Functioning pancreatic neuroendocrine neoplasms: Diagnosis and current treatment

Abstract Pancreatic functioning neuroendocrine neoplasms (PanNENs) are rare and characterized by variable and heterogeneous clinical manifestations, comprising both symptoms and signs of tumor growth and systemic manifestations of hormonal hypersecretion. The diagnosis is challenging due to their rarity, neoplasm localization and diverse secretory profile. A subset of these neoplasms may develop in the context of inherited syndromes and harbor specific genetic alterations requiring genetic counseling of other family members. These particular features make the management of patients with a functioning PanNEN syndrome requiring a multidisciplinary approach. Surgical resection of local disease, antisecretory medical treatment, locoregional treatment and systemic treatments are employed on an individualized basis. The treatment landscape of advanced NENs progressing after systemic treatment or after exhibiting intolerance to sequential lines of treatment is rapidly evolving supporting the introduction of additional therapies with promising results.