Pancreatic neuroendocrine tumours (PanNETs) are rare types of neoplasms, whose incidence and prevalence are increasing. In general, PanNETs represent <3% of all pancreatic tumours. Most PanNETs are sporadic. Risk factors could include diabetes, smoking and chronic pancreatitis. Patients who develop PanNETs in the context of a hereditary syndrome are expected to be associated with a more indolent course.
Most of PanNETs are non-functioning. Among functioning PanNETs, hormone secretion may drive treatment strategy. In this E-Learning module, the author provides basic principles of grading and staging of PanNETs. The author highlights that proliferation index and morphology are the cornerstone of tumour classification which impacts both treatment and prognosis.
In the section on localised disease, the Module covers surgical treatment, adjuvant treatment, risk stratification and post-surgical follow-up. The author underlines that surgery is the only curative treatment for localised disease.
In the section on management of advanced stage disease, the author indicates how to select the most adequate treatment: liver-directed therapies, somatostatin analogues, targeted therapies, chemotherapy and peptide receptor radionuclide therapy (PRRT).
Surgery for metastatic disease (if resectable) does have a role; liver directed therapies are used for patients with unresectable liver disease if liver predominant or functional symptoms are present; systemic treatment includes somatostatin analogues, targeted therapies, chemotherapy and PRRT. However, most adequate treatment sequencing is unknown and is a current challenge.
The author underlines the importance of discussing each patient’s case within expert multidisciplinary teams as the recommended strategy for an adequate treatment planning at the time of the presentation. The selection of systemic treatment relies on tumour proliferation rate, Ki-67 and disease burden.
The author concludes this comprehensive module on PanNETs. by providing an outlook on management in terms of new targeted therapies, elaborating challenges for treatment sequences and assessment perspective of the role of immunotherapy.
