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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]

Professional guidelines

PubMed

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
ENETS Consensus Guidelines Update for the Management of Distant Metastatic Disease of Intestinal, Pancreatic, Bronchial Neuroendocrine Neoplasms (NEN) and NEN of Unknown Primary Site.
Pavel M, O'Toole D, Costa F, Capdevila J, Gross D, Kianmanesh R, Krenning E, Knigge U, Salazar R, Pape UF, Öberg K; Vienna Consensus Conference participants
Neuroendocrinology 2016;103(2):172-85. Epub 2016 Jan 5 doi: 10.1159/000443167. PMID: 26731013
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

Distinctive grade based on Ki67 index and immune microenvironment of metastatic pancreatic neuroendocrine tumors responding to capecitabine plus temozolomide.
Gao H, Zhang W, Li Z, Liu W, Liu M, Zhuo Q, Shi Y, Xu W, Zhou C, Qin Y, Xu J, Chen J, Yu X, Xu X, Ji S
BMC Cancer 2024 Nov 7;24(1):1362. doi: 10.1186/s12885-024-13117-5. PMID: 39511555Free PMC Article
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

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
Belzutifan in a Patient With VHL-Associated Metastatic Pancreatic Neuroendocrine Tumor.
Pelle E, Al-Toubah T, Morse B, Strosberg J
J Natl Compr Canc Netw 2022 Dec;20(12):1285-1287. doi: 10.6004/jnccn.2022.7047. PMID: 36509068
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
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
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

Therapy

Distinctive grade based on Ki67 index and immune microenvironment of metastatic pancreatic neuroendocrine tumors responding to capecitabine plus temozolomide.
Gao H, Zhang W, Li Z, Liu W, Liu M, Zhuo Q, Shi Y, Xu W, Zhou C, Qin Y, Xu J, Chen J, Yu X, Xu X, Ji S
BMC Cancer 2024 Nov 7;24(1):1362. doi: 10.1186/s12885-024-13117-5. PMID: 39511555Free PMC Article
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
DNA repair enzyme expression and differential response to temozolomide in a patient with both glioblastoma and metastatic pancreatic neuroendocrine tumor.
Bracht LK, Wen P, Meyerhardt JA, Kulke MH, Hornick JL, Redston M, LaFrankie DC, Black PM, Kesari S, Norden A, Drappatz J
J Clin Oncol 2008 Oct 10;26(29):4843-4. Epub 2008 Sep 8 doi: 10.1200/JCO.2008.18.1776. PMID: 18779600

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
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
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
DNA repair enzyme expression and differential response to temozolomide in a patient with both glioblastoma and metastatic pancreatic neuroendocrine tumor.
Bracht LK, Wen P, Meyerhardt JA, Kulke MH, Hornick JL, Redston M, LaFrankie DC, Black PM, Kesari S, Norden A, Drappatz J
J Clin Oncol 2008 Oct 10;26(29):4843-4. Epub 2008 Sep 8 doi: 10.1200/JCO.2008.18.1776. PMID: 18779600

Clinical prediction guides

Usefulness of an immunohistochemical score in advanced pancreatic neuroendocrine tumors treated with CAPTEM or everolimus.
Viúdez A, Crespo G, Gómez Dorronsoro ML, Arozarena I, Marín-Méndez JJ, Custodio A, Benavent M, Goñi S, García-Paredes B, Hernando J, Durantez M, Alonso V, Riesco MDC, López C, Jiménez-Fonseca P, San Vicente BL, González-Borja I, Sevilla I, Hernández-Garcia I, Carmona-Bayonas A, Capdevila J, Pérez-Sanz J, García-Carbonero R, Pérez-Ricarte L, Llanos M, Vera R, De Jesús Acosta A
Pancreatology 2021 Jan;21(1):215-223. Epub 2020 Dec 19 doi: 10.1016/j.pan.2020.12.009. PMID: 33358592
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
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

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