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Management of Hormonal Symptoms

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Neuroendocrine Tumours
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Abstract

Gastroenteropancreatic neuroendocrine neoplasms (GEP NEN) oversecreting hormones and/or biogenic amines are the causes of specific hormonal syndromes, such as carcinoid syndrome in serotonin-secreting tumors, Zollinger-Ellison syndrome in gastrinoma, hypoglycemic syndrome in insulinoma, Verner-Morrison syndrome (VIPoma), and others.

Management of hormonal symptoms, which are often life-threatening, requires interdisciplinary approach and implementation of multiple therapeutic methods. This chapter presents the management guidelines, using surgical and endoscopic methods, and pharmacotherapy, including biotherapy, isotope treatments, chemotherapy, and targeted molecular therapy applied in the symptomatic treatment of GEP-NEN clinical syndromes.

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References

  1. Oberg K, Knigge U, Kwekkeboom D, Perren A (2012) Neuroendocrine gastro-entero-pancreatic tumors: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol 23:124–130

    Google Scholar 

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

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  3. Kos-Kudła B, Blicharz-Dorniak J, Handkiewicz-Junak D et al (2013) Diagnostic and therapeutic guidelines for gastro-entero-pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours). Endokrynol Pol 64(6):418–443

    PubMed  Google Scholar 

  4. Le Roith D (1999) Tumor-induced hypoglycemia. N Engl J Med 341:757–758

    PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  6. de Herder WW, van Schaik E, Kwekkeboom D, Feelders RA (2011) New therapeutic options for metastatic malignant insulinomas. Clin Endocrinol (Oxf) 75(3):277–284

    Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  8. Ramage JK, Ahmed A, Ardill J et al (2012) Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs). Gut 61:6–32

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  9. Kos-Kudła B, Hubalewska-Dydejczyk A, Kuśnierz K et al (2013) Pancreatic neuroendocrine neoplasms – management guidelines (recommended by the Polish Network of Neuroendocrine Tumours). Endokrynol Pol 64(6):459–479

    PubMed  Google Scholar 

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

    PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  12. Vannella L, Sbrozzi-Vanni A, Lahner E et al (2011) Development of type I gastric carcinoid in patients with chronic atrophic gastritis. Aliment Pharmacol Ther 33:1361–1369

    CAS  PubMed  Google Scholar 

  13. Rydzewska G, Cichocki A, Cwikła JB et al (2013) Gastroduodenal neuroendocrine neoplasms including gastrinoma – management guidelines (recommended by the Polish Network of Neuroendocrine Tumours). Endokrynol Pol 64(6):444–458

    PubMed  Google Scholar 

  14. Sahani DV, Banaffini PA, Del Castillo CF, Blake MA (2013) Gastroenteropancreatic neuroendocrine tumors: role of imaging in diagnosis and management. Radiology 266(1):38–61

    Article  PubMed  Google Scholar 

  15. 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 95:120–134

    Article  CAS  PubMed  Google Scholar 

  16. Milan SA, Yeo CJ (2012) Neuroendocrine tumors of the pancreas. Curr Opin Oncol 24:46–55

    PubMed  Google Scholar 

  17. Akyildiz HY, Mitchell J, Milas M et al (2010) Laparoscopic radiofrequency thermal ablation of neuroendocrine hepatic metastases: long-term follow-up. Surgery 148:1288–1293

    Article  PubMed  Google Scholar 

  18. Pavel M, Baudin E, Couvelard A et al (2012) ENETS Consensus Guidelines for the management of patients with liver and other distant metastases from neuroendocrine neoplasms of foregut, midgut, hindgut, and unknown primary. Neuroendocrinology 95:157–176

    Article  CAS  PubMed  Google Scholar 

  19. Kianmanesh R, Ruszniewski P, Rindi G et al (2010) ENETS consensus guidelines for the management of peritoneal carcinomatosis from neuroendocrine tumors. Neuroendocrinology 91:333–340

    Article  CAS  PubMed  Google Scholar 

  20. 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 103:169–174

    PubMed  Google Scholar 

  21. Bester L, Meteling B, Pocock N et al (2013) Radioembolisation with yttrium-90 microspheres: an effective treatment modality for unresectable liver metastases. J Med Imaging Radiat Oncol 57(1):72–80

    PubMed  Google Scholar 

  22. Jürgensen C, Schuppan D, Neser F et al (2006) EUS-guided alcohol ablation of an insulinoma. Gastrointest Endosc 63:1059–1062

    PubMed  Google Scholar 

  23. Deprez PH, Claessens A, Borbath I et al (2008) Successful endoscopic ultrasound guided ethanol ablation of a sporadic insulinoma. Acta Gastroenterol Belg 71:333–337

    PubMed  Google Scholar 

  24. Muscatiello N, Salcuni A, Macarini L et al (2008) Treatment of a pancreatic endocrine tumor by ethanol injection guided by endoscopic ultrasound. Endoscopy 40:E258–E259

    Article  PubMed  Google Scholar 

  25. Rosiek V, Kunikowska J, Kos-Kudła B (2012) A non-functioning pancreatic neuroendocrine tumour: a case report. Endokrynol Pol 63(1):59–64

    PubMed  Google Scholar 

  26. Rosiek V, Kos-Kudła B (2010) Guidelines for the management of pancreatic neuroendocrine tumors. Gastroenterol Pract 2(6):28–34

    Google Scholar 

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

    PubMed  Google Scholar 

  28. Tomassetti P, Migliori M, Corinaldesi R et al (2000) Treatment of gastroenteropancreatic neuroendocrine tumours with octreotide LAR. Aliment Pharmacol Ther 14:557–560

    CAS  PubMed  Google Scholar 

  29. Vilar E, Salazar R, Pérez-Garcia J, Cortes J, Öberg K, Tabernero J (2007) Chemotherapy and role of the proliferation marker Ki-67 in digestive neuroendocrine tumors. Endocr Relat Cancer 14:221–232

    CAS  PubMed  Google Scholar 

  30. Moertel CG, Lefkopoulo M, Lipsitz S et al (1992) Streptozocin-doxorubicin, streptozocin-fluorouracil or chlorozotocin in the treatment of advanced islet-cell carcinoma. N Engl J Med 326:519–523

    CAS  PubMed  Google Scholar 

  31. Raymond E, Dahan L, Raoul JL et al (2011) Sunitinib malate for the treatment of pancreatic neuroendocrine tumors. N Engl J Med 364:501–513

    CAS  PubMed  Google Scholar 

  32. Yao JC, Shah MH, Ito T et al (2011) Everolimus for advanced pancreatic neuroendocrine tumors. N Engl J Med 364:514–523

    PubMed Central  CAS  PubMed  Google Scholar 

  33. Kulke MH, Bergsland EK, Yao JC (2009) Glycemic control in patients with insulinoma treated with everolimus. N Engl J Med 360:195–197

    CAS  PubMed  Google Scholar 

  34. Bourcier ME, Sherrod A, DiGuardo M et al (2009) Successful control of intractable hypoglycemia using rapamycin in an 86-year-old man with a pancreatic insulin secreting islet cell tumor and metastases. J Clin Endocrinol Metab 94:3157–3162

    CAS  PubMed  Google Scholar 

  35. Wiedenmann B, Pavel M, Kos-Kudla B (2011) From targets to treatments: a review of molecular targets in pancreatic neuroendocrine tumors. Neuroendocrinology 94:177–190

    Article  CAS  PubMed  Google Scholar 

  36. Kwekkeboom D, Kam B, van Essen M et al (2010) Somatostatin receptor-based imaging and therapy of gastroenteropancreatic neuroendocrine tumors. Endocr Relat Cancer 17:R53–R73

    CAS  PubMed  Google Scholar 

  37. Krenning EP, Kwekkeboom DJ, Valkema R et al (2004) Peptide receptor radionuclide therapy. Ann N Y Acad Sci 1014:234–245

    CAS  PubMed  Google Scholar 

  38. Paprottka P, Hoffmann R, Haug A et al (2011) Radioembolization of symptomatic, unresectable neuroendocrine hepatic metastases using Yttrium-90 microspheres. Cardiovasc Intervent Radiol 35(2):334–342

    PubMed  Google Scholar 

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Correspondence to Beata Kos-Kudla .

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Kos-Kudla, B. (2015). Management of Hormonal Symptoms. In: Yalcin, S., Öberg, K. (eds) Neuroendocrine Tumours. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45215-8_28

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  • DOI: https://doi.org/10.1007/978-3-662-45215-8_28

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-45214-1

  • Online ISBN: 978-3-662-45215-8

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