From diagnosis to treatment

Introduction: Pancreatic neuroendocrine neoplasms (pNENs) represent an increasinglysignificant, unique and complex subgroup of neuroendocrine diseases. Theirheterogeneity 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.Discussion: Recent advances in understanding of these neoplasms have led tosignificant changes in their classification, now distinguishing three grades ofwell-differentiated tumours from poorly differentiated neuroendocrine carcinomas.These neoplasms are rare and can occur sporadically or within the context of hereditarysyndromes, however, due to advances in diagnostic modalities and ageing populationtheir incidence worldwide is on the rise. They can present as functional neoplasms,secreting biologically active hormones and inducing hormonal syndromes, or asnon-functional neoplasms. Surgery remains the primary curative-intent approach forlocalized and locally advanced tumours, while systemic treatment is often the onlyoption for patients with advanced disease. Multifaceted nature of pNENs demands amultidisciplinary approach that incorporates personalized diagnostic and therapeuticstrategies. While clinical guidelines provide an essential framework, they must remainadaptable to accommodate individual patient circumstances and evolving evidence.Conclusions: This review addresses existing gaps, unresolved controversies and areas ofinconsistency in diagnostic workup and management of pNENs. It underscores theneed for continued investigation to refine our understanding and improve patientoutcomes