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Metastatic Pancreatic Neuroendocrine Tumor

MedGen UID:
1662841
•Concept ID:
C2609093
•
Neoplastic Process
Synonym: Metastatic Pancreatic Islet Cell Tumor

Definition

A neuroendocrine tumor that arises from the islet cells of the pancreas and has spread to another anatomic site. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMetastatic Pancreatic Neuroendocrine Tumor

Professional guidelines

PubMed

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 North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Management of Metastatic and/or Unresectable Pheochromocytoma and Paraganglioma.
Fishbein L, Del Rivero J, Else T, Howe JR, Asa SL, Cohen DL, Dahia PLM, Fraker DL, Goodman KA, Hope TA, Kunz PL, Perez K, Perrier ND, Pryma DA, Ryder M, Sasson AR, Soulen MC, Jimenez C
Pancreas 2021 Apr 1;50(4):469-493. doi: 10.1097/MPA.0000000000001792. PMID: 33939658
Management of immune-related adverse events and kinetics of response with ipilimumab.
Weber JS, Kähler KC, Hauschild A
J Clin Oncol 2012 Jul 20;30(21):2691-7. Epub 2012 May 21 doi: 10.1200/JCO.2012.41.6750. PMID: 22614989

Recent clinical studies

Etiology

Opsoclonus-myoclonus syndrome associated with pancreatic neuroendocrine tumor: a case report.
Reinecke R, Reiländer A, Seiler A, Koch C, Voss M
BMC Neurol 2022 Dec 30;22(1):507. doi: 10.1186/s12883-022-03012-6. PMID: 36581905Free PMC Article
Nomograms predict survival outcomes for distant metastatic pancreatic neuroendocrine tumor: A population based STROBE compliant study.
Li G, Tian ML, Bing YT, Wang HY, Yuan CH, Xiu DR
Medicine (Baltimore) 2020 Mar;99(13):e19593. doi: 10.1097/MD.0000000000019593. PMID: 32221079Free PMC Article
The impact of surgery for metastatic pancreatic neuroendocrine tumor: a contemporary evaluation matching for chromogranin a level.
Concors SJ, Sinnamon AJ, Ecker BL, Metz DC, Vollmer CM, Fraker DL, Roses RE
HPB (Oxford) 2020 Jan;22(1):83-90. Epub 2019 Jun 22 doi: 10.1016/j.hpb.2019.05.011. PMID: 31239188
Primary site surgery for elderly patients with distant metastatic pancreatic neuroendocrine tumor: to do or not to do?
Zhang J, Peng CS, Tian YH
Clin Interv Aging 2019;14:1419-1432. Epub 2019 Aug 6 doi: 10.2147/CIA.S209428. PMID: 31496669Free PMC Article
Management of the primary tumor in patients with metastatic pancreatic neuroendocrine tumor: a contemporary single-institution review.
Bruzoni M, Parikh P, Celis R, Are C, Ly QP, Meza JL, Sasson AR
Am J Surg 2009 Mar;197(3):376-81. doi: 10.1016/j.amjsurg.2008.11.005. PMID: 19245918

Diagnosis

Opsoclonus-myoclonus syndrome associated with pancreatic neuroendocrine tumor: a case report.
Reinecke R, Reiländer A, Seiler A, Koch C, Voss M
BMC Neurol 2022 Dec 30;22(1):507. doi: 10.1186/s12883-022-03012-6. PMID: 36581905Free PMC Article
Rare BRAF mutations in pancreatic neuroendocrine tumors may predict response to RAF and MEK inhibition.
Allen A, Qin ACR, Raj N, Wang J, Uddin S, Yao Z, Tang L, Meyers PA, Taylor BS, Berger MF, Yaeger R, Reidy-Lagunes D, Pratilas CA
PLoS One 2019;14(6):e0217399. Epub 2019 Jun 3 doi: 10.1371/journal.pone.0217399. PMID: 31158244Free PMC Article
Bone marrow edema associated with everolimus.
McDevitt RL, Quinlan C, Hersberger K, Sahai V
Am J Health Syst Pharm 2018 Jan 1;75(1):e23-e27. doi: 10.2146/ajhp170269. PMID: 29273609
Metastatic pancreatic neuroendocrine tumor to the central nervous system in a patient with von Hippel-Lindau disease: A case report and literature review.
Reynolds MR, Crilly SM, Sweeney KJ, Farrell M, Rawluk D
Br J Neurosurg 2015 Apr;29(2):291-3. Epub 2014 Sep 18 doi: 10.3109/02688697.2014.957650. PMID: 25232806
High KIT and PDGFRA are associated with shorter patients survival in gastroenteropancreatic neuroendocrine tumors, but mutations are a rare event.
Knösel T, Chen Y, Altendorf-Hofmann A, Danielczok C, Freesmeyer M, Settmacher U, Wurst C, Schulz S, Yang LL, Petersen I
J Cancer Res Clin Oncol 2012 Mar;138(3):397-403. Epub 2011 Dec 8 doi: 10.1007/s00432-011-1107-9. PMID: 22160160

Therapy

Meningioma Presenting with Spontaneous Venous Intraparenchymal Hemorrhage.
Giantini-Larsen A, Abou-Mrad Z, Moss NS
World Neurosurg 2023 Mar;171:8-9. Epub 2022 Dec 8 doi: 10.1016/j.wneu.2022.12.007. PMID: 36503122Free PMC Article
Nomograms predict survival outcomes for distant metastatic pancreatic neuroendocrine tumor: A population based STROBE compliant study.
Li G, Tian ML, Bing YT, Wang HY, Yuan CH, Xiu DR
Medicine (Baltimore) 2020 Mar;99(13):e19593. doi: 10.1097/MD.0000000000019593. PMID: 32221079Free PMC Article
Primary site surgery for elderly patients with distant metastatic pancreatic neuroendocrine tumor: to do or not to do?
Zhang J, Peng CS, Tian YH
Clin Interv Aging 2019;14:1419-1432. Epub 2019 Aug 6 doi: 10.2147/CIA.S209428. PMID: 31496669Free PMC Article
A young man with persistent dyspepsia: the unexpected virtue of proton-pump inhibitors.
Fraticelli P, Mattioli M, Benfaremo D, Biondi L, Cardinali M, Gabrielli A
Acta Biomed 2019 Jan 15;89(4):569-572. doi: 10.23750/abm.v89i4.6371. PMID: 30657126Free PMC Article
Bone marrow edema associated with everolimus.
McDevitt RL, Quinlan C, Hersberger K, Sahai V
Am J Health Syst Pharm 2018 Jan 1;75(1):e23-e27. doi: 10.2146/ajhp170269. PMID: 29273609

Prognosis

Severe hypercalcemia due to metastatic pancreatic neuroendocrine tumor: a case report.
Behdadnia A, Jeddi M
J Med Case Rep 2023 Sep 6;17(1):400. doi: 10.1186/s13256-023-04042-5. PMID: 37670372Free PMC Article
The impact of surgery for metastatic pancreatic neuroendocrine tumor: a contemporary evaluation matching for chromogranin a level.
Concors SJ, Sinnamon AJ, Ecker BL, Metz DC, Vollmer CM, Fraker DL, Roses RE
HPB (Oxford) 2020 Jan;22(1):83-90. Epub 2019 Jun 22 doi: 10.1016/j.hpb.2019.05.011. PMID: 31239188
Rare BRAF mutations in pancreatic neuroendocrine tumors may predict response to RAF and MEK inhibition.
Allen A, Qin ACR, Raj N, Wang J, Uddin S, Yao Z, Tang L, Meyers PA, Taylor BS, Berger MF, Yaeger R, Reidy-Lagunes D, Pratilas CA
PLoS One 2019;14(6):e0217399. Epub 2019 Jun 3 doi: 10.1371/journal.pone.0217399. PMID: 31158244Free PMC Article
Pancreaticoduodenectomy and metastasectomy for metastatic pancreatic neuroendocrine tumors.
Chawla A, Williams RT, Sich N, Clancy T, Wang J, Ashley S, Pezzi C, Swanson R
J Surg Oncol 2018 Nov;118(6):983-990. Epub 2018 Sep 13 doi: 10.1002/jso.25219. PMID: 30212595
High KIT and PDGFRA are associated with shorter patients survival in gastroenteropancreatic neuroendocrine tumors, but mutations are a rare event.
Knösel T, Chen Y, Altendorf-Hofmann A, Danielczok C, Freesmeyer M, Settmacher U, Wurst C, Schulz S, Yang LL, Petersen I
J Cancer Res Clin Oncol 2012 Mar;138(3):397-403. Epub 2011 Dec 8 doi: 10.1007/s00432-011-1107-9. PMID: 22160160

Clinical prediction guides

Nomograms predict survival outcomes for distant metastatic pancreatic neuroendocrine tumor: A population based STROBE compliant study.
Li G, Tian ML, Bing YT, Wang HY, Yuan CH, Xiu DR
Medicine (Baltimore) 2020 Mar;99(13):e19593. doi: 10.1097/MD.0000000000019593. PMID: 32221079Free PMC Article
The impact of surgery for metastatic pancreatic neuroendocrine tumor: a contemporary evaluation matching for chromogranin a level.
Concors SJ, Sinnamon AJ, Ecker BL, Metz DC, Vollmer CM, Fraker DL, Roses RE
HPB (Oxford) 2020 Jan;22(1):83-90. Epub 2019 Jun 22 doi: 10.1016/j.hpb.2019.05.011. PMID: 31239188
Rare BRAF mutations in pancreatic neuroendocrine tumors may predict response to RAF and MEK inhibition.
Allen A, Qin ACR, Raj N, Wang J, Uddin S, Yao Z, Tang L, Meyers PA, Taylor BS, Berger MF, Yaeger R, Reidy-Lagunes D, Pratilas CA
PLoS One 2019;14(6):e0217399. Epub 2019 Jun 3 doi: 10.1371/journal.pone.0217399. PMID: 31158244Free PMC Article
Bone marrow edema associated with everolimus.
McDevitt RL, Quinlan C, Hersberger K, Sahai V
Am J Health Syst Pharm 2018 Jan 1;75(1):e23-e27. doi: 10.2146/ajhp170269. PMID: 29273609
High KIT and PDGFRA are associated with shorter patients survival in gastroenteropancreatic neuroendocrine tumors, but mutations are a rare event.
Knösel T, Chen Y, Altendorf-Hofmann A, Danielczok C, Freesmeyer M, Settmacher U, Wurst C, Schulz S, Yang LL, Petersen I
J Cancer Res Clin Oncol 2012 Mar;138(3):397-403. Epub 2011 Dec 8 doi: 10.1007/s00432-011-1107-9. PMID: 22160160

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