Neuroendokrine Tumoren des Gastrointestinaltraktes

Zusammenfassung

Zwei Drittel aller neuroendokrinen Tumoren (NET) sind im gastroentero-pankreatischen System lokalisiert. Je nach Lokalisation zeigen sich Unterschiede in Differenzierung, klinischer Symptomatik und Prognose. Die Symptome sind meistens durch lokale Tumormasseneffekte oder tumorassoziierte Fibrose bedingt. Ein „Karzinoidsyndrom” mit Flush und Diarrhoe tritt bei < 10 % der Patienten auf. Diagnostisch sind initial die Bestimmung von Chromogranin A (Serum) oder der 5-Hydroxyindolessigsäure (24h-Sammelurin) angezeigt. Bildgebend steht die Somatostatinrezeptorszintigraphie im Vordergrund, kann aber durch die CT/MRT zur genaueren topographischen Abgrenzung ergänzt werden. Neuere endoskopische Verfahren wie die Kapselendoskopie werden in der Diagnostik von NET des Dünndarms momentan evaluiert. In der Behandlung stellt die chirurgische Resektion nach wie vor das einzig kurative Verfahren dar. Allerdings ist diese nur in etwa 20 % der Fälle möglich, abhängig von der Lokalisation und Ausdehnung des Primarius. Bei hepatischer Beteiligung sollten lokal ablative Verfahren erwogen werden. In der konservativen Therapie sind Somatostatinanaloga der Goldstandard bei vergleichbarem therapeutischem Potential von Interferonen. Traditionelle zytotoxische Chemotherapie ist nur bei schlecht differenzierten, therapierefraktären NET indiziert. Neue Agenzien mit Angriff an unterschiedlichen intra- und interzellulären Signalwegen werden momentan geprüft. Eine symptombezogene supportive Therapie ist sinnvoll.

Summary

Two thirds of all neuroendocrine tumors (NET) are located in the gastroentero-pancreatic system. Depending on its localisation, each tumor presents a different histological pattern as well as different clinical symptoms and prognosis. Symptoms are usually due to the mass effects of the local tumor or tumor-related fibrosis. The classical „carcinoid-syndrome” with flush and diarrhea is seen in fewer than 10 %. Tests like chromogranin A (serum) or 5-hydroxyindolacetic acid (24h urine-collection) are indicators for the initial diagnosis of NET. Somatostatin-receptor scintigraphy is the most valuable imaging modality. In addition CT/MRI can be used for further topographical definition. Endoscopic techniques like the use of capsule endoscopy are being evaluated for the diagnosis of small intestinal NETs. The only curative treatment of NET is still complete surgical resection. However, it can only be done in 20 %, depending on localization and local extension of the primary tumor. If the liver is involved local ablation techniques should be considered. The gold standard for medical treatment is the use of somatostatin analogs, although interferons show a comparable therapeutic potential. Traditional cytotoxic agents should only be used for poorly differentiated tumors refractory to other forms of treatment. New compounds that target different pathways at the intra- and intercellular level are under investigation. Supportive therapy should be considered for the control of symptoms.

Schlüsselwörter

Karzinoid - Neuroendokrine Tumoren - Somatostatinanaloga - ablative Therapie - Rezeptorgesteuerte Radiopeptidtherapie - Somatostatinrezeptorszintigraphie

Key words

carcinoids - neuroendocrine tumors - somatostatin analogs - ablation treatment - receptor-targeted radiopeptide treatment - somatostatin receptor scintigraphy

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