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Neuroendokrine Neoplasien des distalen Jejunums und Ileums

Neuroendocrine neoplasms of the distal jejunum and ileum

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Zusammenfassung

Neuroendokrine Neoplasien (NEN) des distalen Jejunums und Ileums leiten sich von den serotoninproduzierenden enterochromaffinen Zellen (EC-Zellen) ab. Aufgrund ihrer niedrigen proliferativen Aktivität und ihres infiltrativen Wachstums werden sie klinisch häufig in einem fortgeschrittenen metastasierten Tumorstadium diagnostiziert. Die Biologie dieser Tumoren unterscheidet sich grundlegend von NEN anderer Lokalisation. Für eine Vereinheitlichung und Verbesserung der Diagnose und Therapie wurden die Leitlinien für die Diagnostik und das klinische Management von jejunoilealen NEN sowie für das Management von Patienten mit hepatisch und fernmetastasierten NEN durch die European Neuroendocrine Tumor Society (ENETS) im Jahre 2012 überarbeitet. Die folgende Übersicht konzentriert sich auf die für die Pathologie besonders relevanten Sachverhalte und deren Einbettung in das klinische Management.

Abstract

Neuroendocrine neoplasms (NEN) of the distal jejunum and ileum derive from serotonin-producing enterochromaffin (EC) cells. Due to their low proliferation rate and their infiltrative growth, they are often discovered at an advanced disease stage when metastasis has already occurred. The biology of these tumours is different from other NEN of the digestive tract. In order to standardise and improve diagnosis and therapy, the guidelines for the diagnosis and clinical management of jejuno–ileal NEN as well as for the management of patients with liver and other distant metastases from NEN were revised by the European Neuroendocrine Tumour Society (ENETS) in 2012. This review focuses on aspects relevant for surgical pathology.

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Literatur

  1. Pape UF, Perren A, Niederle B, Gross D 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 95:135–156

    Article  CAS  PubMed  Google Scholar 

  2. Pavel M, Baudin E, Couvelard A, Krenning E 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 

  3. Salazar R, Wiedenmann B, Rindi G et al (2012) ENETS Consensus Guidelines for the Management of Patients with Digestive Neuroendocrine Tumors: an update. Neuroendocrinology 95:71–73

    Article  CAS  PubMed  Google Scholar 

  4. Rindi G, Arnold R, Bosman FT et al (2010) Nomenclature and classification of Neuroendocrine neoplasms of the digestive system. In: Bosman FT, Carneiro F, Hruban RH, Theise ND (Hrsg) WHO classification of tumors of the digestive system. IARC, Lyon, S 13–14

  5. Capella C, Arnold R, Klimstra DS et al (2010) Neuroendocrine neoplasms of the small intestine. In: Bosman FT, Carneiro F, Hruban RH, Theise ND (Hrsg) WHO classification of tumors of the digestive system. IARC, Lyon, S 102–107

  6. Sobin LH, Gospodarowicz MK, Wittekind C (Hrsg) (2009) TNM classification of malignant tumours, 7. Aufl. Wiley

  7. Klöppel G, Couvelard A, Perren A et al (2009) ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: towards a standardized approach to the diagnosis of gastroenteropancreatic neuroendocrine tumors and their prognostic stratification. Neuroendocrinology 90:162–166

    Article  PubMed  Google Scholar 

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

    Article  Google Scholar 

  9. O’Toole D, Grossman A, Gross D et al (2009) ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: biochemical markers. Neuroendocrinology 90:194–202

    Article  Google Scholar 

  10. Garcia-Carbonero R, Capdevila J, Crespo-Herrero G et al (2010) Incidence, patterns of care and prognostic factors for outcome of gastroenteropancreatic neuroendocrine tumors (GEP-NETs): results from the National Cancer Registry of Spain (RGETNE). Ann Oncol 21:1794–803

    Article  CAS  PubMed  Google Scholar 

  11. Scarpa A, Mantovani W, Capelli P et al (2010) Pancreatic endocrine tumors: improved TNM staging and histopathological grading permit a clinically efficient prognostic stratification of patients. Mod Pathol 23:824–833

    Article  CAS  PubMed  Google Scholar 

  12. Jann H, Roll S, Couvelard A, Hentic O et al (2011) Neuroendocrine tumors of midgut and hindgut origin: tumor-node-metastasis classification determines clinical outcome. Cancer 117:3332–3341

    Article  PubMed  Google Scholar 

  13. Rindi G, Klöppel G, Alhman H et al (2006) TNM staging of foregut (neuro)endocrine tumors: a consensus proposal including a grading system. Virchows Arch 449:395–401

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  14. Rindi G, Klöppel G, Couvelard A et al (2007) European Neuroendocrine Tumor Society. TNM staging of midgut and hindgut (neuro) endocrine tumors: a consensus proposal including a grading system. Virchows Arch 451:757–762

    Article  CAS  PubMed  Google Scholar 

  15. Kwekkeboom DJ, Krenning EP, Scheidhauer K et al (2009) ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: somatostatin receptor imaging with (111)In-pentetreotide. Neuroendocrinology 90:184–189

    Article  CAS  PubMed  Google Scholar 

  16. Adams S, Baum RP, Hertel A, Schumm-Dräger PM et al (1998) Metabolic(PET) and receptor (SPET) imaging of well- and less well-differentiated tumours: comparison with the expression of the Ki-67 antigen. Nucl Med Commun 19:641–647

    Article  CAS  PubMed  Google Scholar 

  17. Rinke A, Müller HH, Schade-Brittinger C, Klose KJ 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 27:4656–4663

    Article  CAS  PubMed  Google Scholar 

  18. 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 26:3063–3072

    Article  PubMed  Google Scholar 

  19. Burke AP, Thomas RM, Elsayed AM et al (1997) Carcinoids of the jejunum and ileum: an immunohistochemical and clinicopathologic study of 167 cases. Cancer 79:1086–1093

    Article  CAS  PubMed  Google Scholar 

  20. 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 95:98–119

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  21. Fischer T, Doll C, Jacobs S et al (2008) Reassessment of sst2 somatostatin receptor expression in human normal and neoplastic tissues using the novel rabbit monoclonal antibody UMB-1. J Clin Endocrinol Metab 93:4519–4524

    Article  CAS  PubMed  Google Scholar 

  22. Miederer M, Seidl S, Buck A et al (2009) Correlation of immunohistopathological expression of somatostatin receptor 2 with standardised uptake values in 68Ga-DOTATOC PET/CT. Eur J Nucl Med Mol Imaging 36:48–52

    Article  CAS  PubMed  Google Scholar 

  23. Körner M, Waser B, Schonbrunn A et al (2012) Somatostatin receptor subtype 2A immunohistochemistry using a new monoclonal antibody selects tumors suitable for in vivo somatostatin receptor targeting. Am J Surg Pathol 36:242–252

    Article  PubMed Central  PubMed  Google Scholar 

  24. Kaemmerer D, Peter L, Lupp A et al (2011) Molecular imaging with 68Ga-SSTR PET/CT and correlation to immunohistochemistry of somatostatin receptors in neuroendocrine tumours. Eur J Nucl Med Mol Imaging 38:1659–1668

    Article  CAS  PubMed  Google Scholar 

  25. Begum N, Maasberg S, Plöckinger U et al (2012) Neuroendocrine Tumours of the GI Tract – Data from the German NET Registry. Zentralbl Chir (Epub ahead of print)

  26. Schmitt AM, Riniker F, Anlauf M et al (2008) Islet 1 (Isl1) expression is a reliable marker for pancreatic endocrine tumors and their metastases. Am J Surg Pathol 32:420–425

    Article  PubMed  Google Scholar 

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Einhaltung ethischer Richtlinien

Interessenkonflikt. M. Anlauf, B. Sipos, I. Boeck, S. E. Baldus, S. Heikaus, M. Krausch, W. T. Knoefel, N. Begum, P. Goretzki, M. Schott, C. J. Auernhammer, B. Cremer, A. Rinke, S. Ezziddin, C. Fottner, G. Pöpperl, H. Lahner, D. Hörsch, H. E. Gabbert, P. Komminoth, A. Perren, G. Klöppel, B. Wiedenmann, M. Pavel und U. Pape geben an, dass kein Interessenkonflikt besteht.

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Anlauf, M., Sipos, B., Boeck, I. et al. Neuroendokrine Neoplasien des distalen Jejunums und Ileums. Pathologe 35, 283–294 (2014). https://doi.org/10.1007/s00292-013-1888-5

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