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Gastroenteropankreatische neuroendokrine Tumoren

Zielgerichtete Diagnostik und Therapie

Gastroenteropancreatic neuroendocrine tumors

Targeted diagnostics and therapy

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Zusammenfassung

Gastroenteropankreatische neuroendokrine Tumoren (GEP-NET) sind selten, aber in ihrer Inzidenz weltweit steigend. GEP-NET sind mit 70 % die häufigsten NET. Außerhalb des Magen-Darm-Traktes finden sich NET in der Lunge (25 %), selten in der Haut, im Urogenitaltrakt und in den Ovarien.

GEP-NET zeigen abhängig von ihrer Lokalisation, Größe, Differenzierung (G1–G3) und der möglichen Freisetzung von Hormonen unterschiedlichste Symptome. Die Krankheitsverläufe der Patienten sind hoch variabel. Patienten mit gut differenzierten G1-NET haben langsame, teilweise auch benigne Verläufe über Jahrzehnte. Daneben zeigen schlecht differenzierte Tumoren (G3) aggressive, kurze Verläufe mit einem frühen Metastasierungsmuster. Fast die Hälfte der Patienten mit NET-Tumoren haben bereits Lymphknoten- oder Lebermetastasen bei Erstdiagnose. Trotzdem stieg die 5- und 10-Jahres-Überlebensrate (JÜL) der Patienten mit NET-Tumoren an (5-JÜL 80 %; 10-JÜL 60 %), was auch durch eine immer bessere interdisziplinären Behandlung (Chirurgie, Endokrinologie, Onkologie, Nuklearmedizin, Radiologe) bedingt ist.

Abstract

Gastroenteropancreatic neuroendocrine tumors (GEP-NET) are rare but an increase in incidence has been recognized worldwide. Approximately 70 % of NETs are localized in the gastrointestinal tract and in the pancreas, other locations are in the lungs (25 %) and rarely in the skin, urogenital tract and ovaries. Depending on the size, localization, grading (G1–G3) and production of hormones, the symptoms of patients can greatly vary. Outcome and survival of patients depend on the biological behavior and grading of the NET. Patients with a well differentiated G1 grade NET have a slow, sometimes also benign course over decades even with metastases in contrast to patients with G3 grade NETs. These tumors exhibit an aggressive behavior and patient survival is short. Liver and lymph node metastases are common (about 50 %) in GEP-NETs even at the initial diagnosis. The 5-year and 10-year survival of patients with GEP-NETs is increasing (currently approximately 80 % and 60 %, respectively), especially when a multidisciplinary team (e.g. surgery, endocrinology, oncology, nuclear medicine and gastroenterology) manages GEP-NET patients.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. K. Holzer gibt an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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  1. Klinik für Allgemein- und Viszeralchirurgie, Universitätsklinikum Frankfurt am Main, Theodor Stern Kai 7, 60590, Frankfurt am Main, Deutschland

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Correspondence to K. Holzer FEBS.

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Holzer, K. Gastroenteropankreatische neuroendokrine Tumoren. Chirurg 85, 731–744 (2014). https://doi.org/10.1007/s00104-013-2679-5

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