Pancreatic neuroendocrine neoplasms are a rare and complex group of neoplastic lesions that develop from pancreatic islet cells. Their incidence has dramatically increased during the last two decades. Due to its complex nature and pathophysiological behaviour, surgical management continues to evolve. Surgery remains the cornerstone of treatment for most non-functional and functional pancreatic neuroendocrine tumours, while lymphadenectomy remains a controversial subject. Different techniques, such as pancreas-preserving and minimally invasive approaches, continue to evolve and offer the same overall outcomes as open surgery. This comprehensive review describes in detail the current and most up-to-date classification and staging of pancreatic neuroendocrine tumours, explores the rationale for non-surgical and surgical management, and focuses on surgical treatment and more specifically, on minimally invasive approaches.
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