Background: Neuroendocrine tumours of the pancreas (PNETs) represent 1–2% of all pancreatic tumours. The terms ‘islet cell tumours’ and ‘carcinoids’ of the pancreas should be avoided. The aim of this review is to offer an overview of the history and diagnosis of PNETs followed by a discussion of the available treatment options. Methods: A search on PubMed using the keywords ‘neuroendocrine’, ‘pancreas’ and ‘carcinoid’ was performed to identify relevant literature over the last 30 years. Results: The introduction of a revised classification of neuroendocrine tumours by the World Health Organisation (WHO) in 2000 significantly changed our understanding of and approach to the management of these tumours. Advances in laboratory and radiological techniques have also led to an increased detection of PNETs. Surgery remains the only treatment that offers a chance of cure with increasing number of non-surgical options serving as beneficial adjuncts. The better understanding of the behaviours of PNETs together with improvements in tumour localisation has resulted in a more aggressive management strategy with a concomitant improvement in symptom palliation and a prolongation of survival. Conclusion: Due to their complex nature and the wide range of therapeutic options, the involvement of specialists from all necessary disciplines in a multidisciplinary team setting is vital to provide optimal treatment of this disease.
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