PANCREATIC NEUROENDOCRINE TUMOURS - A RARE PANCREATIC TUMOUR

Pancreatic neuroendocrine tumours are rare tumours that can either present with syndromes from excess hormonal production or from mass effect - from the primary or metastases. They vary widely in clinical course, with the main determinants of outcome being TNM staging and pathological grade. The available treatment options depend largely on the grade of the tumour - somatostatin analogues, targeted agents, chemotherapy and PRRT for IG1 and G2 PNETs, and chemotherapy as the mainstay of treatment for high grade NET/NECs. The paucity of randomised evidence in the treatment of this disease argues for ongoing research to understand the molecular genetics underlying PNETs, to develop possible future treatment options, as well as optimising use of existing ones.