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Functioning Pancreatic Neuroendocrine Tumor G2

MedGen UID:
310777
•Concept ID:
C1708106
•
Neoplastic Process
Synonyms: Functional Pancreatic Neuroendocrine Tumor G2; Functioning Well Differentiated Pancreatic Endocrine Carcinoma

Definition

An intermediate grade well differentiated neoplasm with neuroendocrine differentiation that arises from the pancreas. It is characterized by the presence of a clinical syndrome that results from hormone hypersecretion. [from NCI]

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
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
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

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
Machine learning-based model for prediction and feature analysis of recurrence in pancreatic neuroendocrine tumors G1/G2.
Murakami M, Fujimori N, Nakata K, Nakamura M, Hashimoto S, Kurahara H, Nishihara K, Abe T, Hashigo S, Kugiyama N, Ozawa E, Okamoto K, Ishida Y, Okano K, Takaki R, Shimamatsu Y, Ito T, Miki M, Oza N, Yamaguchi D, Yamamoto H, Takedomi H, Kawabe K, Akashi T, Miyahara K, Ohuchida J, Ogura Y, Nakashima Y, Ueki T, Ishigami K, Umakoshi H, Ueda K, Oono T, Ogawa Y
J Gastroenterol 2023 Jun;58(6):586-597. Epub 2023 Apr 26 doi: 10.1007/s00535-023-01987-8. PMID: 37099152
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
Sporadic non-functioning pancreatic neuroendocrine tumours: multicentre analysis.
Ricci C, Partelli S, Landoni L, Rinzivillo M, Ingaldi C, Andreasi V, Nessi C, Muffatti F, Fontana M, Tamburrino D, Deiro G, Alberici L, Campana D, Panzuto F, Bassi C, Falconi M, Casadei R
Br J Surg 2021 Jul 23;108(7):811-816. doi: 10.1093/bjs/znaa141. PMID: 33724300
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

Diagnosis

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
Machine learning-based model for prediction and feature analysis of recurrence in pancreatic neuroendocrine tumors G1/G2.
Murakami M, Fujimori N, Nakata K, Nakamura M, Hashimoto S, Kurahara H, Nishihara K, Abe T, Hashigo S, Kugiyama N, Ozawa E, Okamoto K, Ishida Y, Okano K, Takaki R, Shimamatsu Y, Ito T, Miki M, Oza N, Yamaguchi D, Yamamoto H, Takedomi H, Kawabe K, Akashi T, Miyahara K, Ohuchida J, Ogura Y, Nakashima Y, Ueki T, Ishigami K, Umakoshi H, Ueda K, Oono T, Ogawa Y
J Gastroenterol 2023 Jun;58(6):586-597. Epub 2023 Apr 26 doi: 10.1007/s00535-023-01987-8. PMID: 37099152
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
Sporadic non-functioning pancreatic neuroendocrine tumours: multicentre analysis.
Ricci C, Partelli S, Landoni L, Rinzivillo M, Ingaldi C, Andreasi V, Nessi C, Muffatti F, Fontana M, Tamburrino D, Deiro G, Alberici L, Campana D, Panzuto F, Bassi C, Falconi M, Casadei R
Br J Surg 2021 Jul 23;108(7):811-816. doi: 10.1093/bjs/znaa141. PMID: 33724300
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

Therapy

Combined Lanreotide Autogel and Temozolomide Treatment of Progressive Pancreatic and Intestinal Neuroendocrine Tumors: The Phase II SONNET Study.
Pavel M, Lahner H, Hörsch D, Rinke A, Denecke T, Koch A, Regnault B, Helbig D, Hoffmanns P, Raderer M
Oncologist 2024 May 3;29(5):e643-e654. doi: 10.1093/oncolo/oyad325. PMID: 38206830Free PMC Article
Machine learning-based model for prediction and feature analysis of recurrence in pancreatic neuroendocrine tumors G1/G2.
Murakami M, Fujimori N, Nakata K, Nakamura M, Hashimoto S, Kurahara H, Nishihara K, Abe T, Hashigo S, Kugiyama N, Ozawa E, Okamoto K, Ishida Y, Okano K, Takaki R, Shimamatsu Y, Ito T, Miki M, Oza N, Yamaguchi D, Yamamoto H, Takedomi H, Kawabe K, Akashi T, Miyahara K, Ohuchida J, Ogura Y, Nakashima Y, Ueki T, Ishigami K, Umakoshi H, Ueda K, Oono T, Ogawa Y
J Gastroenterol 2023 Jun;58(6):586-597. Epub 2023 Apr 26 doi: 10.1007/s00535-023-01987-8. PMID: 37099152
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
Heterogeneity of grade 3 gastroenteropancreatic neuroendocrine carcinomas: New insights and treatment implications.
Fazio N, Milione M
Cancer Treat Rev 2016 Nov;50:61-67. Epub 2016 Aug 28 doi: 10.1016/j.ctrv.2016.08.006. PMID: 27636009
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

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
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
Chemotherapy in NEN: still has a role?
Espinosa-Olarte P, La Salvia A, Riesco-Martinez MC, Anton-Pascual B, Garcia-Carbonero R
Rev Endocr Metab Disord 2021 Sep;22(3):595-614. Epub 2021 Apr 11 doi: 10.1007/s11154-021-09638-0. PMID: 33843007Free PMC Article
Sporadic non-functioning pancreatic neuroendocrine tumours: multicentre analysis.
Ricci C, Partelli S, Landoni L, Rinzivillo M, Ingaldi C, Andreasi V, Nessi C, Muffatti F, Fontana M, Tamburrino D, Deiro G, Alberici L, Campana D, Panzuto F, Bassi C, Falconi M, Casadei R
Br J Surg 2021 Jul 23;108(7):811-816. doi: 10.1093/bjs/znaa141. PMID: 33724300
Genetics and Epigenetics of Gastroenteropancreatic Neuroendocrine Neoplasms.
Mafficini A, Scarpa A
Endocr Rev 2019 Apr 1;40(2):506-536. doi: 10.1210/er.2018-00160. PMID: 30657883Free PMC Article

Clinical prediction guides

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
Machine learning-based model for prediction and feature analysis of recurrence in pancreatic neuroendocrine tumors G1/G2.
Murakami M, Fujimori N, Nakata K, Nakamura M, Hashimoto S, Kurahara H, Nishihara K, Abe T, Hashigo S, Kugiyama N, Ozawa E, Okamoto K, Ishida Y, Okano K, Takaki R, Shimamatsu Y, Ito T, Miki M, Oza N, Yamaguchi D, Yamamoto H, Takedomi H, Kawabe K, Akashi T, Miyahara K, Ohuchida J, Ogura Y, Nakashima Y, Ueki T, Ishigami K, Umakoshi H, Ueda K, Oono T, Ogawa Y
J Gastroenterol 2023 Jun;58(6):586-597. Epub 2023 Apr 26 doi: 10.1007/s00535-023-01987-8. PMID: 37099152
Management and outcomes of patients for non-functioning pancreatic neuroendocrine tumours: a multi-institutional analysis.
Du B, Wang X, Zhang W, Tan Q, Wei Y, Shao Z
ANZ J Surg 2022 Apr;92(4):787-793. Epub 2021 Nov 1 doi: 10.1111/ans.17326. PMID: 34723424
Sporadic non-functioning pancreatic neuroendocrine tumours: multicentre analysis.
Ricci C, Partelli S, Landoni L, Rinzivillo M, Ingaldi C, Andreasi V, Nessi C, Muffatti F, Fontana M, Tamburrino D, Deiro G, Alberici L, Campana D, Panzuto F, Bassi C, Falconi M, Casadei R
Br J Surg 2021 Jul 23;108(7):811-816. doi: 10.1093/bjs/znaa141. PMID: 33724300
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

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
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

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