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Published in: Journal of Endocrinological Investigation 7/2023

01-07-2023 | Neuroendocrine Tumor | Original Article

The incidence, prevalence, and survival analysis of pancreatic neuroendocrine tumors in the United States

Authors: X. Liu, B. Chen, J. Chen, Z. Su, S. Sun

Published in: Journal of Endocrinological Investigation | Issue 7/2023

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Abstract

Purpose

The incidence of pancreatic neuroendocrine tumors (pNETs) was increasing. The main purpose of this study was to statistically analyze the incidence and prevalence of pNETs and the main risk factors for the prognosis.

Methods

Based on the Surveillance, Epidemiology, and End Results (SEER) database, with three registries integrated, this study comprehensively displayed the annual age adjust incidence of pNETs from 1975 to 2018, the estimated 20-year limited-duration prevalence, and conducted the univariate and multivariate survival analysis.

Results

The incidence of pNETs has increased to about 1.5 per 100,000 population, and the prevalence has reached about 0.008% with the aged, Grade 1 and nonfunctional tumors accounting for the majority. The average median overall survival (OS), 5-year survival rate, and median disease-free survival (DFS) of pNETs patients from 1975 to 2018 were 85 months, 57.55%, and 220 months, respectively. From 2000 to 2018, the median OS was 94 months, and the 5-year survival rate was 59.94%. In multivariate survival analysis, the greatest risk factor was Grade 3&4 with HR = 3.62 (3.10–4.28), followed by distant stage with HR = 2.77 (2.28–3.36), and aged over 80 years old with HR = 2.26 (1.33–3.83). Surgery was a protective prognostic factor with HR = 0.34 (0.29–0.40).

Conclusion

The incidence and prevalence of pNETs were still increasing, but the trend was gradual and aging in recent years. The survival time of pNETs was longer but has not changed much in recent years. The degrees of malignancy, stage, and operation were the most important prognosis factors.
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Literature
1.
go back to reference Schimmack S, Svejda B, Lawrence B, Kidd M, Modlin IM (2011) The diversity and commonalities of gastroenteropancreatic neuroendocrine tumors. Langenbecks Arch Surg 396(3):273–298PubMedCrossRef Schimmack S, Svejda B, Lawrence B, Kidd M, Modlin IM (2011) The diversity and commonalities of gastroenteropancreatic neuroendocrine tumors. Langenbecks Arch Surg 396(3):273–298PubMedCrossRef
2.
go back to reference Dasari A, Shen C, Halperin D et al (2017) Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol 3(10):1335–1342PubMedPubMedCentralCrossRef Dasari A, Shen C, Halperin D et al (2017) Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol 3(10):1335–1342PubMedPubMedCentralCrossRef
3.
go back to reference Hallet J, Law CH, Cukier M, Saskin R, Liu N, Singh S (2015) Exploring the rising incidence of neuroendocrine tumors: a population-based analysis of epidemiology, metastatic presentation, and outcomes. Cancer 121(4):589–597PubMedCrossRef Hallet J, Law CH, Cukier M, Saskin R, Liu N, Singh S (2015) Exploring the rising incidence of neuroendocrine tumors: a population-based analysis of epidemiology, metastatic presentation, and outcomes. Cancer 121(4):589–597PubMedCrossRef
4.
go back to reference Sundin A, Arnold R, Baudin E et al (2017) ENETS consensus guidelines for the standards of care in neuroendocrine tumors: radiological nuclear medicine & hybrid imaging. Neuroendocrinology 105(3):212–244PubMedCrossRef Sundin A, Arnold R, Baudin E et al (2017) ENETS consensus guidelines for the standards of care in neuroendocrine tumors: radiological nuclear medicine & hybrid imaging. Neuroendocrinology 105(3):212–244PubMedCrossRef
5.
go back to reference Liu Y, Chen W, Cui W et al (2020) Quantitative pretreatment CT parameters as predictors of tumor response of neuroendocrine tumor liver metastasis to transcatheter arterial bland embolization. Neuroendocrinology 110(7–8):697–704PubMedCrossRef Liu Y, Chen W, Cui W et al (2020) Quantitative pretreatment CT parameters as predictors of tumor response of neuroendocrine tumor liver metastasis to transcatheter arterial bland embolization. Neuroendocrinology 110(7–8):697–704PubMedCrossRef
6.
go back to reference Ruf J, Heuck F, Schiefer J et al (2010) Impact of Multiphase 68Ga-DOTATOC-PET/CT on therapy management in patients with neuroendocrine tumors. Neuroendocrinology 91(1):101–109PubMedCrossRef Ruf J, Heuck F, Schiefer J et al (2010) Impact of Multiphase 68Ga-DOTATOC-PET/CT on therapy management in patients with neuroendocrine tumors. Neuroendocrinology 91(1):101–109PubMedCrossRef
7.
go back to reference Liu Y, Shi S, Hua J et al (2020) Differentiation of solid-pseudopapillary tumors of the pancreas from pancreatic neuroendocrine tumors by using endoscopic ultrasound. Clin Res Hepatol Gastroenterol 44(6):947–953PubMedCrossRef Liu Y, Shi S, Hua J et al (2020) Differentiation of solid-pseudopapillary tumors of the pancreas from pancreatic neuroendocrine tumors by using endoscopic ultrasound. Clin Res Hepatol Gastroenterol 44(6):947–953PubMedCrossRef
8.
go back to reference Rinke A, Krug S (2016) Neuroendocrine tumours—medical therapy: biological. Best Pract Res Clin Endocrinol Metab 30(1):79–91PubMedCrossRef Rinke A, Krug S (2016) Neuroendocrine tumours—medical therapy: biological. Best Pract Res Clin Endocrinol Metab 30(1):79–91PubMedCrossRef
9.
go back to reference Wang W, Zhang Y, Peng Y et al (2021) A Ki-67 index to predict treatment response to the capecitabine/temozolomide regimen in neuroendocrine neoplasms: a retrospective multicenter study. Neuroendocrinology 111(8):752–763PubMedCrossRef Wang W, Zhang Y, Peng Y et al (2021) A Ki-67 index to predict treatment response to the capecitabine/temozolomide regimen in neuroendocrine neoplasms: a retrospective multicenter study. Neuroendocrinology 111(8):752–763PubMedCrossRef
10.
go back to reference Vaghaiwalla T, Keutgen XM (2020) Surgical management of pancreatic neuroendocrine tumors. Surg Oncol Clin N Am 29(2):243–252PubMedCrossRef Vaghaiwalla T, Keutgen XM (2020) Surgical management of pancreatic neuroendocrine tumors. Surg Oncol Clin N Am 29(2):243–252PubMedCrossRef
11.
go back to reference Scott AT, Howe JR (2019) Evaluation and management of neuroendocrine tumors of the pancreas. Surg Clin N Am 99(4):793–814PubMedCrossRef Scott AT, Howe JR (2019) Evaluation and management of neuroendocrine tumors of the pancreas. Surg Clin N Am 99(4):793–814PubMedCrossRef
12.
go back to reference Perri G, Prakash LR, Katz M (2019) Pancreatic neuroendocrine tumors. Curr Opin Gastroenterol 35(5):468–477PubMedCrossRef Perri G, Prakash LR, Katz M (2019) Pancreatic neuroendocrine tumors. Curr Opin Gastroenterol 35(5):468–477PubMedCrossRef
13.
go back to reference Landoni L, Marchegiani G, Pollini T et al (2019) The evolution of surgical strategies for pancreatic neuroendocrine tumors (Pan-NENs): time-trend and outcome analysis from 587 consecutive resections at a high-volume institution. Ann Surg 269(4):725–732PubMedCrossRef Landoni L, Marchegiani G, Pollini T et al (2019) The evolution of surgical strategies for pancreatic neuroendocrine tumors (Pan-NENs): time-trend and outcome analysis from 587 consecutive resections at a high-volume institution. Ann Surg 269(4):725–732PubMedCrossRef
14.
go back to reference Mpilla GB, Philip PA, El-Rayes B, Azmi AS (2020) Pancreatic neuroendocrine tumors: therapeutic challenges and research limitations. World J Gastroenterol 26(28):4036–4054PubMedPubMedCentralCrossRef Mpilla GB, Philip PA, El-Rayes B, Azmi AS (2020) Pancreatic neuroendocrine tumors: therapeutic challenges and research limitations. World J Gastroenterol 26(28):4036–4054PubMedPubMedCentralCrossRef
15.
go back to reference Ma ZY, Gong YF, Zhuang HK et al (2020) Pancreatic neuroendocrine tumors: a review of serum biomarkers, staging, and management. World J Gastroenterol 26(19):2305–2322PubMedPubMedCentralCrossRef Ma ZY, Gong YF, Zhuang HK et al (2020) Pancreatic neuroendocrine tumors: a review of serum biomarkers, staging, and management. World J Gastroenterol 26(19):2305–2322PubMedPubMedCentralCrossRef
16.
go back to reference Pavel M, Jann H, Prasad V, Drozdov I, Modlin IM, Kidd M (2017) NET blood transcript analysis defines the crossing of the clinical rubicon: when stable disease becomes progressive. Neuroendocrinology 104(2):170–182PubMedCrossRef Pavel M, Jann H, Prasad V, Drozdov I, Modlin IM, Kidd M (2017) NET blood transcript analysis defines the crossing of the clinical rubicon: when stable disease becomes progressive. Neuroendocrinology 104(2):170–182PubMedCrossRef
17.
go back to reference Kemm MH, Manly CD, Hoang TD, Mai VQ, Shakir M (2019) Octreotide use in a patient with MEN-1 syndrome and multifocal pancreatic neuroendocrine tumors: a case report and review of the literature. Case Rep Gastrointest Med 2019:9462942PubMedPubMedCentral Kemm MH, Manly CD, Hoang TD, Mai VQ, Shakir M (2019) Octreotide use in a patient with MEN-1 syndrome and multifocal pancreatic neuroendocrine tumors: a case report and review of the literature. Case Rep Gastrointest Med 2019:9462942PubMedPubMedCentral
18.
go back to reference Shah MH, Goldner WS, Benson AB et al (2021) Neuroendocrine and adrenal tumors, version 2.2021, NCCN clinical practice guidelines in oncology. J Natl Compr Canc Netw 19(7):839–868PubMedCrossRef Shah MH, Goldner WS, Benson AB et al (2021) Neuroendocrine and adrenal tumors, version 2.2021, NCCN clinical practice guidelines in oncology. J Natl Compr Canc Netw 19(7):839–868PubMedCrossRef
19.
go back to reference de Mestier L, Walter T, Brixi H et al (2019) Comparison of temozolomide-capecitabine to 5-fluorouracile-dacarbazine in 247 patients with advanced digestive neuroendocrine tumors using propensity score analyses. Neuroendocrinology 108(4):343–353PubMedCrossRef de Mestier L, Walter T, Brixi H et al (2019) Comparison of temozolomide-capecitabine to 5-fluorouracile-dacarbazine in 247 patients with advanced digestive neuroendocrine tumors using propensity score analyses. Neuroendocrinology 108(4):343–353PubMedCrossRef
20.
go back to reference Cives M, Ghayouri M, Morse B et al (2016) Analysis of potential response predictors to capecitabine/temozolomide in metastatic pancreatic neuroendocrine tumors. Endocr Relat Cancer 23(9):759–767PubMedCrossRef Cives M, Ghayouri M, Morse B et al (2016) Analysis of potential response predictors to capecitabine/temozolomide in metastatic pancreatic neuroendocrine tumors. Endocr Relat Cancer 23(9):759–767PubMedCrossRef
21.
go back to reference Cronin KA, Ries LA, Edwards BK (2014) The surveillance, epidemiology, and end results (SEER) program of the national cancer institute. Cancer 120(Suppl 23):3755–3757PubMedCrossRef Cronin KA, Ries LA, Edwards BK (2014) The surveillance, epidemiology, and end results (SEER) program of the national cancer institute. Cancer 120(Suppl 23):3755–3757PubMedCrossRef
22.
go back to reference Sonbol MB, Mazza GL, Mi L et al (2022) Survival and incidence patterns of pancreatic neuroendocrine tumors over the last 2 decades: a SEER Database Analysis. Oncologist 27(7):573–578PubMedPubMedCentralCrossRef Sonbol MB, Mazza GL, Mi L et al (2022) Survival and incidence patterns of pancreatic neuroendocrine tumors over the last 2 decades: a SEER Database Analysis. Oncologist 27(7):573–578PubMedPubMedCentralCrossRef
23.
go back to reference Wang J, Liu J, He C et al (2021) Trends in incidence and survival of patients with pancreatic neuroendocrine neoplasm, 1987–2016. J Oncol 2021:4302675PubMedPubMedCentralCrossRef Wang J, Liu J, He C et al (2021) Trends in incidence and survival of patients with pancreatic neuroendocrine neoplasm, 1987–2016. J Oncol 2021:4302675PubMedPubMedCentralCrossRef
25.
go back to reference Rinzivillo M, Partelli S, Prosperi D et al (2018) Clinical usefulness of (18)F-fluorodeoxyglucose positron emission tomography in the diagnostic algorithm of advanced entero-pancreatic neuroendocrine neoplasms. Oncologist 23(2):186–192PubMedCrossRef Rinzivillo M, Partelli S, Prosperi D et al (2018) Clinical usefulness of (18)F-fluorodeoxyglucose positron emission tomography in the diagnostic algorithm of advanced entero-pancreatic neuroendocrine neoplasms. Oncologist 23(2):186–192PubMedCrossRef
26.
go back to reference Dietrich CF, Jenssen C (2020) Modern ultrasound imaging of pancreatic tumors. Ultrasonography 39(2):105–113PubMedCrossRef Dietrich CF, Jenssen C (2020) Modern ultrasound imaging of pancreatic tumors. Ultrasonography 39(2):105–113PubMedCrossRef
27.
go back to reference Jansen L, Dauphin S, De Burghgraeve T, Schoenmakers B, Buntinx F, van den Akker M (2021) Caregiver burden: An increasing problem related to an aging cancer population. J Health Psychol 26(11):1833–1849PubMedCrossRef Jansen L, Dauphin S, De Burghgraeve T, Schoenmakers B, Buntinx F, van den Akker M (2021) Caregiver burden: An increasing problem related to an aging cancer population. J Health Psychol 26(11):1833–1849PubMedCrossRef
28.
go back to reference Jafari MD, Jafari F, Halabi WJ et al (2014) Colorectal cancer resections in the aging US population: a trend toward decreasing rates and improved outcomes. JAMA Surg 149(6):557–564PubMedCrossRef Jafari MD, Jafari F, Halabi WJ et al (2014) Colorectal cancer resections in the aging US population: a trend toward decreasing rates and improved outcomes. JAMA Surg 149(6):557–564PubMedCrossRef
29.
go back to reference Halfdanarson TR, Rabe KG, Rubin J, Petersen GM (2008) Pancreatic neuroendocrine tumors (PNETs): incidence, prognosis and recent trend toward improved survival. Ann Oncol 19(10):1727–1733PubMedPubMedCentralCrossRef Halfdanarson TR, Rabe KG, Rubin J, Petersen GM (2008) Pancreatic neuroendocrine tumors (PNETs): incidence, prognosis and recent trend toward improved survival. Ann Oncol 19(10):1727–1733PubMedPubMedCentralCrossRef
30.
31.
go back to reference Welch HG, Kramer BS, Black WC (2019) Epidemiologic signatures in cancer. N Engl J Med 381(14):1378–1386PubMedCrossRef Welch HG, Kramer BS, Black WC (2019) Epidemiologic signatures in cancer. N Engl J Med 381(14):1378–1386PubMedCrossRef
32.
go back to reference Shyr BS, Shyr BU, Chen SC, Shyr YM, Wang SE (2022) Impact of tumor grade on pancreatic neuroendocrine tumors. Asian J Surg 45:2659–2663PubMedCrossRef Shyr BS, Shyr BU, Chen SC, Shyr YM, Wang SE (2022) Impact of tumor grade on pancreatic neuroendocrine tumors. Asian J Surg 45:2659–2663PubMedCrossRef
33.
go back to reference Lee L, Ito T, Jensen RT (2019) Prognostic and predictive factors on overall survival and surgical outcomes in pancreatic neuroendocrine tumors: recent advances and controversies. Expert Rev Anticancer Ther 19(12):1029–1050PubMedPubMedCentralCrossRef Lee L, Ito T, Jensen RT (2019) Prognostic and predictive factors on overall survival and surgical outcomes in pancreatic neuroendocrine tumors: recent advances and controversies. Expert Rev Anticancer Ther 19(12):1029–1050PubMedPubMedCentralCrossRef
34.
go back to reference Harimoto N, Hoshino K, Muranushi R et al (2019) Significance of lymph node metastasis in resectable well-differentiated pancreatic neuroendocrine tumor. Pancreas 48(7):943–947PubMedCrossRef Harimoto N, Hoshino K, Muranushi R et al (2019) Significance of lymph node metastasis in resectable well-differentiated pancreatic neuroendocrine tumor. Pancreas 48(7):943–947PubMedCrossRef
35.
go back to reference Botling J, Lamarca A, Bajic D et al (2020) High-grade progression confers poor survival in pancreatic neuroendocrine tumors. Neuroendocrinology 110(11–12):891–898PubMedCrossRef Botling J, Lamarca A, Bajic D et al (2020) High-grade progression confers poor survival in pancreatic neuroendocrine tumors. Neuroendocrinology 110(11–12):891–898PubMedCrossRef
36.
go back to reference Fujimori N, Miki M, Lee L et al (2020) Natural history and clinical outcomes of pancreatic neuroendocrine neoplasms based on the WHO 2017 classification; a single-center experience of 30 years. Pancreatology 20(4):709–715PubMedCrossRef Fujimori N, Miki M, Lee L et al (2020) Natural history and clinical outcomes of pancreatic neuroendocrine neoplasms based on the WHO 2017 classification; a single-center experience of 30 years. Pancreatology 20(4):709–715PubMedCrossRef
37.
go back to reference Martínez ME, Anderson K, Murphy JD et al (2016) Differences in marital status and mortality by race/ethnicity and nativity among California cancer patients. Cancer 122(10):1570–1578PubMedCrossRef Martínez ME, Anderson K, Murphy JD et al (2016) Differences in marital status and mortality by race/ethnicity and nativity among California cancer patients. Cancer 122(10):1570–1578PubMedCrossRef
38.
go back to reference Cives M, Strosberg JR (2018) Gastroenteropancreatic neuroendocrine tumors. CA Cancer J Clin 68(6):471–487PubMedCrossRef Cives M, Strosberg JR (2018) Gastroenteropancreatic neuroendocrine tumors. CA Cancer J Clin 68(6):471–487PubMedCrossRef
39.
go back to reference Chen HY, Zhou YL, Chen YH et al (2020) Functionality is not an independent prognostic factor for pancreatic neuroendocrine tumors. World J Gastroenterol 26(25):3638–3649PubMedPubMedCentralCrossRef Chen HY, Zhou YL, Chen YH et al (2020) Functionality is not an independent prognostic factor for pancreatic neuroendocrine tumors. World J Gastroenterol 26(25):3638–3649PubMedPubMedCentralCrossRef
40.
go back to reference Falconi M, Eriksson B, Kaltsas G et al (2016) ENETS consensus guidelines update for the management of patients with functional pancreatic neuroendocrine tumors and non-functional pancreatic neuroendocrine tumors. Neuroendocrinology 103(2):153–171PubMedCrossRef Falconi M, Eriksson B, Kaltsas G et al (2016) ENETS consensus guidelines update for the management of patients with functional pancreatic neuroendocrine tumors and non-functional pancreatic neuroendocrine tumors. Neuroendocrinology 103(2):153–171PubMedCrossRef
41.
go back to reference Câmara-de-Souza AB, Toyoshima M, Giannella ML et al (2018) Insulinoma: a retrospective study analyzing the differences between benign and malignant tumors. Pancreatology 18(3):298–303PubMedCrossRef Câmara-de-Souza AB, Toyoshima M, Giannella ML et al (2018) Insulinoma: a retrospective study analyzing the differences between benign and malignant tumors. Pancreatology 18(3):298–303PubMedCrossRef
42.
go back to reference Assi HA, Mukherjee S, Kunz PL et al (2020) Surgery versus surveillance for well-differentiated, nonfunctional pancreatic neuroendocrine tumors: an 11-year analysis of the national cancer database. Oncologist 25(2):e276–e283PubMedCrossRef Assi HA, Mukherjee S, Kunz PL et al (2020) Surgery versus surveillance for well-differentiated, nonfunctional pancreatic neuroendocrine tumors: an 11-year analysis of the national cancer database. Oncologist 25(2):e276–e283PubMedCrossRef
44.
go back to reference Stueven AK, Kayser A, Wetz C et al (2019) Somatostatin analogues in the treatment of neuroendocrine tumors: past, present and future. Int J Mol Sci 20(12):3049PubMedPubMedCentralCrossRef Stueven AK, Kayser A, Wetz C et al (2019) Somatostatin analogues in the treatment of neuroendocrine tumors: past, present and future. Int J Mol Sci 20(12):3049PubMedPubMedCentralCrossRef
Metadata
Title
The incidence, prevalence, and survival analysis of pancreatic neuroendocrine tumors in the United States
Authors
X. Liu
B. Chen
J. Chen
Z. Su
S. Sun
Publication date
01-07-2023
Publisher
Springer International Publishing
Published in
Journal of Endocrinological Investigation / Issue 7/2023
Electronic ISSN: 1720-8386
DOI
https://doi.org/10.1007/s40618-022-01985-2

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