Neuroendokrine Tumoren des Dünndarms nehmen zu: frühe Tumoren und deren Management

Zusammenfassung

Neuroendokrine Tumoren (NET) des Dünndarms sind in den letzten 35 Jahren 3- bis 5-fach häufiger geworden. Zeitgleich hat sich die Prognose der Patienten wesentlich verbessert. NET des Duodenums werden häufig „en passant” im Rahmen einer Gastroduodenoskopie diagnostiziert, die unter anderer Fragestellung erfolgt. Dadurch werden die Tumoren in zunehmendem Maße in früheren Stadien festgestellt. Sind sie gut differenziert, nicht funktionell und von bis zu 10 mm Größe, können sie endoskopisch entfernt werden. Eine Operationsindikation besteht dagegen bei gut differenzierten, duodenalen NET von > 20 mm Größe, bei schlecht differenzierten neuroendokrinen Karzinomen und bei sporadischen Gastrinomen. Bei gut differenzierten NET mit einer Größe zwischen 10 und 20 mm als auch bei MEN1-Gastrinomen muss das therapeutische Vorgehen interdisziplinär entschieden werden. Für die Bestimmung der Größe und Infiltrationstiefe der duodenalen NET und zum Nachweis von Lymphknotenmetastasen ist die Endosonografie die Methode der Wahl. Für NET des Ileums ist die Chirurgie die Therapie der Wahl. Bei metastasierten ilealen NET mit hormoneller Symptomatik koupieren stabile Somatostatinanaloga effektiv das Karzinoidsyndrom und führen zudem zu einer signifikanten Verlängerung des (progressionsfreien) Überlebens der Patienten. Therapieempfehlungen für NET-Patienten müssen Tumorbiologie, Tumortyp, Tumorlokalisation, Tumorstadium und die individuelle Situation berücksichtigen.

Abstract

Neuroendocrine tumors (NETs) of the small bowels are on the rise: in the US they have increased by 300 – 500 % in the last 35 years. At the same time their prognosis has been much improved. Most NETs of the duodenum are nowadays detected ”incidentally” and therefore recognized at an early stage. Duodenal NETs that are well differentiated, not larger than 10 mm in greatest dimension and limited to the mucosa/submucosa can be endoscopically resected. In NETs with a size between 10 mm and 20 mm the therapeutic strategy has to be individually discussed. Endoscopic ultrasound is the method of choice to determine tumor size and depth of infiltration. Surgery is indicated for well differentiated duodenal NETs greater than 20 mm, for localized sporadic gastrinomas and for localized poorly differentiated NE cancers. Surgery is also indicated for localized/regional ileal NETs. Advanced ileal NETs with a carcinoid syndrome are treated with stable somatostatin analogs. This treatment also significantly improves the (progression-free) survival in patients with metastatic NETs of the ileum. For optimal NET management tumor biology, type, localization and stage of the neoplasm as well as the individual situation of the patient have to be taken into account.

Schlüsselwörter

Karzinoid - Gastrinom - Duodenum - Ileum - Karzinom - Krebs

Key words

carcinoid - gastrinoma - duodenum - ileum - carcinoma - cancer

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