First surgery for pancreatic neuroendocrine tumours in a patient with MEN1: enucleation versus disease-modifying...

Pancreatic neuroendocrine tumours (PNETs) are the second most common manifestation of MEN1, affecting up to 80% of patients. The secretion of peptide hormones by PNETs causes clinical syndromes requiring therapeutic intervention. Malignant progression of PNETs is a leading cause of mortality in patients with MEN1. The goal of surgery, when required, is to alleviate a biochemical syndrome or to treat established tumour(s) to reduce the risk of local progression or metastases against the background of preservation of pancreatic function. Determining the need and optimum timing for an operative intervention is complex and requires an approach individualized for each patient. When a clinically significant biochemical syndrome is confirmed, the time course to surgery is clear. In patients with a potentially malignant PNET, the decision as to when to intervene is more challenging. In all cases surgical treatment carries the potential for harm, of more than usual concern because many of the patients are young. In this study, we explain an approach to the surgical treatment of MEN1 patients with biochemical or radiological evidence of PNETs where other manifestations of the syndrome are either treated or controlled and the patient lacks comorbidity that would preclude pancreatic surgery. In each scenario we present, a normal serum gastrin will be assumed as the surgical approach to this usually duodenal manifestation of MEN1 is significantly different to the management of other PNETs.