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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

Factors Affecting Nonfunctioning Small Pancreatic Neuroendocrine Neoplasms and Proposed New Treatment Strategies.
Hijioka S, Yamashige D, Esaki M, Honda G, Higuchi R, Masui T, Shimizu Y, Ohtsuka M, Kumamoto Y, Katanuma A, Gotohda N, Akita H, Unno M, Endo I, Yokoyama Y, Yamada S, Matsumoto I, Ohtsuka T, Hirano S, Yasuda H, Kawai M, Aoki T, Nakamura M, Hashimoto D, Rikiyama T, Horiguchi A, Fujii T, Mizuno S, Hanada K, Tani M, Hatori T, Ito T, Okuno M, Kagawa S, Tajima H, Ishii T, Sugimoto M, Onoe S, Takami H, Takada R, Miura T, Kurita Y, Kamei K, Mataki Y, Okazaki K, Takeyama Y, Yamaue H, Satoi S; Japan Pancreas Society Clinical Research Promotion Committee Group (Corporate Authorship)
Clin Gastroenterol Hepatol 2024 Jul;22(7):1416-1426.e5. Epub 2024 Apr 13 doi: 10.1016/j.cgh.2024.03.029. PMID: 38615727
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
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

Recent clinical studies

Etiology

An Update on Appendiceal Neuroendocrine Tumors.
Andrini E, Lamberti G, Alberici L, Ricci C, Campana D
Curr Treat Options Oncol 2023 Jul;24(7):742-756. Epub 2023 May 4 doi: 10.1007/s11864-023-01093-0. PMID: 37140773Free PMC Article
PRRT for higher-grade neuroendocrine neoplasms: What is still acceptable?
Kong G, Hicks RJ
Curr Opin Pharmacol 2022 Dec;67:102293. Epub 2022 Oct 1 doi: 10.1016/j.coph.2022.102293. PMID: 36195008
Prognosis of incompletely resected small rectal neuroendocrine tumor using endoscope without additional treatment.
Cha B, Shin J, Ko WJ, Kwon KS, Kim H
BMC Gastroenterol 2022 Jun 9;22(1):293. doi: 10.1186/s12876-022-02365-z. PMID: 35681149Free PMC Article
Management of non-functional pancreatic neuroendocrine tumors by endoscopic ultrasound-guided radiofrequency ablation: Retrospective study in two tertiary centers.
Marx M, Godat S, Caillol F, Poizat F, Ratone JP, Pesenti C, Schoepfer A, Hoibian S, Dahel Y, Giovannini M
Dig Endosc 2022 Sep;34(6):1207-1213. Epub 2022 Jan 19 doi: 10.1111/den.14224. PMID: 34963025
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

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
An Update on Appendiceal Neuroendocrine Tumors.
Andrini E, Lamberti G, Alberici L, Ricci C, Campana D
Curr Treat Options Oncol 2023 Jul;24(7):742-756. Epub 2023 May 4 doi: 10.1007/s11864-023-01093-0. PMID: 37140773Free PMC Article
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
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
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

Therapy

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
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
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
Endoscopic Resection of Ampullary Neuroendocrine Tumor.
Fukasawa H, Tounou S, Nabetani M, Michida T
Intern Med 2017;56(5):499-503. Epub 2017 Mar 1 doi: 10.2169/internalmedicine.56.7520. PMID: 28250294Free PMC Article

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
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
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
Chemotherapy in NETs: When and how.
Angelousi A, Kaltsas G, Koumarianou A, Weickert MO, Grossman A
Rev Endocr Metab Disord 2017 Dec;18(4):485-497. doi: 10.1007/s11154-017-9432-1. PMID: 28890993

Clinical prediction guides

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
Management of non-functional pancreatic neuroendocrine tumors by endoscopic ultrasound-guided radiofrequency ablation: Retrospective study in two tertiary centers.
Marx M, Godat S, Caillol F, Poizat F, Ratone JP, Pesenti C, Schoepfer A, Hoibian S, Dahel Y, Giovannini M
Dig Endosc 2022 Sep;34(6):1207-1213. Epub 2022 Jan 19 doi: 10.1111/den.14224. PMID: 34963025
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
Distinctive features of pancreatic neuroendocrine neoplasms exhibiting an increment in proliferative activity during the course of the disease.
Alexandraki KI, Kaltsatou M, Kyriakopoulos G, Mavroeidi V, Kostopoulou A, Atlan K, Theocharis S, Rindi G, Grossman AB, Grozinsky-Glasberg S, Kaltsas GA
Endocrine 2021 Apr;72(1):279-286. Epub 2020 Nov 11 doi: 10.1007/s12020-020-02540-w. PMID: 33175321
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

Recent systematic reviews

Well-differentiated G1 and G2 pancreatic neuroendocrine tumors: a meta-analysis of published expanded DNA sequencing data.
Andersen KØ, Detlefsen S, Brusgaard K, Christesen HT
Front Endocrinol (Lausanne) 2024;15:1351624. Epub 2024 May 29 doi: 10.3389/fendo.2024.1351624. PMID: 38868744Free PMC Article
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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