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Pancreatic neuroendocrine tumor G1

MedGen UID:
459467
•Concept ID:
C2987241
•
Neoplastic Process
Synonyms: grade 1 neuroendocrine neoplasm of pancreas; pancreas carcinoid tumor; pancreas carcinoid tumour; pancreas NET G1; pancreas neuroendocrine neoplasm G1; pancreas neuroendocrine tumor, well differentiated, low grade; Pancreatic NET G1; pancreatic NET G1; Pancreatic Neuroendocrine Tumor G1; pancreatic neuroendocrine tumor G1; Serotonin-Producing Tumors With and Without Carcinoid Syndrome
 
Monarch Initiative: MONDO:0021535

Definition

A low grade well differentiated neoplasm with neuroendocrine differentiation that arises from the pancreas. The mitotic count is less than 2 per 10 HPF and/or the Ki67 index is equal or less than 2%. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPancreatic neuroendocrine tumor G1

Professional guidelines

PubMed

The evolution of PRRT for the treatment of neuroendocrine tumors; What comes next?
Harris PE, Zhernosekov K
Front Endocrinol (Lausanne) 2022;13:941832. Epub 2022 Oct 31 doi: 10.3389/fendo.2022.941832. PMID: 36387893Free PMC Article
Gastro-entero-pancreatic neuroendocrine neoplasia: The rules for non-operative management.
Panzuto F, Massironi S, Partelli S, Campana D, Rinzivillo M, Invernizzi P, Andreasi V, Lamberti G, Falconi M
Surg Oncol 2020 Dec;35:141-148. Epub 2020 Aug 20 doi: 10.1016/j.suronc.2020.08.015. PMID: 32877883
Pancreatic neuroendocrine tumors: Nosography, management and treatment.
Orditura M, Petrillo A, Ventriglia J, Diana A, Laterza MM, Fabozzi A, Savastano B, Franzese E, Conzo G, Santini L, Ciardiello F, De Vita F
Int J Surg 2016 Apr;28 Suppl 1:S156-62. Epub 2015 Dec 18 doi: 10.1016/j.ijsu.2015.12.052. PMID: 26708853

Recent clinical studies

Etiology

Double Ki-67 and synaptophysin labeling in pancreatic neuroendocrine tumor biopsies.
Ahn B, Jung JK, Jung H, Ryu YM, Kim YW, Song TJ, Park DH, Hwang DW, Cho H, Kim SY, Hong SM
Pancreatology 2022 Apr;22(3):427-434. Epub 2022 Mar 8 doi: 10.1016/j.pan.2022.03.005. PMID: 35292233
Single-cell RNA sequencing reveals spatiotemporal heterogeneity and malignant progression in pancreatic neuroendocrine tumor.
Zhou Y, Liu S, Liu C, Yang J, Lin Q, Zheng S, Chen C, Zhou Q, Chen R
Int J Biol Sci 2021;17(14):3760-3775. Epub 2021 Aug 28 doi: 10.7150/ijbs.61717. PMID: 34671197Free PMC Article
Epidemiologic Trends of and Factors Associated With Overall Survival for Patients With Gastroenteropancreatic Neuroendocrine Tumors in the United States.
Xu Z, Wang L, Dai S, Chen M, Li F, Sun J, Luo F
JAMA Netw Open 2021 Sep 1;4(9):e2124750. doi: 10.1001/jamanetworkopen.2021.24750. PMID: 34554237Free PMC Article
Pancreatic neuroendocrine tumors.
Perri G, Prakash LR, Katz MHG
Curr Opin Gastroenterol 2019 Sep;35(5):468-477. doi: 10.1097/MOG.0000000000000571. PMID: 31306159
Detection of NRG1 Gene Fusions in Solid Tumors.
Jonna S, Feldman RA, Swensen J, Gatalica Z, Korn WM, Borghaei H, Ma PC, Nieva JJ, Spira AI, Vanderwalde AM, Wozniak AJ, Kim ES, Liu SV
Clin Cancer Res 2019 Aug 15;25(16):4966-4972. Epub 2019 Apr 15 doi: 10.1158/1078-0432.CCR-19-0160. PMID: 30988082Free PMC Article

Diagnosis

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for gastroduodenal neuroendocrine tumours (NETs) G1-G3.
Panzuto F, Ramage J, Pritchard DM, van Velthuysen MF, Schrader J, Begum N, Sundin A, Falconi M, O'Toole D
J Neuroendocrinol 2023 Aug;35(8):e13306. Epub 2023 Jul 4 doi: 10.1111/jne.13306. PMID: 37401795
Overview of the 2022 WHO Classification of Neuroendocrine Neoplasms.
Rindi G, Mete O, Uccella S, Basturk O, La Rosa S, Brosens LAA, Ezzat S, de Herder WW, Klimstra DS, Papotti M, Asa SL
Endocr Pathol 2022 Mar;33(1):115-154. Epub 2022 Mar 16 doi: 10.1007/s12022-022-09708-2. PMID: 35294740
Epidemiologic Trends of and Factors Associated With Overall Survival for Patients With Gastroenteropancreatic Neuroendocrine Tumors in the United States.
Xu Z, Wang L, Dai S, Chen M, Li F, Sun J, Luo F
JAMA Netw Open 2021 Sep 1;4(9):e2124750. doi: 10.1001/jamanetworkopen.2021.24750. PMID: 34554237Free PMC Article
Consensus on molecular imaging and theranostics in neuroendocrine neoplasms.
Ambrosini V, Kunikowska J, Baudin E, Bodei L, Bouvier C, Capdevila J, Cremonesi M, de Herder WW, Dromain C, Falconi M, Fani M, Fanti S, Hicks RJ, Kabasakal L, Kaltsas G, Lewington V, Minozzi S, Cinquini M, Öberg K, Oyen WJG, O'Toole D, Pavel M, Ruszniewski P, Scarpa A, Strosberg J, Sundin A, Taïeb D, Virgolini I, Wild D, Herrmann K, Yao J
Eur J Cancer 2021 Mar;146:56-73. Epub 2021 Feb 12 doi: 10.1016/j.ejca.2021.01.008. PMID: 33588146Free PMC Article
Pancreatic neuroendocrine tumors.
Perri G, Prakash LR, Katz MHG
Curr Opin Gastroenterol 2019 Sep;35(5):468-477. doi: 10.1097/MOG.0000000000000571. PMID: 31306159

Therapy

The evolution of PRRT for the treatment of neuroendocrine tumors; What comes next?
Harris PE, Zhernosekov K
Front Endocrinol (Lausanne) 2022;13:941832. Epub 2022 Oct 31 doi: 10.3389/fendo.2022.941832. PMID: 36387893Free PMC Article
Consensus on molecular imaging and theranostics in neuroendocrine neoplasms.
Ambrosini V, Kunikowska J, Baudin E, Bodei L, Bouvier C, Capdevila J, Cremonesi M, de Herder WW, Dromain C, Falconi M, Fani M, Fanti S, Hicks RJ, Kabasakal L, Kaltsas G, Lewington V, Minozzi S, Cinquini M, Öberg K, Oyen WJG, O'Toole D, Pavel M, Ruszniewski P, Scarpa A, Strosberg J, Sundin A, Taïeb D, Virgolini I, Wild D, Herrmann K, Yao J
Eur J Cancer 2021 Mar;146:56-73. Epub 2021 Feb 12 doi: 10.1016/j.ejca.2021.01.008. PMID: 33588146Free PMC Article
Systematic Review and Metaanalysis of Lymph Node Metastases of Resected Pancreatic Neuroendocrine Tumors.
Tanaka M, Heckler M, Mihaljevic AL, Probst P, Klaiber U, Heger U, Schimmack S, Büchler MW, Hackert T
Ann Surg Oncol 2021 Mar;28(3):1614-1624. Epub 2020 Jul 27 doi: 10.1245/s10434-020-08850-7. PMID: 32720049
Management of Well-Differentiated High-Grade (G3) Neuroendocrine Tumors.
Sonbol MB, Halfdanarson TR
Curr Treat Options Oncol 2019 Aug 19;20(9):74. doi: 10.1007/s11864-019-0670-1. PMID: 31428952
Biotherapies for GEP-NETs.
Öberg K
Best Pract Res Clin Gastroenterol 2012 Dec;26(6):833-41. doi: 10.1016/j.bpg.2013.01.001. PMID: 23582922

Prognosis

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for gastroduodenal neuroendocrine tumours (NETs) G1-G3.
Panzuto F, Ramage J, Pritchard DM, van Velthuysen MF, Schrader J, Begum N, Sundin A, Falconi M, O'Toole D
J Neuroendocrinol 2023 Aug;35(8):e13306. Epub 2023 Jul 4 doi: 10.1111/jne.13306. PMID: 37401795
Single-cell RNA sequencing reveals spatiotemporal heterogeneity and malignant progression in pancreatic neuroendocrine tumor.
Zhou Y, Liu S, Liu C, Yang J, Lin Q, Zheng S, Chen C, Zhou Q, Chen R
Int J Biol Sci 2021;17(14):3760-3775. Epub 2021 Aug 28 doi: 10.7150/ijbs.61717. PMID: 34671197Free PMC Article
Epidemiologic Trends of and Factors Associated With Overall Survival for Patients With Gastroenteropancreatic Neuroendocrine Tumors in the United States.
Xu Z, Wang L, Dai S, Chen M, Li F, Sun J, Luo F
JAMA Netw Open 2021 Sep 1;4(9):e2124750. doi: 10.1001/jamanetworkopen.2021.24750. PMID: 34554237Free PMC Article
Pancreatic neuroendocrine tumors.
Perri G, Prakash LR, Katz MHG
Curr Opin Gastroenterol 2019 Sep;35(5):468-477. doi: 10.1097/MOG.0000000000000571. PMID: 31306159
Pancreatic neuroendocrine tumors: Nosography, management and treatment.
Orditura M, Petrillo A, Ventriglia J, Diana A, Laterza MM, Fabozzi A, Savastano B, Franzese E, Conzo G, Santini L, Ciardiello F, De Vita F
Int J Surg 2016 Apr;28 Suppl 1:S156-62. Epub 2015 Dec 18 doi: 10.1016/j.ijsu.2015.12.052. PMID: 26708853

Clinical prediction guides

Impact of tumor grade on pancreatic neuroendocrine tumors.
Shyr BS, Shyr BU, Chen SC, Shyr YM, Wang SE
Asian J Surg 2022 Dec;45(12):2659-2663. Epub 2022 Mar 1 doi: 10.1016/j.asjsur.2022.01.094. PMID: 35246343
Epidemiologic Trends of and Factors Associated With Overall Survival for Patients With Gastroenteropancreatic Neuroendocrine Tumors in the United States.
Xu Z, Wang L, Dai S, Chen M, Li F, Sun J, Luo F
JAMA Netw Open 2021 Sep 1;4(9):e2124750. doi: 10.1001/jamanetworkopen.2021.24750. PMID: 34554237Free PMC Article
Pancreatic neuroendocrine tumors.
Perri G, Prakash LR, Katz MHG
Curr Opin Gastroenterol 2019 Sep;35(5):468-477. doi: 10.1097/MOG.0000000000000571. PMID: 31306159
Pancreatic Neuroendocrine Neoplasms: Basic Biology, Current Treatment Strategies and Prospects for the Future.
Ohmoto A, Rokutan H, Yachida S
Int J Mol Sci 2017 Jan 13;18(1) doi: 10.3390/ijms18010143. PMID: 28098761Free PMC Article
Pathology: Classification and Immunoprofile.
Blank A, Schmitt A, Perren A
Front Horm Res 2015;44:104-14. Epub 2015 Aug 14 doi: 10.1159/000382135. PMID: 26303707

Recent systematic reviews

Systemic Therapy for Tumor Control in Metastatic Well-Differentiated Gastroenteropancreatic Neuroendocrine Tumors: ASCO Guideline.
Del Rivero J, Perez K, Kennedy EB, Mittra ES, Vijayvergia N, Arshad J, Basu S, Chauhan A, Dasari AN, Bellizzi AM, Gangi A, Grady E, Howe JR, Ivanidze J, Lewis M, Mailman J, Raj N, Soares HP, Soulen MC, White SB, Chan JA, Kunz PL, Singh S, Halfdanarson TR, Strosberg JR, Bergsland EK
J Clin Oncol 2023 Nov 10;41(32):5049-5067. Epub 2023 Sep 29 doi: 10.1200/JCO.23.01529. PMID: 37774329
Predictors of disease recurrence after curative surgery for nonfunctioning pancreatic neuroendocrine neoplasms (NF-PanNENs): a systematic review and meta-analysis.
Andreasi V, Ricci C, Partelli S, Guarneri G, Ingaldi C, Muffatti F, Crippa S, Casadei R, Falconi M
J Endocrinol Invest 2022 Apr;45(4):705-718. Epub 2021 Nov 13 doi: 10.1007/s40618-021-01705-2. PMID: 34773595
Systematic Review and Metaanalysis of Lymph Node Metastases of Resected Pancreatic Neuroendocrine Tumors.
Tanaka M, Heckler M, Mihaljevic AL, Probst P, Klaiber U, Heger U, Schimmack S, Büchler MW, Hackert T
Ann Surg Oncol 2021 Mar;28(3):1614-1624. Epub 2020 Jul 27 doi: 10.1245/s10434-020-08850-7. PMID: 32720049
Cystic pancreatic neuroendocrine tumors: A distinctive subgroup with indolent biological behavior? A systematic review and meta-analysis.
Zhu JK, Wu D, Xu JW, Huang X, Jiang YY, Edil BH, Li M, Hu SY, Zhan HX
Pancreatology 2019 Jul;19(5):738-750. Epub 2019 May 27 doi: 10.1016/j.pan.2019.05.462. PMID: 31160191
Surgical treatment of neuroendocrine tumors in the second portion of the duodenum: a single center experience and systematic review of the literature.
Iwasaki T, Nara S, Kishi Y, Esaki M, Shimada K, Hiraoka N
Langenbecks Arch Surg 2017 Sep;402(6):925-933. Epub 2016 Dec 3 doi: 10.1007/s00423-016-1537-6. PMID: 27915372

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