Most of these controversies are unique to the management of patients with ZES including those related to control of the acid hypersecretion; controversies related to the diagnosis of ZES; each of the three MEN1/ZES controversies, and controversies related to the non-surgical primary NET ablation for treatment, whereas controversies related to the selection sequence of anti-tumor medical treatments in patients with advanced disease is shared by patients with all malignant pNETs and in many aspects with the management of patients with any advanced NET. This evidence concerns the gene MEN1 and neoplasm.