Seltene chirurgische Tumoren im biliopankreatoduodenalen Bereich

Gastrointestinale Stromatumoren, Choledochuszysten und pankreatische neuroendokrine Tumoren sind seltene Tumoren des biliopankreatoduodenalen Bereichs. Eine frühzeitige Diagnose ist entscheidend für die Prognose und das Überleben der betroffenen Patienten. Dieser Beitrag gibt einen Überblick über das diagnostische Vorgehen und die therapeutischen Optionen.

Kernaussagen

• Sowohl Patienten mit KIT- als auch mit PDGFRA-Mutation eines GIST sprechen auf Imatinib an. Dagegen gelten Patienten mit c-KIT/PDGFRA-Wildtyp-Status oder PDGFRA-D842V-Mutation als Imatinib-resistent.

• Resektable GIST sollten reseziert werden, lokal fortgeschrittene Tumoren neoadjuvant therapiert und im Anschluss reseziert und metastasierte Tumoren nur selten chirurgisch behandelt werden.

• Bei Patienten mit lokalisierter, nicht metastasierter GIST-Erkrankung, bei denen eine R0-Resektion unwahrscheinlich ist, sollte eine Resektion nach Vorbehandlung mit Imatinib erfolgen.

• Potenzielle Folgen von Choledochuszysten sind Strikturen, Steinbildung, Cholangitiden, Pankreatitiden, Rupturen, Entstehung einer sekundären biliären Zirrhose sowie von Malignomen. Ihre Therapie ist deshalb chirurgisch.

• Das Operationsziel bei Choledochuszysten ist heute, die Zyste in toto zu exzidieren und im Anschluss die Kontinuität durch eine biliodigestive Anastomose wiederherzustellen.

• PNET werden in funktionell aktive und funktionell inaktive Tumoren unterteilt. Die meisten der Tumoren sind funktionell inaktiv.

• Die chirurgische Resektion ist die einzige Möglichkeit auf kurative Entfernung des Tumors und um im Falle von funktionell aktiven PNET eine Symptomkontrolle zu erlangen.

• Die medikamentöse Erstlinientherapie bei PNET besteht aus Somatostatinanaloga.

Publication History

Article published online:
01 September 2022

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