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Neuroendokrine Tumoren des Duodenums und Pankreas

Chirurgische Strategie

Neuroendocrine tumors of the duodenum and pancreas

Surgical strategy

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Zusammenfassung

Die Inzidenz neuroendokriner Tumoren (NET) ist in den letzten Jahrzehnten weltweit um das 3- bis 5-Fache angestiegen. Eine wesentliche Ursache liegt im umfassenden Einsatz bildgebender Verfahren und der Endoskopie begründet. Das führt dazu, dass ein Großteil duodenaler und pankreatischer NET in einem früheren Stadium diagnostiziert wird, was letztlich auch zu einer Prognoseverbesserung geführt hat. Nur ca. 20% der Patienten mit NET weisen spezifische, hormonell assoziierbare Symptome auf. In der Diagnostik spielen neben der Bestimmung von Chromogranin A im Serum und 5-Hydroxyindolessigsäure im 24-h-Sammelurin die Computertomographie, die Endosonographie und die Endoskopie eine zentrale Rolle. Mehr als 95% der diagnostizierten NET sind sporadische Tumoren. Hier sollte eine Resektion angestrebt werden. Gutartige NET des Duodenums bis zu 1 cm Größe lassen sich endoskopisch entfernen. Die Exzision größerer Tumoren sollte chirurgisch erfolgen. Bei hereditären NET sowohl des Duodenums als auch des Pankreas sind interdisziplinäre Behandlungspfade unabdingbar. Aber auch hier scheinen die Patienten von einer Resektion zu profitieren. Im fortgeschrittenen Tumorstadium sollte eine Debulking-Operation in Betracht gezogen werden, wenn mehr als 90% der Tumormassen entfernt werden können. Bei Patienten mit ausgedehnter hepatischer Metastasierung und resektablem Primärtumor kann eine Lebertransplantation hilfreich sein. Adjuvante oder neoadjuvante Therapieregime sind wenig standardisiert. Neueste Therapieregime mit Everolimus oder Sunitinib in fortgeschrittenen Tumorstadien zeigen vielversprechende Ergebnisse. Durch Gabe von Somatostatinanaloga bzw. Protonenpumpenhemmern kann eine adäquate Symptomreduzierung erreicht werden.

Abstract

The incidence of neuroendocrine tumors (NET) has increased worldwide by 3–5 times over the last decades. This is mainly based on the broad use of imaging modalities such as computed tomography (CT) and endoscopic approaches. As a consequence many duodenal and pancreatic tumors are detected in an early stage resulting in an improved prognosis of these patients. Besides the measurement of serum chromogranin A and 5-hydroxy indolic acid measured in 24 h urine collection, CT, endosonographic ultrasound (EUS) and endoscopy are important diagnostic tools. About 20% of all patients with pancreatic and duodenal NETs are diagnosed because of specific symptoms. More than 95% of diagnosed NETs are sporadic tumors. Whenever possible these patients should be treated by resection. Benign neuroendocrine duodenal tumors up to 1 cm in size can be removed endoscopically. The endoscopic resection of larger tumors should be performed surgically. The therapy of hereditary NETs of the duodenum and the pancreas should be decided after interdisciplinary discussion. However, even these patients seem to benefit from resection. In case of metastatic disease debulking surgery should be considered if more than 90% of the tumor mass can be resected. In patients with extensive liver metastases but resectable primary NET, liver transplantation is a reasonable option. There is no consensus about adjuvant or neoadjuvant treatment of duodenal or pancreatic NETs. The therapy with everolimus or sunitinib in advanced tumor stages has shown promising results. The administration of somatostatin analogues or antacids is appropriate for symptom reduction.

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  1. Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Ruprecht-Karls-Universität Heidelberg, Im Neuenheimer Feld 110, 69120, Heidelberg, Deutschland

    L. Fischer, A. Mehrabi &  M.W. Büchler

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  1. L. Fischer
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Fischer, L., Mehrabi, A. & Büchler, M. Neuroendokrine Tumoren des Duodenums und Pankreas. Chirurg 82, 583–590 (2011). https://doi.org/10.1007/s00104-011-2069-9

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