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Functional pancreatic neuroendocrine tumor

MedGen UID:
310778
•Concept ID:
C1708107
•
Neoplastic Process
Synonyms: Functional Pancreatic NET; functional pancreatic NET; Functional Pancreatic Neuroendocrine Tumor; functional pancreatic neuroendocrine tumor; functioning neuroendocrine tumor of pancreas; Functioning neuroendocrine tumor of pancreas; functioning neuroendocrine tumour of pancreas; Functioning Pancreatic Endocrine Tumor; functioning pancreatic endocrine tumor; functioning pancreatic endocrine tumour; Functioning Pancreatic NET; functioning pancreatic NET; Functioning pancreatic NET; Functioning Pancreatic Neuroendocrine Tumor; Functioning pancreatic neuroendocrine tumor; functioning pancreatic neuroendocrine tumor; functioning pancreatic neuroendocrine tumour; Functioning PNET; functioning PNET; Functioning Well Differentiated Pancreatic Endocrine Neoplasm; functioning well differentiated pancreatic endocrine neoplasm; Functioning Well Differentiated Pancreatic Endocrine Tumor; functioning well differentiated pancreatic endocrine tumor; functioning well differentiated pancreatic endocrine tumour; Functioning well-differentiated NEN of pancreas; functioning well-differentiated NEN of pancreas; functioning well-differentiated neuroendocrine neoplasm of pancreas; Functioning well-differentiated neuroendocrine neoplasm of pancreas; Functioning well-differentiated pancreatic NEN; functioning well-differentiated pancreatic NEN; Functioning well-differentiated pancreatic neuroendocrine neoplasm; functioning well-differentiated pancreatic neuroendocrine neoplasm; Syndromic Pancreatic NET; syndromic pancreatic NET; Syndromic Pancreatic Neuroendocrine Tumor; syndromic pancreatic neuroendocrine tumor; syndromic pancreatic neuroendocrine tumour
 
Monarch Initiative: MONDO:0023206
Orphanet: ORPHA506060

Definition

A well differentiated, low, intermediate, or high grade 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

Characteristics and treatment options of glucagonomas: a national study from the French Group of Endocrine Tumors and ENDOCAN-RENATEN network.
Perrier M, Brugel M, Gérard L, Goichot B, Lièvre A, Lepage C, Hautefeuille V, Do Cao C, Smith D, Thuillier P, Cros J, Cadiot G, Walter T, de Mestier L
Eur J Endocrinol 2023 Dec 6;189(6):575-583. doi: 10.1093/ejendo/lvad157. PMID: 38039101
Clinical characteristics and management of insulin-producing neuroendocrine carcinomas.
Lowette K, Verslype C, Van Cutsem E
Acta Gastroenterol Belg 2016 Jul-Sep;79(2):321-327. PMID: 27821028

Recent clinical studies

Etiology

Characteristics and treatment options of glucagonomas: a national study from the French Group of Endocrine Tumors and ENDOCAN-RENATEN network.
Perrier M, Brugel M, Gérard L, Goichot B, Lièvre A, Lepage C, Hautefeuille V, Do Cao C, Smith D, Thuillier P, Cros J, Cadiot G, Walter T, de Mestier L
Eur J Endocrinol 2023 Dec 6;189(6):575-583. doi: 10.1093/ejendo/lvad157. PMID: 38039101
A single-center experience on endoscopic ultrasonography-guided ethanol ablation of insulinomas.
Yan Z, Zhu C, Wu X, Zhu H, Yuan T, Luo Y, Feng Y, Wang Q, Zhang S, Xue H, Zheng Z, Yang A
Pancreatology 2023 Jan;23(1):98-104. Epub 2022 Dec 13 doi: 10.1016/j.pan.2022.12.007. PMID: 36577553
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
Tumor-Infiltrating Neutrophils Predict Poor Survival of Non-Functional Pancreatic Neuroendocrine Tumor.
Zhang WH, Wang WQ, Gao HL, Xu SS, Li S, Li TJ, Han X, Xu HX, Li H, Jiang W, Ye LY, Lin X, Wu CT, Yu XJ, Liu L
J Clin Endocrinol Metab 2020 Jul 1;105(7) doi: 10.1210/clinem/dgaa196. PMID: 32285127
Clinical characteristics and management of insulin-producing neuroendocrine carcinomas.
Lowette K, Verslype C, Van Cutsem E
Acta Gastroenterol Belg 2016 Jul-Sep;79(2):321-327. PMID: 27821028

Diagnosis

ABCD1 as a Novel Diagnostic Marker for Solid Pseudopapillary Neoplasm of the Pancreas.
Liu YA, Liu Y, Tu J, Shi Y, Pang J, Huang Q, Wang X, Lin Z, Zhao Y, Wang W, Peng J, Wu W
Am J Surg Pathol 2024 May 1;48(5):511-520. Epub 2024 Apr 3 doi: 10.1097/PAS.0000000000002205. PMID: 38567813Free PMC Article
Characteristics and treatment options of glucagonomas: a national study from the French Group of Endocrine Tumors and ENDOCAN-RENATEN network.
Perrier M, Brugel M, Gérard L, Goichot B, Lièvre A, Lepage C, Hautefeuille V, Do Cao C, Smith D, Thuillier P, Cros J, Cadiot G, Walter T, de Mestier L
Eur J Endocrinol 2023 Dec 6;189(6):575-583. doi: 10.1093/ejendo/lvad157. PMID: 38039101
Differentiation of atypical non-functional pancreatic neuroendocrine tumor and pancreatic ductal adenocarcinoma using CT based radiomics.
He M, Liu Z, Lin Y, Wan J, Li J, Xu K, Wang Y, Jin Z, Tian J, Xue H
Eur J Radiol 2019 Aug;117:102-111. Epub 2019 May 31 doi: 10.1016/j.ejrad.2019.05.024. PMID: 31307634
Clinical characteristics and management of insulin-producing neuroendocrine carcinomas.
Lowette K, Verslype C, Van Cutsem E
Acta Gastroenterol Belg 2016 Jul-Sep;79(2):321-327. PMID: 27821028
Pitfalls in diagnosing a pancreatic neuroendocrine tumor: a case report.
Popa SG, Ungureanu BS, Gheonea IA, Mitrea A, Ardeleanu CM, Ghiluşi MC, Şurlin V, Georgescu EF, Georgescu I, MoŢa M, Marioara OM, Săftoiu A
Rom J Morphol Embryol 2015;56(4):1495-502. PMID: 26743299

Therapy

Study protocol for a multicenter randomized controlled trial to compare radiofrequency ablation with surgical resection for treatment of pancreatic insulinoma.
Crinò SF, Partelli S, Napoleon B, Conti Bellocchi MC, Facciorusso A, Salvia R, Forti E, Cintolo M, Mazzola M, Ferrari G, Carrara S, Repici A, Zerbi A, Lania A, Tacelli M, Arcidiacono PG, Falconi M, Larghi A, Rizzatti G, Alfieri S, Panzuto F, Hindryckx P, Berrevoet F, Lapauw B, Lakhtakia S, Sundaram S, Samanta J, Rastogi A, Landoni L
Dig Liver Dis 2023 Sep;55(9):1187-1193. Epub 2023 Jul 3 doi: 10.1016/j.dld.2023.06.021. PMID: 37407318
A single-center experience on endoscopic ultrasonography-guided ethanol ablation of insulinomas.
Yan Z, Zhu C, Wu X, Zhu H, Yuan T, Luo Y, Feng Y, Wang Q, Zhang S, Xue H, Zheng Z, Yang A
Pancreatology 2023 Jan;23(1):98-104. Epub 2022 Dec 13 doi: 10.1016/j.pan.2022.12.007. PMID: 36577553
A rare case of metastasized non-functional pancreatic neuroendocrine tumor with a good long-term survival.
Mirică A, Bădărău IA, Mirică R, Păun S, Păun DL
J Med Life 2016 Oct-Dec;9(4):369-372. PMID: 27928440Free PMC Article
Clinical characteristics and management of insulin-producing neuroendocrine carcinomas.
Lowette K, Verslype C, Van Cutsem E
Acta Gastroenterol Belg 2016 Jul-Sep;79(2):321-327. PMID: 27821028

Prognosis

Characteristics and treatment options of glucagonomas: a national study from the French Group of Endocrine Tumors and ENDOCAN-RENATEN network.
Perrier M, Brugel M, Gérard L, Goichot B, Lièvre A, Lepage C, Hautefeuille V, Do Cao C, Smith D, Thuillier P, Cros J, Cadiot G, Walter T, de Mestier L
Eur J Endocrinol 2023 Dec 6;189(6):575-583. doi: 10.1093/ejendo/lvad157. PMID: 38039101
Tumor-Infiltrating Neutrophils Predict Poor Survival of Non-Functional Pancreatic Neuroendocrine Tumor.
Zhang WH, Wang WQ, Gao HL, Xu SS, Li S, Li TJ, Han X, Xu HX, Li H, Jiang W, Ye LY, Lin X, Wu CT, Yu XJ, Liu L
J Clin Endocrinol Metab 2020 Jul 1;105(7) doi: 10.1210/clinem/dgaa196. PMID: 32285127
Clinical characteristics and management of insulin-producing neuroendocrine carcinomas.
Lowette K, Verslype C, Van Cutsem E
Acta Gastroenterol Belg 2016 Jul-Sep;79(2):321-327. PMID: 27821028
Non-functional pancreatic neuroendocrine tumor as an incidentaloma--a case report and review of literature.
Rustagi T, Rai M, Bauer F
J Gastrointest Cancer 2013 Sep;44(3):336-42. doi: 10.1007/s12029-012-9445-7. PMID: 23065708

Clinical prediction guides

ABCD1 as a Novel Diagnostic Marker for Solid Pseudopapillary Neoplasm of the Pancreas.
Liu YA, Liu Y, Tu J, Shi Y, Pang J, Huang Q, Wang X, Lin Z, Zhao Y, Wang W, Peng J, Wu W
Am J Surg Pathol 2024 May 1;48(5):511-520. Epub 2024 Apr 3 doi: 10.1097/PAS.0000000000002205. PMID: 38567813Free 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
Tumor-Infiltrating Neutrophils Predict Poor Survival of Non-Functional Pancreatic Neuroendocrine Tumor.
Zhang WH, Wang WQ, Gao HL, Xu SS, Li S, Li TJ, Han X, Xu HX, Li H, Jiang W, Ye LY, Lin X, Wu CT, Yu XJ, Liu L
J Clin Endocrinol Metab 2020 Jul 1;105(7) doi: 10.1210/clinem/dgaa196. PMID: 32285127
Pitfalls in diagnosing a pancreatic neuroendocrine tumor: a case report.
Popa SG, Ungureanu BS, Gheonea IA, Mitrea A, Ardeleanu CM, Ghiluşi MC, Şurlin V, Georgescu EF, Georgescu I, MoŢa M, Marioara OM, Săftoiu A
Rom J Morphol Embryol 2015;56(4):1495-502. PMID: 26743299

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