Abstract
Neuroendocrine tumors (NETs) constitute a group of malignancies arising from neuroendocrine precursor cells that can arise anywhere in the body. The incidence and prevalence of NETs have steadily increased over the past four decades. Given their heterogeneous and often indolent presentation, the initial diagnosis of NETs can be challenging. In this chapter, we provide a comprehensive overview on the epidemiology and diagnosis of NETs.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
References
Dasari A, Shen C, Halperin D, et al. Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol. 2017;3(10):1335–42.
Choti MA, Bobiak S, Strosberg JR, et al. Prevalence of functional tumors in neuroendocrine carcinoma: an analysis from the NCCN NET database. J Clin Oncol. 2012;30(15_suppl):4126.
Broder MS, Cai B, Chang E, Neary MP. Incidence and prevalence of neuroendocrine tumors of the lung: analysis of a US commercial insurance claims database. BMC Pulm Med. 2018;18(1):135.
Smith-Bindman R, Kwan ML, Marlow EC, et al. Trends in use of medical imaging in US health care systems and in Ontario, Canada, 2000-2016. JAMA. 2019;322(9):843–56.
Shen C, Gu D, Zhou S, et al. Racial differences in the incidence and survival of patients with neuroendocrine tumors. Pancreas. 2019;48(10):1373–9.
Obendorfer. Karzinoide tumoren des dunndarms. Frankf Z Pathol. 1907;1:425–9.
Ter-Minassian M, Chan JA, Hooshmand SM, et al. Clinical presentation, recurrence, and survival in patients with neuroendocrine tumors: results from a prospective institutional database. Endocr Relat Cancer. 2013;20(2):187–96.
Verbeek WH, Korse CM, Tesselaar ME. GEP-NETs UPDATE: secreting gastro-enteropancreatic neuroendocrine tumours and biomarkers. Eur J Endocrinol. 2016;174(1):R1–7.
Soga J, Yakuwa Y, Osaka M. Carcinoid syndrome: a statistical evaluation of 748 reported cases. J Exp Clin Cancer Res. 1999;18(2):133–41.
Palaniswamy C, Frishman WH, Aronow WS. Carcinoid heart disease. Cardiol Rev. 2012;20(4):167–76.
Feldman JM, O’Dorisio TM. Role of neuropeptides and serotonin in the diagnosis of carcinoid tumors. Am J Med. 1986;81(6B):41–8.
Tellez MR, Mamikunian G, O'Dorisio TM, Vinik AI, Woltering EA. A single fasting plasma 5-HIAA value correlates with 24-hour urinary 5-HIAA values and other biomarkers in midgut neuroendocrine tumors (NETs). Pancreas. 2013;42(3):405–10.
Carling RS, Degg TJ, Allen KR, Bax ND, Barth JH. Evaluation of whole blood serotonin and plasma and urine 5-hydroxyindole acetic acid in diagnosis of carcinoid disease. Ann Clin Biochem 2002;39(Pt 6):577–582.
Yang X, Yang Y, Li Z, et al. Diagnostic value of circulating chromogranin a for neuroendocrine tumors: a systematic review and meta-analysis. PLoS One. 2015;10(4):e0124884.
Metz DC, Jensen RT. Gastrointestinal neuroendocrine tumors: pancreatic endocrine tumors. Gastroenterology. 2008;135(5):1469–92.
Yu F, Venzon DJ, Serrano J, et al. Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome. J Clin Oncol. 1999;17(2):615–30.
Ito T, Cadiot G, Jensen RT. Diagnosis of Zollinger-Ellison syndrome: increasingly difficult. World J Gastroenterol. 2012;18(39):5495–503.
Feng J, Petersen CD, Coy DH, et al. Calcium-sensing receptor is a physiologic multimodal chemosensor regulating gastric G-cell growth and gastrin secretion. Proc Natl Acad Sci U S A. 2010;107(41):17791–6.
Poitras P, Gingras MH, Rehfeld JF. Secretin stimulation test for gastrin release in Zollinger-Ellison syndrome: to do or not to do? Pancreas. 2013;42(6):903–4.
Yao JC, Eisner MP, Leary C, et al. Population-based study of islet cell carcinoma. Ann Surg Oncol. 2007;14(12):3492–500.
Wu SL, Bai JG, Xu J, Ma QY, Wu Z. Necrolytic migratory erythema as the first manifestation of pancreatic neuroendocrine tumor. World J Surg Oncol. 2014;12:220.
Castro PG, de Leon AM, Trancon JG, et al. Glucagonoma syndrome: a case report. J Med Case Rep. 2011;5:402.
Eldor R, Glaser B, Fraenkel M, Doviner V, Salmon A, Gross DJ. Glucagonoma and the glucagonoma syndrome – cumulative experience with an elusive endocrine tumour. Clin Endocrinol. 2011;74(5):593–8.
Kimbara S, Fujiwara Y, Toyoda M, et al. Rapid improvement of glucagonoma-related necrolytic migratory erythema with octreotide. Clin J Gastroenterol. 2014;7(3):255–9.
Kanakis G, Kaltsas G. Biochemical markers for gastroenteropancreatic neuroendocrine tumours (GEP-NETs). Best Pract Res Clin Gastroenterol. 2012;26(6):791–802.
La Rosa S, Klersy C, Uccella S, et al. Improved histologic and clinicopathologic criteria for prognostic evaluation of pancreatic endocrine tumors. Hum Pathol. 2009;40(1):30–40.
Service FJ, McMahon MM, O'Brien PC, Ballard DJ. Functioning insulinoma – incidence, recurrence, and long-term survival of patients: a 60-year study. Mayo Clin Proc. 1991;66(7):711–9.
Nozieres C, Chardon L, Goichot B, et al. Neuroendocrine tumors producing calcitonin: characteristics, prognosis and potential interest of calcitonin monitoring during follow-up. Eur J Endocrinol. 2016;174(3):335–41.
Adrian TE, Uttenthal LO, Williams SJ, Bloom SR. Secretion of pancreatic polypeptide in patients with pancreatic endocrine tumors. N Engl J Med. 1986;315(5):287–91.
Panzuto F, Severi C, Cannizzaro R, et al. Utility of combined use of plasma levels of chromogranin A and pancreatic polypeptide in the diagnosis of gastrointestinal and pancreatic endocrine tumors. J Endocrinol Investig. 2004;27(1):6–11.
Walter T, Chardon L, Chopin-laly X, et al. Is the combination of chromogranin A and pancreatic polypeptide serum determinations of interest in the diagnosis and follow-up of gastro-entero-pancreatic neuroendocrine tumours? Eur J Cancer. 2012;48(12):1766–73.
Deppen SA, Liu E, Blume JD, et al. Safety and efficacy of 68Ga-DOTATATE PET/CT for diagnosis, staging, and treatment management of neuroendocrine tumors. J Nucl Med. 2016;57(5):708–14.
Sundin A, Arnold R, Baudin E, et al. ENETS consensus guidelines for the standards of care in neuroendocrine tumors: radiological, nuclear medicine & hybrid imaging. Neuroendocrinology. 2017;105(3):212–44.
Ayati N, Lee ST, Zakavi R, et al. Long-acting somatostatin analog therapy differentially alters (68)Ga-DOTATATE uptake in normal tissues compared with primary tumors and metastatic lesions. J Nucl Med. 2018;59(2):223–7.
Bhattacharyya S, Toumpanakis C, Chilkunda D, Caplin ME, Davar J. Risk factors for the development and progression of carcinoid heart disease. Am J Cardiol. 2011;107(8):1221–6.
Korse CM, Taal BG, de Groot CA, Bakker RH, Bonfrer JM. Chromogranin-A and N-terminal pro-brain natriuretic peptide: an excellent pair of biomarkers for diagnostics in patients with neuroendocrine tumor. J Clin Oncol. 2009;27(26):4293–9.
Anlauf M, Garbrecht N, Bauersfeld J, et al. Hereditary neuroendocrine tumors of the gastroenteropancreatic system. Virchows Arch. 2007;451(Suppl 1):S29–38.
Thakker RV, Newey PJ, Walls GV, et al. Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1). J Clin Endocrinol Metab. 2012;97(9):2990–3011.
Anlauf M, Schlenger R, Perren A, et al. Microadenomatosis of the endocrine pancreas in patients with and without the multiple endocrine neoplasia type 1 syndrome. Am J Surg Pathol. 2006;30(5):560–74.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2021 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Sukrithan, V., Konda, B. (2021). Epidemiology and Diagnosis of Neuroendocrine Tumors. In: Cloyd, J.M., Pawlik, T.M. (eds) Neuroendocrine Tumors. Springer, Cham. https://doi.org/10.1007/978-3-030-62241-1_1
Download citation
DOI: https://doi.org/10.1007/978-3-030-62241-1_1
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-62240-4
Online ISBN: 978-3-030-62241-1
eBook Packages: MedicineMedicine (R0)