Although several options of drug therapy exist for advanced neuroendocrine cancer of pancreatic origin, few data based on randomized studies are available. The results of the most recent randomized placebo-controlled studies are obscured by the assignment of patients from the placebo branch to the treatment branch. Application of the study results is further hampered by small patient groups, heterogeneity and retrospective set-up of study materials and, in older studies, inaccurate response assessment. We describe drug therapies that can be utilized to affect the clinical course of locally advanced or metastatic neuroendocrine cancer of pancreatic origin. Supportive therapy of hormonally active tumors has been restricted to somatostatin analogue treatment.