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Neuroendokrine Neoplasien des Magens

Klassifikation und therapeutische Strategien

Neuroendocrine neoplasms of the stomach

Classification and treatment options

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Zusammenfassung

Neuroendokrine Neoplasien des Magens (g-NEN) gehören zu den häufigsten neuroendokrinen Neoplasien des Verdauungstrakts. Aufgrund des zunehmenden Fortschritts der bildgebenden Qualität in der Endoskopie werden g‑NEN darüber hinaus häufiger diagnostiziert als früher. Neben der prognostischen Klassifikation der Weltgesundheitsorganisation (WHO) bzw. der TNM-Klassifikation gibt es zusätzlich die Differenzierung in 3 biologisch relevante Subtypen: Der Typ 1 repräsentiert den häufigsten Subtyp (70–80 %) und ist pathophysiologisch mit einer chronisch-atrophischen Gastritis (autoimmun sowie durch Helicobacter pylori bedingt) assoziiert. Die g‑NEN Typ 2 (5 %) treten im Rahmen der multiplen endokrinen Neoplasien Typ 1 bzw. des Zollinger-Ellison-Syndroms (MEN1/ZES) auf. Der Typ 3 (10–15 %) tritt isoliert auf und weist keine pathophysiologische Assoziation auf. Bei den Typen 1 und 2 liegt eine Hypergastrinämie vor. Sie sind gewöhnlich von geringer Größe, multipel auftretend und mit guter Prognose vergesellschaftet. Dagegen liegt beim Typ 3 in der Regel keine vermehrte Gastrinsekretion vor. Stattdessen handelt es sich um große, singulär auftretende, bösartige Tumoren (G3). Entsprechend des biologischen Verhaltens (Typ 1–3) unterscheidet sich die Therapie von der endoskopischen Abtragung und Surveillance bis hin zur chirurgischen Resektion mit Chemotherapie. Eine frühe g‑NEN-Typ-1-Diagnose geht bei entsprechender endoskopischer Therapie bzw. Surveillance in der Regel mit einer ausgezeichneten Prognose einher.

Abstract

Gastric neuroendocrine neoplasms (g-NEN) are one of the most frequent neuroendocrine neoplasia of the digestive system. Due to technical progress of endoscopy with high-definition imaging modalities, NEN are also diagnosed more frequently than in former times. Besides the prognostic World Health Organization (WHO) or TNM classification system, there is also the differentiation into three biological subgroups: type 1 represents the most frequent subtype (70–80%) and is associated with chronic atrophic gastritis (autoimmune or Helicobacter pylori mediated). Type 2 (5% of the cases) is associated with multiple endocrine neoplasia type 1 and Zollinger–Ellison syndrome (MEN1/ZES). Type 3 (10–15%) usually shows no association. Type 1 and 2 are related to hypergastrinemia. They are small in size, occur at multiple locations within the stomach and are of benign behavior. In contrast, type 3 is not related to an increased gastrin secretion, and this subtype is usually represented by a single, large gastric tumor that is of malignant nature. According to their biological behavior (type 1–3), the respective treatment varies from endoscopic resection to surgery with chemotherapy. Early detection of type 1 NEN with adequate endoscopic treatment and surveillance is associated with a very good prognosis.

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Correspondence to A. Kandulski.

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H. Azam-Zangeneh, M. Müller-Schilling und A. Kandulski geben an, dass kein Interessenkonflikt besteht.

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M. Ebert, Mannheim

M. Müller-Schilling, Regensburg

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Azam-Zangeneh, H., Müller-Schilling, M. & Kandulski, A. Neuroendokrine Neoplasien des Magens. Gastroenterologe 13, 126–129 (2018). https://doi.org/10.1007/s11377-018-0231-3

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