Diagnostik und Therapie beifunktionell aktiven pankreatischen neuroendokrinen Tumoren

Pancreatic neuroendocrine tumours (PNET) are rare entities withan annual incidence of < 100,000. About1 – 2 % of pancreaticneoplasias are neuroendocrine tumours. About one third of thesetumours secrete biologically active substances that lead to developmentof specific clinical syndromes. PNET may occur sporadically or inassociation with hereditary syndromes, such as multiple endocrineneoplasia type 1 (MEN1). Among the functional PNET, insulinomasand gastrinomas are the most common entities. In contrast, vasoactiveintetinale peptide (VIP)-secreting tumours, glucagonomas, serotonin-secretingcarcinoid tumors, and tumours with secretion of ectopic hormones,such as calcitonin, are extremely rare. Once diagnosis has beenestablished on the basis of clinical and laboratory findings, localizationof the source of pathologic hormone secretion is warranted. Imagingmethods frequently used for localization of PNET comprise anatomicalimaging modalities, computed tomography, and magnetic resonanceimaging, endoscopic ultrasound, selective arterial catheterizationwith hepatic venous sampling, DTPA-octreotid scintigraphy and DOTA-D-Phe(1)-Tyr(3)-octreotidpositron emission tomography. Therapy is based on the specific tumourentity 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.