Skip to main content
SpringerLink
Log in

Sporadic Gastroenteropancreatic Neuroendocrine Tumors

  • Chapter
Abdominal Neuroendocrine Tumors

Part of the book series: Updates in Surgery ((UPDATESSURG))

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 109.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Kaltsas GA, Besser GM, Grossman AB (2004) The diagnosis and medical management of advanced neuroendocrine tumors. Endocr Rev 25(3):458–511

    Article  CAS  PubMed  Google Scholar 

  2. Modlin IM, Oberg K, Chung DC et al (2008) Gastroenteropancreatic neuroendocrine tumours. Lancet Oncol 9(1):61–72

    Article  CAS  PubMed  Google Scholar 

  3. Tomassetti P, Migliori M, Lalli S et al (2001) Epidemiology, clinical features and diagnosis of gastroenteropancreatic endocrine tumours. Ann Oncol 12(Suppl 2):S95–S99

    Article  PubMed  Google Scholar 

  4. Moller JE, Connolly HM, Rubin J et al (2003) Factors associated with progression of carcinoid heart disease. N Engl J Med 348(11): 1005–1015

    Article  PubMed  Google Scholar 

  5. Bernheim AM, Connolly HM, Pellikka PA (2007) Carcinoid heart disease in patients without hepatic metastases. Am J Cardiol 99(2):292–294

    Article  PubMed  Google Scholar 

  6. Appetecchia M, Baldelli R (2010) Somatostatin analogues in the treatment of gastroenteropancreatic neuroendocrine tumours, current aspects and new perspectives. J Exp Clin Cancer Res 29:19

    Article  PubMed  PubMed Central  Google Scholar 

  7. Eriksson B, Kloppel G, Krenning E et al (2008) Consensus guidelines for the management of patients with digestive neuroendocrine tumors — well-differentiated jejunal-ileal tumor/ carcinoma. Neuroendocrinology 87(1):8–19

    Article  CAS  PubMed  Google Scholar 

  8. Modlin IM, Kidd M, Latich I et al (2005) Current status of gastrointestinal carcinoids. Gastroenterology 128(6): 1717–1751

    Article  PubMed  Google Scholar 

  9. van der Lely AJ, de Herder WW (2005) Carcinoid syndrome: diagnosis and medical management. Arq Bras Endocrinol Metabol 49(5):850–860

    Article  PubMed  Google Scholar 

  10. Vinik AI, McLeod MK, Fig LM et al (1989) Clinical features, diagnosis, and localization of carcinoid tumors and their management. Gastroenterol Clin North Am 18(4):865–896

    CAS  PubMed  Google Scholar 

  11. Mota JM, Sousa LG, Riechelmann RP (2016) Complications from carcinoid syndrome: review of the current evidence. Ecancermedicalscience 10:662 doi:10.3332/ecancer.2016.662

    Article  PubMed  PubMed Central  Google Scholar 

  12. Anthuber M, Pratschke E, Jauch KW et al (1992) Liver retransplantation — indications, frequency, results. Transplant Proc 24(5): 1965–1966

    CAS  PubMed  Google Scholar 

  13. Walsh JS, Newell-Price JD, DeBono M et al (2013) Circulating serotonin and bone density structure, and turnover in carcinoid syndrome. J Clin Endocrinol Metab 98(7):2902–2907

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Shah GM, Shah RG, Veillette H et al (2005) Biochemical assessment of niacin deficiency among carcinoid cancer patients. Am J Gastroenterol 100(10):2307–2314

    Article  CAS  PubMed  Google Scholar 

  15. Chambers AJ, Longman RS, Pasieka JL et al (2010) Impairment of cognitive function reported by patients suffering from carcinoid syndrome. World J Surg 34(6): 1356–1360

    Article  PubMed  Google Scholar 

  16. Russo S, Boon JC, Kema IP et al (2004) Patients with carcinoid syndrome exhibit symptoms of aggressive impulse dysregulation. Psychosom Med 66(3):422–425

    PubMed  Google Scholar 

  17. Engelman K, Lovenberg W, Sjoerdsma A (1967) Inhibition of serotonin synthesis by para-chlorophenylalanine in patients with the carcinoid syndrome. N Engl J Med 277(21): 1103–1108

    Article  CAS  PubMed  Google Scholar 

  18. Pasieka JL Longman RS, Chambers AJ et al (2014) Cognitive impairment associated with carcinoid syndrome. Ann Surg 259(2):355–359

    Article  PubMed  Google Scholar 

  19. Grozinsky-Glasberg S, Grossman AB, Gross DJ (2015) Carcinoid heart disease: from pathophysiology to treatment — ‘Something in the way it moves’. Neuroendocrinology 101(4):263–273

    Article  CAS  PubMed  Google Scholar 

  20. Simula DV, Edwards WD, Tazelaar HD et al (2002) Surgical pathology of carcinoid heart disease: A study of 139 valves from 75 patients spanning 20 years. Mayo Clin Proc 77(2): 139–147

    Article  PubMed  Google Scholar 

  21. Anthony LB (2013) Practical guide to supportive care of patients with functional neuroendocrine tumors. Semin Oncol 40(1):45–55

    Article  PubMed  Google Scholar 

  22. Kulke MH, Mayer RJ (1999) Carcinoid tumors. N Engl J Med 340(11):858–868

    Article  CAS  PubMed  Google Scholar 

  23. Kahil ME, Brown H, Fred HL (1964) The carcinoid crisis. Arch Intern Med 114:26–28

    Article  CAS  PubMed  Google Scholar 

  24. Moneret-Vautrin DA, Laxenaire MC (1993) Anaphylactoid or carcinoid crisis? Br J Anaesth 71(4):609–610

    Article  CAS  PubMed  Google Scholar 

  25. Yao JC, Hassan M, Phan A et al (2008) One hundred years after “carcinoid”: Epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 26(18):3063–3072

    Article  PubMed  Google Scholar 

  26. Somasundaram NP, Garusinghe C, Berney D, Grossman AB (2013) A thymic carcinoid tumour causing Zollinger-Ellison and Cushing’s syndromes due to ectopic ACTH and gastrin secretion. Hormones (Athens) 12(2):305–308

    Article  Google Scholar 

  27. Metz DC, Jensen RT (2008) Gastrointestinal neuroendocrine tumors: Pancreatic endocrine tumors. Gastroenterology 135(5): 1469–1492

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Jensen RT, Niederle B, Mitry E et al (2006) Gastrinoma (duodenal and pancreatic). Neuroendocrinology 84(3): 173–182

    Article  CAS  PubMed  Google Scholar 

  29. Oberg K (2010) Pancreatic endocrine tumors. Semin Oncol 37(6):594–618

    Article  PubMed  Google Scholar 

  30. Ellison EC, Johnson JA (2009) The Zollinger-Ellison syndrome: A comprehensive review of historical, scientific, and clinical considerations. Curr Probl Surg 46(1): 13–106

    Article  PubMed  Google Scholar 

  31. Jensen RT, Cadiot G, Brandi ML et al (2012) ENETS Consensus guidelines for the management of patients with digestive neuroendocrine neoplasms: Functional pancreatic endocrine tumor syndromes. Neuroendocrinology 95(2):98–119

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  32. Anlauf M, Garbrecht N, Henopp T et al (2006) Sporadic versus hereditary gastrinomas of the duodenum and pancreas: distinct clinico-pathological and epidemiological features. World J Gastroenterol 12(34):5440–5446

    Article  PubMed  PubMed Central  Google Scholar 

  33. Kloppel G, Anlauf M (2007) Gastrinoma — morphological aspects. Wien Klin Wochenschr 119(19–20):579–584

    Article  PubMed  Google Scholar 

  34. Gibril F, Jensen RT (2005) Advances in evaluation and management of gastrinoma in patients with Zollinger-Ellison syndrome. Curr Gastroenterol Rep 7(2): 114–121

    Article  PubMed  Google Scholar 

  35. Jensen RT (1996) Gastrointestinal endocrine tumors. Gastrinoma. Baillieres Clin Gastroenterol 10(4):603–643

    Article  CAS  PubMed  Google Scholar 

  36. Roy PK, Venzon DJ, Shojamanesh H et al (2000) Zollinger-Ellison syndrome. Clinical presentation in 261 patients. Medicine (Baltimore) 79(6):379–411

    Article  CAS  Google Scholar 

  37. Roy PK, Venzon DJ, Feigenbaum KM et al (2001) Gastric secretion in Zollinger-Ellison syndrome. Correlation with clinical expression, tumor extent and role in diagnosis — a prospective NIH study of 235 patients and a review of 984 cases in the literature. Medicine (Baltimore) 80(3): 189–222

    CAS  Google Scholar 

  38. Osefo N, Ito T, Jensen RT (2009) Gastric acid hypersecretory states: recent insights and advances. Curr Gastroenterol Rep 11(6):433–441

    Article  PubMed  PubMed Central  Google Scholar 

  39. Gibril F, Schumann M, Pace A, Jensen RT (2004) Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective study of 107 cases and comparison with 1009 cases from the literature. Medicine (Baltimore) 83(1):43–83

    Article  Google Scholar 

  40. Jensen RT, Berna MJ, Bingham DB, Norton, JA (2008) Inherited pancreatic endocrine tumor syndromes: advances in molecular pathogenesis, diagnosis, management, and controversies. Cancer 113(Suppl 7): 1807–1843

    Article  PubMed  PubMed Central  Google Scholar 

  41. Vanderveen K, Grant C (2010) Insulinoma. Cancer Treat Res 153:235–252

    Article  PubMed  Google Scholar 

  42. de Herder WW, Niederle B, Scoazec JY et al (2006) Well-differentiated pancreatic tumor/ carcinoma: Insulinoma. Neuroendocrinology 84(3): 183–188

    Article  PubMed  Google Scholar 

  43. Zhao YP, Zhan HX, Zhang TP et al (2011) Surgical management of patients with insulinomas: result of 292 cases in a single institution. J Surg Oncol 103(2): 169–174

    Article  PubMed  Google Scholar 

  44. Jensen RT, Norton JA (2010) Endocrine tumors of the pancreas and gastrointestinal tract. In: Feldman M, Friedman LS, Brandt LJ (eds) Sleisenger and Fordtran’s Gastrointestinal and liver disease, 9th edn. Elsevier Saunders, Philadelphia, pp 491–522

    Chapter  Google Scholar 

  45. Stefanini P, Carboni M, Patrassi N, Basoli A (1974) Beta-islet cell tumors of the pancreas: results of a study on 1,067 cases. Surgery 75(4):597–609

    CAS  PubMed  Google Scholar 

  46. Grant CS (2005) Insulinoma. Best Pract Res Clin Gastroenterol 19(5):783–798

    Article  CAS  PubMed  Google Scholar 

  47. Cryer PE, Axelrod L, Grossman AB et al (2009) Evaluation and management of adult hypoglycemic disorders: An Endocrine Society clinical practice guideline. J Clin Endocrinol Metab 94(3):709–728

    Article  CAS  PubMed  Google Scholar 

  48. Kulke MH, Anthony LB, Bushnell DL et al (2010) NANETS treatment guidelines: well-differentiated neuroendocrine tumors of the stomach and pancreas. Pancreas 39(6):735–752

    Article  PubMed  PubMed Central  Google Scholar 

  49. Ekeblad S (2010) Islet cell tumours. Adv Exp Med Biol 654:771–789

    Article  PubMed  Google Scholar 

  50. O’Toole D, Salazar R Falconi M et al (2006) Rare functioning pancreatic endocrine tumors. Neuroendocrinology 84(3): 189–195

    Article  PubMed  Google Scholar 

  51. Kaltsas G, Androulakis II, de Herder WW, Grossman, AB (2010) Paraneoplastic syndromes secondary to neuroendocrine tumours. Endocr Relat Cancer 17(3):R173–R193

    Article  PubMed  Google Scholar 

  52. Levy-Bohbot N, Merle C, Goudet P et al (2004) Prevalence, characteristics and prognosis of MEN 1-associated glucagonomas, VIPomas, and somatostatinomas: study from the GTE (Groupe des Tumeurs Endocrines) registry. Gastroenterol Clin Biol 28(11): 1075–1081

    Article  PubMed  Google Scholar 

  53. Appetecchia M, Ferretti E, Carducci M et al (2006) Malignant glucagonoma. New options of treatment. J Exp Clin Cancer Res 25(1): 135–139

    CAS  PubMed  Google Scholar 

  54. Wermers RA, Fatourechi V, Kvols LK (1996) Clinical spectrum of hyperglucagonemia associated with malignant neuroendocrine tumors. Mayo Clin Proc 71(11): 1030–1038

    Article  CAS  PubMed  Google Scholar 

  55. Eldor R Glaser B, Fraenkel M et al (2011) Glucagonoma and the glucagonoma syndrome — cumulative experience with an elusive endocrine tumour. Clin Endocrinol (Oxford) 74(5):593–598

    Article  Google Scholar 

  56. Garbrecht N, Anlauf M, Schmitt A et al (2008) Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity. Endocr Relat Cancer 15(1):229–241

    Article  PubMed  Google Scholar 

  57. Hoffmann KM, Furukawa M, Jensen RT (2005) Duodenal neuroendocrine tumors: Classification, functional syndromes, diagnosis and medical treatment. Best Pract Res Clin Gastroenterol 19(5):675–697

    Article  CAS  PubMed  Google Scholar 

  58. Nesi G, Marcucci T, Rubio CA et al (2008) Somatostatinoma: clinico-pathological features of three cases and literature reviewed. J Gastroenterol Hepatol 23(4):521–526

    Article  CAS  PubMed  Google Scholar 

  59. Nikou GC, Toubanakis C, Nikolaou P et al (2005) VIPomas: an update in diagnosis and management in a series of 11 patients. Hepatogastroenterology 52(64): 1259–1265

    PubMed  Google Scholar 

  60. O’Dorisio T, Vinik A (1985) Pancreatic polypeptide and mixed peptide-producing tumors of the gastrointestinal tract. In: Cohen S, Soloway RD (eds) Contemporary issues in gastroenterology, pp 117–128. Churchill-Livingstone, New York

    Google Scholar 

  61. Greenberg GR, McCloy RF, Adrian TE et al (1978) Inhibition of pancreas and gallbladder by pancreatic polypeptide. Lancet 2(8103): 1280–1282

    Article  CAS  PubMed  Google Scholar 

  62. Larsson LI, Schwartz T, Lundqvist G et al (1976) Occurrence of human pancreatic polypeptide in pancreatic endocrine tumors. Possible implication in the watery diarrhea syndrome. Am J Pathol 85(3):675–684

    CAS  PubMed  Google Scholar 

  63. Garby L, Caron P, Claustrat F et al (2012) Clinical characteristics and outcome of acromegaly induced by ectopic secretion of growth hormone-releasing hormone (GHRH): a French nationwide series of 21 cases. J Clin Endocrinol Metab 97(6):2093–2104

    Article  CAS  PubMed  Google Scholar 

  64. Kamp K, Feelders RA, van Adrichem RC et al (2014) Parathyroid hormone-related peptide (PTHrP) secretion by gastroenteropancreatic neuroendocrine tumors (GEP-NETs): clinical features, diagnosis, management, and follow-up. J Clin Endocrinol Metab 99(9):3060–3069

    Article  CAS  PubMed  Google Scholar 

  65. Kanakis G, Kaltsas G, Granberg D et al (2012) Unusual complication of a pancreatic neuroendocrine tumor presenting with malignant hypercalcemia. J Clin Endocrinol Metab 97(4):E627–E631

    Article  CAS  PubMed  Google Scholar 

  66. Giannetta E, Gianfrilli D, Pozza, C et al (2016) Extrathyroidal calcitonin secreting tumors: pancreatic neuroendocrine tumors in patients with multinodular goiter: two case reports. Medicine (Baltimore) 95(3):e2419

    Article  PubMed  PubMed Central  Google Scholar 

  67. Piaditis G, Angellou A, Kontogeorgos G et al (2005) Ectopic bioactive luteinizing hormone secretion by a pancreatic endocrine tumor, manifested as luteinized granulosa-thecal cell tumor of the ovaries. J Clin Endocrinol Metab 90(4):2097–2103

    Article  CAS  PubMed  Google Scholar 

  68. Schneider R Waldmann J, Swaid Z et al (2011) Calcitonin-secreting pancreatic endocrine tumors: systematic analysis of a rare tumor entity. Pancreas 40(2):213–221

    Article  CAS  PubMed  Google Scholar 

  69. Brignardello E, Manti R, Papotti M et al (2004) Ectopic secretion of LH by an endocrine pancreatic tumor. J Endocrinol Invest 27(4):361–365

    Article  CAS  PubMed  Google Scholar 

  70. Ruddy MC, Atlas SA, Salerno FG (1982) Hypertension associated with a renin-secreting adenocarcinoma of the pancreas. N Engl J Med 307(16):993–997

    Article  CAS  PubMed  Google Scholar 

  71. Roberts RE, Zhao M, Whitelaw BC et al (2012) GLP-1 and glucagon secretion from a pancreatic neuroendocrine tumor causing diabetes and hyperinsulinemic hypoglycemia. J Clin Endocrinol Metab 97(9):3039–3045

    Article  CAS  PubMed  Google Scholar 

  72. Chung JO, Hong SI, Cho DH et al (2008) Hypoglycemia associated with the production of insulin-like growth factor II in a pancreatic islet cell tumor: a case report. Endocr J 55(3):607–612

    Article  PubMed  Google Scholar 

  73. Samyn I, Fontaine C, Van TF et al (2004) Paraneoplastic syndromes in cancer: Case 1. Polycythemia as a result of ectopic erythropoietin production in metastatic pancreatic carcinoid tumor. J Clin Oncol 22(11):2240–2242

    CAS  PubMed  Google Scholar 

  74. Rehfeld JF, Federspiel B, Bardram L (2013) A neuroendocrine tumor syndrome from cholecystokinin secretion. N Engl J Med 368(12): 1165–1166

    Article  CAS  PubMed  Google Scholar 

  75. Falconi M, Bartsch DK, Eriksson B et al (2012) ENETS Consensus guidelines for the management of patients with digestive neuroendocrine neoplasms of the digestive system: well-differentiated pancreatic non-functioning tumors. Neuroendocrinology 95(2): 120–134

    Article  CAS  PubMed  Google Scholar 

  76. Villaume K, Blanc M, Gouysse G et al (2010) VEGF secretion by neuroendocrine tumor cells is inhibited by octreotide and by inhibitors of the PI3K/AKT/mTOR pathway. Neuroendocrinology 91(3):268–278

    Article  CAS  PubMed  Google Scholar 

  77. Cidon EU (2017) New therapeutic approaches to metastatic gastroenteropancreatic neuroendocrine tumors: A glimpse into the future. World J Gastrointest Oncol 9(1):4–20

    Article  PubMed  PubMed Central  Google Scholar 

  78. 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): 1727–1733

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  79. Falconi M, Plockinger U, Kwekkeboom DJ et al (2006) Well-differentiated pancreatic nonfunctioning tumors/carcinoma. Neuroendocrinology 84(3): 196–211

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Endocrinology Unit, Regina Elena National Cancer Institute, Rome, Italy

    Marialuisa Appetecchia

Authors
  1. Marialuisa Appetecchia
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Rosa Lauretta
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Francesca Rota
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Massimo Carlini
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Marialuisa Appetecchia .

Editor information

Editors and Affiliations

  1. Department of Surgery, Division of General Surgery, S. Eugenio Hospital, Rome, Italy

    Massimo Carlini

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer-Verlag Italia

About this chapter

Cite this chapter

Appetecchia, M., Lauretta, R., Rota, F., Carlini, M. (2018). Sporadic Gastroenteropancreatic Neuroendocrine Tumors. In: Carlini, M. (eds) Abdominal Neuroendocrine Tumors. Updates in Surgery. Springer, Milano. https://doi.org/10.1007/978-88-470-3955-1_4

Download citation

  • DOI: https://doi.org/10.1007/978-88-470-3955-1_4

  • Publisher Name: Springer, Milano

  • Print ISBN: 978-88-470-3954-4

  • Online ISBN: 978-88-470-3955-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation