From diagnosis to treatment: navigating the course for pancreatic neuroendocrine neoplasms

Pancreatic neuroendocrine neoplasms (pNENs) represent an increasingly significant, unique and complex subgroup of neuroendocrine diseases. Their heterogeneity is reflected in wide variations in biological behaviour, metastatic potential, functionality and aetiology. This review synthesizes the current understanding of pNENs, from diagnosis to treatment.

Recent advances in understanding of these neoplasms have led to significant changes in their classification, now distinguishing three grades of well-differentiated tumours from poorly differentiated neuroendocrine carcinomas. These neoplasms are rare and can occur sporadically or within the context of hereditary syndromes, however, due to advances in diagnostic modalities and ageing population their incidence worldwide is on the rise. They can present as functional neoplasms, secreting biologically active hormones and inducing hormonal syndromes, or as non-functional neoplasms. Surgery remains the primary curative-intent approach for localized and locally advanced tumours, while systemic treatment is often the only option for patients with advanced disease. Multifaceted nature of pNENs demands a multidisciplinary approach that incorporates personalized diagnostic and therapeutic strategies. While clinical guidelines provide an essential framework, they must remain adaptable to accommodate individual patient circumstances and evolving evidence.

This review addresses existing gaps, unresolved controversies and areas of inconsistency in diagnostic workup and management of pNENs. It underscores the need for continued investigation to refine our understanding and improve patient outcomes.