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Gastroenteropankreatische neuroendokrine Neoplasien (GEP-NEN)

Leitliniengerechte medikamentöse Therapie

Gastroenteropancreatic neuroendocrine neoplasms (GEP-NEN)

Guideline-compliant medicinal therapy

  • Schwerpunkt: Neuroendokrine Tumoren
  • Published:
Der Gastroenterologe Aims and scope

Zusammenfassung

Dieser Beitrag gibt einen Überblick über die leitliniengerechte Therapie gastroenteropankreatischer neuroendokriner Neoplasien (GEP-NEN). Vorgestellt werden zunächst die verschiedenen Einsatzbereiche der Biotherapie. Als Biotherapie wird eine medikamentöse Behandlung mit Hormonanaloga wie Somatostatinanaloga (SSA) oder Interferon alfa bezeichnet. Der Stellenwert der systemischen Chemotherapie wird bei extrapankreatischen Primärtumoren abhängig von deren Lokalisation sehr unterschiedlich bewertet. Bei pankreatischen NEN wird die Chemotherapie empfohlen, wenn unter Biotherapie ein Progress aufgetreten ist. Im Weiteren wird die aktuelle Daten- und Empfehlungslage hinsichtlich des Einsatzes der modernen molekular-zielgerichteten („targeted therapies“) beleuchtet. Hier sind mittlerweile zwei Wirkstoffe (Everolimus und Sunitinib) zur Behandlung pankreatischer NEN zugelassen. Bei anderen Primärtumorlokalisationen ist der Nutzen der neuen molekular-zielgerichteten Substanzen noch nicht vollständig geklärt. Abschließend werden kurz die Möglichkeiten der antiresorptiven Therapie bei Knochenmetastasen erörtert.

Abstract

This article gives an overview of the guideline-compliant therapy of gastroenteropancreatic neuroendocrine neoplasms (GEP-NEN). Firstly, the various fields of application of biologics therapy are presented. A biologic therapy involves a medicinal treatment with homone analogs, such as somatostatin analogs (SAS) and interferon alpha. The importance of systemic chemotherapy for extrapancreatic primary tumors is assessed very differently depending of the location. For pancreatic NEN chemotherapy is recommended when progress occurs under biologics therapy. Furthermore, the current situation on data and recommendations with respect to the use of modern molecular targeted therapy is described. In this field two substances are now approved for the treatment of pancreatic neuroendocrine tumors, everolimus and sunitinib. For other primary tumor localizations the benefit of the new molecular targeted substances has not yet been fully clarified. Finally, the possibilities of antiresorptive therapy for bone metastases are briefly described.

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Literatur

  1. Pape UF, Perren A, Niederle B et al (2012) ENETS Consensus Guidelines for the management of patients with neuroendocrine neoplasms from the jejuno-ileum and the appendix including goblet cell carcinomas. Neuroendocrinology 95(2):135–156

    Article  CAS  PubMed  Google Scholar 

  2. Oberg K, Knigge U, Kwekkeboom D et al (2012) Neuroendocrine gastro-entero-pancreatic tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 23(Suppl 7):vii124–vii130

    PubMed  Google Scholar 

  3. Boudreaux JP, Klimstra DS, Hassan MM et al (2010) The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: well-differentiated neuroendocrine tumors of the Jejunum, Ileum, Appendix, and Cecum. Pancreas 39(6):753–766

    Article  PubMed  Google Scholar 

  4. Kunz PL, Reidy-Lagunes D, Anthony LB et al (2013) Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas 42(4):557–577

    Article  PubMed Central  PubMed  Google Scholar 

  5. 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(2):157–176

    Article  CAS  PubMed  Google Scholar 

  6. Rinke A, Müller HH, Schade-Brittinger C et al (2009) Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID Study Group. J Clin Oncol 27(28):4656–4663

    Article  CAS  PubMed  Google Scholar 

  7. Caplin ME, Pavel M, Ćwikła JB et al (2014) Lanreotide in metastatic enteropancreatic neuroendocrine tumors. N Engl J Med 371(3):224–233

    Article  PubMed  Google Scholar 

  8. IPSEN Pharma GmbH (2015) Fachinformation Somatuline Autogel 60 mg/90 mg/120 mg

  9. Delle Fave G, Kwekkeboom DJ, Van Cutsem E et al (2012) ENETS Consensus Guidelines for the management of patients with gastroduodenal neoplasms. Neuroendocrinology 95(2):74–87

    Article  CAS  PubMed  Google Scholar 

  10. 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 Central  PubMed  Google Scholar 

  11. Ramage JK, Goretzki PE, Manfredi R et al (2008) Consensus guidelines for the management of patients with digestive neuroendocrine tumours: well-differentiated colon and rectum tumour/carcinoma. Neuroendocrinology 87(1):31–39

    Article  CAS  PubMed  Google Scholar 

  12. 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  PubMed Central  CAS  PubMed  Google Scholar 

  13. 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 

  14. Eriksson B, Klöppel 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 

  15. Caplin M, Sundin A, Nillson O et al (2012) ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: colorectal neuroendocrine neoplasms. Neuroendocrinology 95(2):88–97

    Article  CAS  PubMed  Google Scholar 

  16. Strosberg JR, Coppola D, Klimstra DS et al (2010) The NANETS consensus guidelines for the diagnosis and management of poorly differentiated (high-grade) extrapulmonary neuroendocrine carcinomas. Pancreas 39(6):799–800

    Article  PubMed Central  PubMed  Google Scholar 

  17. Kulke MH, Shah MH, Benson AB 3rd et al (2015) Neuroendocrine tumors, version 1.2015. J Natl Compr Canc Netw 13(1):78–108

    CAS  PubMed  Google Scholar 

  18. Walter T, Bruneton D, Cassier PA et al (2010) Evaluation of the combination 5-fluorouracil, dacarbazine, and epirubicin in patients with advanced well-differentiated neuroendocrine tumors. Clin Colorectal Cancer 9(4):248–254

    Article  CAS  PubMed  Google Scholar 

  19. Strosberg JR, Fine RL, Choi J et al (2011) First-line chemotherapy with capecitabine and temozolomide in patients with metastatic pancreatic endocrine carcinomas. Cancer 117(2):268–275

    Article  CAS  PubMed  Google Scholar 

  20. Sorbye H, Welin S, Langer SW et al (2013) Predictive and prognostic factors for treatment and survival in 305 patients with advanced gastrointestinal neuroendocrine carcinoma (WHO G3): the NORDIC NEC study. Ann Oncol 24(1):152–160

    Article  CAS  PubMed  Google Scholar 

  21. Yao JC, Shah MH, Ito T et al (2011) Everolimus for advanced pancreatic neuroendocrine tumors. N Engl J Med 364(6):514–523

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  22. Dahan REL, Raoul JL et al (2011) Sunitinib malate for the treatment of pancreatic neuroendocrine tumors. N Engl J Med 364(6):501–513

    Article  PubMed  Google Scholar 

  23. Kos-Kudla B, O’Toole D, Falconi M et al (2010) ENETS consensus guidelines for the management of bone and lung metastases from neuroendocrine tumors. Neuroendocrinology 91(4):341–350

    Article  CAS  PubMed  Google Scholar 

  24. Ross EM, Roberts WC (1985) The carcinoid syndrome: comparison of 21 necropsy subjects with carcinoid heart disease to 15 necropsy subjects without carcinoid heart disease. Am J Med 79(3):339–354

    Article  CAS  PubMed  Google Scholar 

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Correspondence to S. Noe.

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S. Noe und B. Neu geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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R. Schmid, München

B.Wiedenmann, Berlin

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Noe, S., Neu, B. Gastroenteropankreatische neuroendokrine Neoplasien (GEP-NEN). Gastroenterologe 10, 418–421 (2015). https://doi.org/10.1007/s11377-015-0013-0

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  • DOI: https://doi.org/10.1007/s11377-015-0013-0

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