Diagnostik und Therapie bei funktionell aktiven pankreatischen neuroendokrinen Tumoren

Zusammenfassung

Pankreatische neuroendokrine Tumore (PNET), sind seltene Tumorentitäten mit einer jährlichen Inzidenz von < 1 pro 100 000. Bei etwa 1 – 2 % der vom Pankreas ausgehenden Neoplasien handelt es sich um neuroendokrine Tumoren. Etwa ein Drittel der PNET sezernieren biologisch aktive Substanzen, die zu der Entwicklung spezifischer klinischer Syndrome führen. PNET können sporadisch auftreten oder mit hereditären Syndromen wie der Multiplen Endokrinen Neoplasie Typ 1 (MEN1) assoziiert sein. Unter den endokrinologisch aktiven PNET sind Insulinome und Gastrinome die häufigsten Entitäten. Hingegen sind Vasoaktives Intestinales Polypeptid (VIP)-sezernierende Tumore, Glukagonome, Serotonin-bildende Karzinoide oder Tumoren mit Sekretion von ektopen Hormonen wie Kalzitonin sehr selten. Nach Sicherung der Diagnose aufgrund klinischer und biochemischer Befunde gilt es, den zugrunde liegenden Tumor nachzuweisen. Häufig zur Lokalisation von PNET eingesetzte Methoden umfassen Computertomographie, Magnetresonaztomographie, endoskopische Sonographie, selektive arterielle Katheterisierung mit venöser Blutentnahme, DTPA-Octreotid-Szintigraphie und DOTA-D-Phe(1)-Tyr(3)-Octreotid-Positronenemissionstomographie. Die Therapie richtet sich nach der spezifischen Tumorentität und dem Ausmaß der Erkrankung. In der Mehrzahl der Patienten, auch wenn eine maligne Erkrankung zugrunde liegt, ist ein chirurgisches Vorgehen, eventuell kombiniert mit einer medikamentösen Therapie, indiziert.

Abstract

Pancreatic neuroendocrine tumours (PNET) are rare entities with an annual incidence of < 100,000. About 1 – 2 % of pancreatic neoplasias are neuroendocrine tumours. About one third of these tumours secrete biologically active substances that lead to development of specific clinical syndromes. PNET may occur sporadically or in association with hereditary syndromes, such as multiple endocrine neoplasia type 1 (MEN1). Among the functional PNET, insulinomas and gastrinomas are the most common entities. In contrast, vasoactive intetinale peptide (VIP)-secreting tumours, glucagonomas, serotonin-secreting carcinoid tumors, and tumours with secretion of ectopic hormones, such as calcitonin, are extremely rare. Once diagnosis has been established on the basis of clinical and laboratory findings, localization of the source of pathologic hormone secretion is warranted. Imaging methods frequently used for localization of PNET comprise anatomical imaging modalities, computed tomography, and magnetic resonance imaging, endoscopic ultrasound, selective arterial catheterization with hepatic venous sampling, DTPA-octreotid scintigraphy and DOTA-D-Phe(1)-Tyr(3)-octreotid positron emission tomography. Therapy is based on the specific tumour entity and the extent of the disease. In the majority of patients, even in the case of malignant disease, a surgical approach is warranted, eventually combined with a medical treatment.

Insulinom - Gastrinom - VIPom - Glukagonom - neuroendokriner Tumor

insulinoma - gastrinoma - VIPoma - glucagonoma - neuroendocrine tumour

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