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Systemic Treatment Selection for Patients with Advanced Pancreatic Neuroendocrine Tumours (PanNETs)
Vera G Megdanova-Chipeva1,2,3, Angela Lamarca1,4, Alison Backen1,4
1Department of Medical Oncology, The Christie NHS Foundation Trust, Manchester M204BX, UK.
Abstract:
Pancreatic neuroendocrine tumours (PanNETs) are rare diseases and a good example of how research is not only feasible, but also of crucial importance in the scenario of rare tumours. Many clinical trials have been performed over the past two decades expanding therapeutic options for patients with advanced PanNETs. Adequate management relies on optimal selection of treatment, which may be challenging for clinicians due to the fact that multiple options of therapy are currently available. A number of therapies already exist, which are supported by data from phase III studies, including somatostatin analogues and targeted therapies (sunitinib and everolimus). In addition, chemotherapy remains an option, with temozolomide and capecitabine being one of the most popular doublets to use. Peptide receptor radionuclide therapy was successfully implemented in patients with well-differentiated gastro-entero-pancreatic neuroendocrine tumours, but with certain questions waiting to be solved for the management of PanNETs. Finally, the role of immunotherapy is still poorly understood. In this review, the data supporting current systemic treatment options for locally advanced or metastatic PanNETs are summarized. Strategies for treatment selection in patients with PanNETs based on patient, disease, or drug characteristics is provided, as well as a summary of current evidence on prognostic and predictive biomarkers. Future perspectives are discussed, focusing on current and forthcoming challenges and unmet needs of patients with these rare tumours.
Insights
Research on pancreatic neuroendocrine tumours (PanNETs) is vital for rare cancer treatment. This review summarizes current therapies and biomarkers for advanced PanNETs, guiding optimal patient management.
Area of Science:
- Oncology
- Rare Diseases
- Translational Research
Background:
- Pancreatic neuroendocrine tumours (PanNETs) are rare neoplasms requiring specialized research.
- Advances in clinical trials have expanded therapeutic options for advanced PanNETs over the past two decades.
Purpose of the Study:
- To review current systemic treatment options for locally advanced or metastatic PanNETs.
- To provide strategies for treatment selection based on patient, disease, and drug characteristics.
- To summarize evidence on prognostic and predictive biomarkers and discuss future perspectives.
Main Methods:
- Systematic review of phase III studies and clinical trial data.
- Analysis of current therapeutic strategies including somatostatin analogues, targeted therapies, chemotherapy, and peptide receptor radionuclide therapy.
- Evaluation of prognostic and predictive biomarkers and emerging treatments like immunotherapy.
Main Results:
- Established therapies include somatostatin analogues, targeted agents (sunitinib, everolimus), and chemotherapy (temozolomide, capecitabine).
- Peptide receptor radionuclide therapy is effective for well-differentiated tumors, though challenges remain.
- The role of immunotherapy in PanNET management is still under investigation.
Conclusions:
- Optimal management of PanNETs requires careful selection of available therapies based on individual patient and disease characteristics.
- Further research is needed to address unmet needs, particularly regarding immunotherapy and biomarker development.
- Continued investigation into rare tumor research is crucial for improving patient outcomes.
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