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Neuroendokrine Tumoren des Magens

Risikostratifizierung und Therapie

Neuroendocrine tumors of the stomach

Risk stratification and therapy

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Zusammenfassung

Diagnose und Therapie der neuroendokrinen Tumoren (NET) des Magens basieren auf ihrer sicheren Klassifikation und Risikostratifizierung. Da zudem die NET des Magens in den letzten 35 Jahren deutlich an Häufigkeit zugenommen haben und die Mehrzahl dieser Tumoren heutzutage endoskopisch in einem frühen Stadium entdeckt wird, sind sie zu einer Herausforderung für den Pathologen geworden. Zusätzlich zur prognostischen WHO- und TNM-Klassifizierung werden 4 biologisch relevante Typen der gastralen NET unterschieden: Gut differenzierte NET (G1) des Magens vom Typ 1 und Typ 2 kleiner als 2 cm und Typ 3 kleiner als 1 cm haben, sofern sie weder die Muscularis propria infiltrieren noch angioinvasiv sind, eine gute Prognose und können endoskopisch entfernt werden. Gut differenzierte NET (G1 bis G2) über 2 cm Größe und/oder mit Infiltration der Muskelwand und/oder Gefäßinvasion sowie schlecht differenzierte neuroendokrine Karzinome (Typ 4) haben eine ungünstige oder schlechte Prognose und müssen aggressiv behandelt werden. Für die Größenbestimmung, die Infiltrationstiefe und den Nachweis von Lymphknotenmetastasen können endosonographische Befunde herangezogen werden. Bei eindeutiger Diagnose und adäquater Therapie haben die meisten Patienten mit NET des Magens heutzutage eine gute Prognose.

Abstract

The diagnosis and therapy of neuroendocrine tumors (NETs) of the stomach are based on their exact classification and risk stratification. Since the incidence of gastric NETs has risen sharply over the last 35 years and most tumors are detected endoscopically at an early stage, they have come to represent a challenge for the pathologist. Gastric NETs are classified according to the WHO and TNM classifications and additionally separated into four biologically distinct types: Well differentiated type 1 and 2 gastric NETs (G1) smaller than 2 cm, and type 3 smaller than 1 cm that do not infiltrate the muscularis propria or show angioinvasion have a good prognosis and can be removed endoscopically. Well differentiated type 1 and 2 gastric NETs (G1-G2) larger than 2 cm or type 3 with a diameter above 1 cm or with infiltration of the muscular wall and/or angioinvasion and poorly differentiated (type 4) neuroendocrine carcinomas carry a poor prognosis and need to be treated aggressively. Endosonography is the method of choice for determining the size, depth of infiltration and presence of lymph node metastases. With exact diagnosis and adequate treatment, the majority of patients with gastric NETs have a favorable prognosis.

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Abbreviations

EC:

Enterochromaffin

ECL:

„Enterochromaffin-like“

MEN1:

Multiple endokrine Neoplasie Typ 1

NEK:

Neuroendokrine Karzinome

NET:

Neuroendokrine Tumoren

SEER:

„Surveillance Epidemiology and End Results“

VMAT2:

Vesikulärer Monoamintransporter 2

WHO:

„World Health Organisation“

ZES:

Zollinger-Ellison-Syndrom

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Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehung hin: Konsulent für Pankreas- und endokrine Tumoren am Institut für Pathologie der Technischen Universität München.

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Authors and Affiliations

  1. Institut für Pathologie, Klinikum rechts der Isar,Technische Universität München, Ismaningerstr. 22, 81675, München, Deutschland

    G. Klöppel

  2. Klinik für Gastroenterologie und GI Onkologie, Vivantes Klinikum Am Urban, Berlin, Deutschland

    H. Scherübl

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  1. G. Klöppel
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  2. H. Scherübl
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Correspondence to G. Klöppel.

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Klöppel, G., Scherübl , H. Neuroendokrine Tumoren des Magens. Pathologe 31, 182–187 (2010). https://doi.org/10.1007/s00292-009-1268-3

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