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Neuroendocrine pancreatic tumor

MedGen UID:
277875
Concept ID:
C1337011
Neoplastic Process
Synonyms: Islet Cell Tumor; islet cell tumor; islet cell tumors - pancreas; islet cell tumors of the pancreas; islet cell tumour; islet cell tumours - pancreas; islet cell tumours of the pancreas; neuroendocrine tumor of pancreas; Neuroendocrine tumor of pancreas; neuroendocrine tumour of pancreas; pancreatic endocrine tumor; pancreatic endocrine tumour; Pancreatic NET; pancreatic NET; Pancreatic Neuroendocrine Tumor; Pancreatic neuroendocrine tumor; pancreatic neuroendocrine tumor; PANET; PNET; Well Differentiated Pancreatic Endocrine Neoplasm; well differentiated pancreatic endocrine neoplasm; Well Differentiated Pancreatic Endocrine Tumor; well differentiated pancreatic endocrine tumor; well differentiated pancreatic endocrine tumour; Well-differentiated NEN of pancreas; well-differentiated NEN of pancreas; Well-differentiated neuroendocrine neoplasm of pancreas; well-differentiated neuroendocrine neoplasm of pancreas; well-differentiated pancreatic NEN; Well-differentiated pancreatic NEN; Well-differentiated pancreatic neuroendocrine neoplasm; well-differentiated pancreatic neuroendocrine neoplasm
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019954
Orphanet: ORPHA97253

Definition

Pancreatic endocrine tumor, also known as pancreatic neuroendocrine tumor (PNET), describes a group of endocrine tumors originating in the pancreas that are usually indolent and benign, but may have the potential to be malignant. They can be functional, exhibiting a hormonal hypersecretion syndrome, but can be non-functional presenting with non-specific symptoms and include insulinoma, glucagonoma, VIPoma, somatostatinoma (SSoma), PPoma and Zollinger-Ellison syndrome (ZES, or gastrinoma) and other ectopic hormone producing tumors (such as GRFoma) (see these terms). [from ORDO]

Professional guidelines

PubMed

Giannis D, Moris D, Karachaliou GS, Tsilimigras DI, Karaolanis G, Papalampros A, Felekouras E
J BUON 2020 May-Jun;25(3):1302-1314. PMID: 32862570
Ma ZY, Gong YF, Zhuang HK, Zhou ZX, Huang SZ, Zou YP, Huang BW, Sun ZH, Zhang CZ, Tang YQ, Hou BH
World J Gastroenterol 2020 May 21;26(19):2305-2322. doi: 10.3748/wjg.v26.i19.2305. PMID: 32476795Free PMC Article
Okabayashi T, Shima Y, Sumiyoshi T, Kozuki A, Ito S, Ogawa Y, Kobayashi M, Hanazaki K
World J Gastroenterol 2013 Feb 14;19(6):829-37. doi: 10.3748/wjg.v19.i6.829. PMID: 23430217Free PMC Article

Recent clinical studies

Etiology

Kamath GR, Kim MK, Taioli E
Pancreas 2019 Feb;48(2):161-168. doi: 10.1097/MPA.0000000000001232. PMID: 30589832Free PMC Article
Radu EC, Saizu AI, Grigorescu RR, Croitoru AE, Gheorghe C
J Med Life 2018 Jan-Mar;11(1):57-61. PMID: 29696066Free PMC Article
Rickes S, Unkrodt K, Ocran K, Neye H, Wermke W
Pancreas 2003 Jan;26(1):76-81. doi: 10.1097/00006676-200301000-00013. PMID: 12499921
de Baère T, Roche A, Amenabar JM, Lagrange C, Ducreux M, Rougier P, Elias D, Lasser P, Patriarche C
Hepatology 1996 Jun;23(6):1436-40. doi: 10.1002/hep.510230620. PMID: 8675161
Zilembo N, Buzzoni R, Bajetta E, Di Bartolomeo M, de Braud F, Castellani R, Maffioli L, Celio L, Villa E, Lorusso V
Acta Oncol 1993;32(2):245-50. doi: 10.3109/02841869309083920. PMID: 8391833

Diagnosis

Kamath GR, Kim MK, Taioli E
Pancreas 2019 Feb;48(2):161-168. doi: 10.1097/MPA.0000000000001232. PMID: 30589832Free PMC Article
Radu EC, Saizu AI, Grigorescu RR, Croitoru AE, Gheorghe C
J Med Life 2018 Jan-Mar;11(1):57-61. PMID: 29696066Free PMC Article
Jimborean G, Ianoşi ES, Comes AD, Gurzu S, Niţu MF, Lupuţ GD, Mezei T, Borz OC
Rom J Morphol Embryol 2015;56(2):619-22. PMID: 26193240
Okabayashi T, Shima Y, Sumiyoshi T, Kozuki A, Ito S, Ogawa Y, Kobayashi M, Hanazaki K
World J Gastroenterol 2013 Feb 14;19(6):829-37. doi: 10.3748/wjg.v19.i6.829. PMID: 23430217Free PMC Article
Stamatakos M, Safioleas C, Tsaknaki S, Safioleas P, Iannescu R, Safioleas M
Chirurgia (Bucur) 2009 Nov-Dec;104(6):669-73. PMID: 20187464

Therapy

Hu MG, Xiao YH, Song DD, Zhao GD, Liu YZ, Wang Z, Li HY, Liu R
World J Surg Oncol 2017 Nov 9;15(1):199. doi: 10.1186/s12957-017-1265-6. PMID: 29121944Free PMC Article
Dubey N, Varshney R, Shukla J, Ganeshpurkar A, Hazari PP, Bandopadhaya GP, Mishra AK, Trivedi P
Drug Deliv 2012 Apr;19(3):132-42. doi: 10.3109/10717544.2012.657718. PMID: 22428685
Stoeltzing O, Loss M, Huber E, Gross V, Eilles C, Mueller-Brand J, Schlitt HJ
Langenbecks Arch Surg 2010 Feb;395(2):185-92. Epub 2009 Jun 9 doi: 10.1007/s00423-009-0520-x. PMID: 19506898
Kaemmerer D, Prasad V, Daffner W, Hörsch D, Klöppel G, Hommann M, Baum RP
World J Gastroenterol 2009 Dec 14;15(46):5867-70. doi: 10.3748/wjg.15.5867. PMID: 19998512Free PMC Article
Zilembo N, Buzzoni R, Bajetta E, Di Bartolomeo M, de Braud F, Castellani R, Maffioli L, Celio L, Villa E, Lorusso V
Acta Oncol 1993;32(2):245-50. doi: 10.3109/02841869309083920. PMID: 8391833

Prognosis

Sabol M, Donát R, Macháleková K, Šintál D, Durdík S
Neuro Endocrinol Lett 2020 Sep;41(2):60-68. PMID: 33185992
Kamath GR, Kim MK, Taioli E
Pancreas 2019 Feb;48(2):161-168. doi: 10.1097/MPA.0000000000001232. PMID: 30589832Free PMC Article
Radu EC, Saizu AI, Grigorescu RR, Croitoru AE, Gheorghe C
J Med Life 2018 Jan-Mar;11(1):57-61. PMID: 29696066Free PMC Article
Surace A, Ferrarese A, Benvenga R, Marola S, Cumbo J, Rivelli M, Martino V, Solej M, Nano M
Int J Surg 2014;12 Suppl 1:S222-4. Epub 2014 May 24 doi: 10.1016/j.ijsu.2014.05.035. PMID: 24866074
Okabayashi T, Shima Y, Sumiyoshi T, Kozuki A, Ito S, Ogawa Y, Kobayashi M, Hanazaki K
World J Gastroenterol 2013 Feb 14;19(6):829-37. doi: 10.3748/wjg.v19.i6.829. PMID: 23430217Free PMC Article

Clinical prediction guides

Okabayashi T, Shima Y, Sumiyoshi T, Kozuki A, Ito S, Ogawa Y, Kobayashi M, Hanazaki K
World J Gastroenterol 2013 Feb 14;19(6):829-37. doi: 10.3748/wjg.v19.i6.829. PMID: 23430217Free PMC Article
Mergler S, Strauss O, Strowski M, Prada J, Drost A, Langrehr J, Neuhaus P, Wiedenmann B, Ploeckinger U
Neuroendocrinology 2005;82(2):87-102. Epub 2006 Jan 16 doi: 10.1159/000091008. PMID: 16424676

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