Zufallsbefund NEN Appendix

Zusammenfassung

Neuroendokrine Neoplasien (NEN) der Appendix stellen mit 80% die häufigste Neoplasie dieser Lokalisation dar. In den meisten Fällen werden diese Tumoren als Zufallsbefund nach stattgehabter Appendektomie bei V. a. Appendizitis diagnostiziert. Hierbei handelt es sich meist um hoch differenzierte neuroendokrine Tumoren. Aufgrund ihrer häufigen Lage im Bereich der Appendixspitze sind die NEN der Appendix meist nicht ursächlich für die Appendizitis-typischen Beschwerden.

Die meisten Patienten (80–90%) sind durch die bereits durchgeführte Appendektomie ausreichend onkologisch therapiert. Sollten sich jedoch Risikofaktoren wie eine Tumorgröße > 2 cm, basisnahe Lage, Angioinvasion, Perforation oder Infiltration von Nachbarorganen, Proliferationsindex von > 2% oder eine Infiltration des Mesoappendix von mehr als 3 mm im endgültigen histopathologischen Befund ergeben, so wird eine Nachresektion als onkologische Hemikolektomie rechts empfohlen.

Aufgrund des meist frühen Tumorstadiums bei Diagnosestellung ohne nachgewiesene Lymphknotenmetastasierung weisen Patienten mit einer NEN der Appendix exzellente 5-Jahres-Überlebensraten über alle Tumorstadien hinweg von 70–85% auf.

Abstract

With an incidence of 80%, neuroendocrine neoplasia (NEN) is the most common neoplasia of the appendix. In most cases, these tumours are diagnosed as an incidental finding after appendectomy with suspected appendicitis. They are usually highly differentiated neuroendocrine tumours. Due to their frequent location on the apex of the appendix, the NENs of the appendix are usually not the cause of the symptoms typical for appendicitis.

Most patients (80–90%) receive adequate oncological treatment by laparoscopic or open appendectomy that has already been performed. However, if there are risk factors such as tumour size > 2 cm, location close to the base, angioinvasion, perforation or infiltration of neighbouring organs, proliferation index of > 2% or infiltration of the mesoappendix by more than 3 mm in the final histopathological finding, subsequent resection as an oncological right sided hemicolectomy is recommended .

Due to their mostly early tumour stage at diagnosis without proven lymph node metastasis, patients with NEN of the appendix have an excellent 5-year survival rate of 70–85% across all tumour stages.

Schlüsselwörter

Abdominalchirurgie - NEN Appendix - Appendektomie - Lymphknotenmetastase - Neoplasie

Keywords

acute appendicitis - appendectomy - cancer - hemicolectomy - appendiceal neuroendocrine tumor

Publication History

Received: 11 January 2022

Accepted after revision: 11 March 2022

Article published online:
15 June 2022

• Literatur

• 1 Pawa N, Clift AK, Osmani H. et al. Surgical Management of Patients with Neuroendocrine Neoplasms of the Appendix: Appendectomy or More. Neuroendocrinology 2018; 106: 242-251
• 2 Niederle MB, Hackl M, Kaserer K. et al. Gastroenteropancreatic neuroendocrine tumours: The current incidence and staging based on the WHO and European Neuroendocrine Tumour Society classification: An analysis based on prospectively collected parameters. Endocr Relat Cancer 2010; 17: 909-918
• 3 Ellis L, Shale MJ, Coleman MP. Carcinoid tumors of the gastrointestinal tract: Trends in incidence in England since 1971. Am J Gastroenterol 2010; 105: 2563-2569
• 4 Pape UF, Niederle B, Costa F. et al. ENETS consensus guidelines for neuroendocrine neoplasms of the appendix (excluding goblet cell carcinomas). Neuroendocrinology 2016; 103: 144-152
• 5 Heetfeld M, Chougnet CN, Olsen IH. et al. Characteristics and treatment of patients with G3 gastroenteropancreatic neuroendocrine neoplasms. Endocr Relat Cancer 2015; 22: 657-664
• 6 Rindi G, Klöppel G, Alhman H. et al. TNM staging of foregut (neuro)endocrine tumors: A consensus proposal including a grading system. Virchows Arch 2006; 449: 395-401
• 7 Vélayoudom-Céphise FL, Duvillard P, Foucan L. et al. Are G3 ENETS neuroendocrine neoplasms heterogeneous?. Endocr Relat Cancer 2013; 20: 649-657
• 8 Farchione A, Rufini V, Brizi MG. et al. Evaluation of the Added Value of Diffusion-Weighted Imaging to Conventional Magnetic Resonance Imaging in Pancreatic Neuroendocrine Tumors and Comparison With 68 Ga-DOTANOC Positron Emission Tomography/Computed Tomography. Pancreas 2016; 45: 345-354
• 9 Nagtegaal ID, Odze RD, Klimstra D. et al. The 2019 WHO classification of tumours of the digestive system. Histopathology 2020; 76: 182-188
• 10 Nagtegaal ID, Odze RD, Klimstra D. et al. The 2019 WHO classification of tumours of the digestive system. Histopathology 2020; 76: 182-188
• 11 Begum N, Maasberg S, Plöckinger U. et al. Neuroendokrine Tumoren des Verdauungstrakts – Daten des deutschen NET-Registers. Zentralbl Chir 2014; 139: 276-283
• 12 Maasberg S, Pape UF, Fottner C. et al. Neuroendocrine Neoplasia within the German NET Registry. Z Gastroenterol 2018; 56: 1237-1246