H00045 |
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 |
Network | - |
Gene | MEN1 [HSA:4221] [KO:K14970] DAXX [HSA:1616] [KO:K02308] ATRX [HSA:546] [KO:K10779] |
Pathogen | - |
Env factor | - |
Carcinogen | - |
Drug | Lanreotide acetate [DR:D04666] Streptozocin [DR:D05932] Everolimus [DR:D02714] Sunitinib malate [DR:D06402] Lutetium (177Lu) oxodotreotide [DR:D11033] (somatostatin receptor-positive) |
Comment | - |
Other DBs | ICD-11: 2C10.1 ICD-10: C25.4 MeSH: D018273 |
Reference | PMID:27021395 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 PMID:25341008 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 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 |