Operationsindikationen bei neuroendokrinen Neoplasien des Pankreas

Zusammenfassung

Neuroendokrine Neoplasien des Pankreas (pNEN) haben aufgrund ihrer ansteigenden Inzidenz und Prävalenz eine zunehmende klinische Bedeutung. Ihr Erscheinungsbild variiert sehr stark, da manche dieser Tumoren Hormone sezernieren. Ihnen gemeinsam ist die Tendenz zur frühen Metastasierung. Dennoch ist die Tumorgröße, neben der Graduierung, ein wichtiger prognostischer Marker. Anhand einer systematischen Literaturrecherche und der Formulierung von konkreten Schlüsselfragen nach dem PICO-Schema sind Therapieempfehlungen formuliert worden. Durch die Befragung im Delphi-Verfahren unter den Mitgliedern der „Arbeitsgruppe Pankreas“ und der „Arbeitsgruppe Indikationsqualität“ der Deutschen Gesellschaft für Allgemein- und Viszeralchirurgie erstellten wir sodann evidenzbasierte Therapieempfehlungen für Patienten mit einem pNEN. Die chirurgische Resektion dieser Tumoren stellt das einzige potenzielle kurative Verfahren dar. Sporadische wie auch hereditäre pNEN > 2 cm stellen eine Operationsindikation dar, unabhängig von ihrer funktionellen Aktivität. Bei kleineren Tumoren kann u. U. auch ein abwartendes Verhalten indiziert sein. Anhand der aktuellen Daten ist eine Empfehlung hierzu aber nicht grundsätzlich zu geben und auch pNEN von 1 – 2 cm Größe sind daher eine relative Operationsindikation. Hereditäre pNEN stellen eine interdisziplinäre Herausforderung dar und diese Patienten sollten gemeinsam an einem Zentrum für neuroendokrine Tumoren betreut werden. Patienten mit Fernmetastasen sind nicht grundsätzlich als palliativ anzusehen, da neben der Metastasenchirurgie auch Debulking-Operationen indiziert sein können.

Abstract

Neuroendocrine neoplasms of the pancreas (pNEN) have an increasing incidence and prevalence. Thus, this entity is of increasing clinical significance. Patients with pNEN become clinically apparent due to different and unspecific symptoms. Some tumours secrete hormones and peptides and become clinically symptomatic. In general, these tumours can metastasise early and even small tumours lead to distant metastases. Nonetheless, primary tumour size and grading are important prognostic factors. On the basis of a systematic literature research and the formulation of key issues according to the PICO system, therapeutic concepts were established. These concepts were evaluated with a Delphi process among the members of the pancreas research group and the indications for surgery group of the German Society of General and Visceral Surgery. Thus this article gives an overview of the surgical treatment modalities and indications for the treatment of pNEN. Surgery is still the gold standard in treatment and the only potential chance of cure. Surgery is indicated for sporadic as well as hereditary pNEN > 2 cm independent of the functional activity. A so called “wait and see” strategy might be indicated in smaller pNEN; however, there is little evidence for this approach. In this respect, pNEN of 1 – 2 cm represent a surgical indication. The treatment of hereditary pNEN is challenging and should be interdisciplinary. Even in the case of distant metastases, a curative approach might be feasible and multimodal treatment is indicated.

Kernaussagen

• Die chirurgische Resektion von pNEN stellt das einzige kurative Verfahren dar.

• pNEN > 2 cm stellen eine OP-Indikation dar.

• pNEN < 2 cm können beobachtet oder operiert werden.

• Die hereditären und funktionellen pNEN sind von den nicht funktionellen sporadischen pNEN zu unterscheiden; ihre Therapie unterscheidet sich.

• Ein extrahepatisches Debulking kann indiziert sein, um eine zielgerichtete Therapie der hepatischen Metastasen zu ermöglichen.

• Die Metastasenchirurgie kann beim gut differenzierten pNEN nach interdisziplinärer Diskussion indiziert sein.

• Beim schlecht differenzierten pNEN ist eine Resektion nur dann indiziert, wenn Tumorfreiheit erzielt werden kann.

Schlüsselwörter

neuroendokrine Neoplasie - Pankreas - Operationsindikation - Pankreaschirurgie - Tumorgröße

Key words

neuroendocrine neoplasm - pancreas - operative indication - pancreatic surgery - tumour size

Publikationsverlauf

Artikel online veröffentlicht:
29. Juni 2020

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