Zusammenfassung
Neuroendokrine Neoplasien (NEN) des Verdauungstrakts nehmen ihren Ausgangspunkt vom diffusen neuroendokrinen System, können ubiquitär im gesamten gastroenteropankreatischen (GEP) System auftreten und besitzen die Fähigkeit zur Hormonproduktion. Die Grundlage für die Diagnostik und Therapie bilden die standardisierte morphologische Klassifizierung dieser Tumoren nach der WHO-Klassifikation von 2010, das proliferationsbasierte Grading und die lokalisationsbezogene TNM-Klassifikation. Auch wenn die Prognose von GEP-NEN meist als günstig bezeichnet wird, zeigt sie eine weite prognostische Spannweite, die eine differenzierte Betrachtung der einzelnen Tumorentitäten bezüglich ihrer prognostischen Einschätzung nötig macht. Das Beschwerdebild, das GEP-NEN verursachen, ist meist unspezifisch. In erster Linie ist es abhängig vom primären Ursprungsort des Tumors und der Funktionalität. Nur selten liegt eine spezifische Beschwerdesymptomatik vor, sodass die Tumoren meist erst in einem fortgeschrittenen Tumorstadium diagnostiziert werden. Neben der biochemischen Diagnostik spezieller Hormonsyndrome ist Chromogranin A der unspezifische Marker mit der größten Relevanz für die Verlaufsbeurteilung. Neben den klassischen endoskopischen und radiologischen Verfahren kommt der funktionellen nuklearmedizinischen Diagnostik eine besondere Rolle zu.
Abstract
Neuroendocrine neoplasms of the digestive system represent a rare and heterogeneous group of malignancies with various clinical presentations and prognoses. The WHO classification for the year 2000 was updated in 2010 to take the histopathology and tumor biology of these tumors into account. Together with proliferation-based grading and the recently established staging system using the ENETS TNM classification, it forms the basis for the further diagnostic and therapeutic approach. Clinical presentation depends mainly on the primary site of the tumor and its functionality. Characteristic symptoms are seen only rarely, this being the reason these tumors are usually detected at an advanced stage. Approximately 30% of GEP-NEN are hormonally active and can cause a specific clinical syndrome. In addition to these specific hormones, chromogranin A is considered the most accurate general marker for the biochemical follow-up of these patients. In addition to commonly used radiological and endoscopic imaging modalities, somatostatin receptor-based functional imaging using either octreotide scintigraphy or novel PET-based techniques with specific isotopes such as Ga68-DOTA-octreotate play a crucial role in the detection of the primary tumor as well as in the evaluation of tumor extent and the selection of patients for receptor-based radionuclide therapy.
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Interessenkonflikt
Der korrespondierende Autor weist auf folgende Beziehungen hin: C. Fottner hat Vortragshonorare und Kongressreisekostenunterstützungen von Novartis und Ipsen erhalten. M.M. Weber hat Vortragshonorare und Kongressreisekostenunterstützungen von Novartis, Ipsen, Pfizer sowie Forschungsförderungen von Novartis und Ipsen erhalten.
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Fottner, C., Weber, M. Neuroendokrine Neoplasien des Gastrointestinaltrakts. Internist 53, 131–144 (2012). https://doi.org/10.1007/s00108-011-2916-2
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DOI: https://doi.org/10.1007/s00108-011-2916-2