ABSTRACT
Neuroendocrine neoplasms of the digestive system represent a rare and heterogeneous group of malignancies with various clinical presentations and prognoses. The WHO classification for the year 2000 was updated in 2010 to take the histopathology and tumor biology of these tumors into account. Together with proliferation-based grading and the recently established staging system using the ENETS TNM classification, it forms the basis for the further diagnostic and therapeutic approach. Clinical presentation depends mainly on the primary site of the tumor and its functionality. Characteristic symptoms are seen only rarely, this being the reason these tumors are usually detected at an advanced stage. Approximately 30% of GEP-NEN are hormonally active and can cause a specific clinical syndrome. In addition to these specific hormones, chromogranin A is considered the most accurate general marker for the biochemical follow-up of these patients. In
addition to commonly used radiological and endoscopic imaging modalities, somatostatin receptor-based functional imaging using either octreotide scintigraphy or novel PET-based techniques with specific isotopes such as Ga68-DOTA-octreotate play a crucial role in the detection of the primary tumor as well as in the evaluation of tumor extent and the selection of patients for receptor-based radionuclide therapy.