Multimodal treatment options for advanced gastroenteropancreatic neuroendocrine tumours (NET) of jejunum/ileum and of pancreatic origin are reviewed. Current topics being discussed are: European Neuroendocrine Tumour Society 2006/7, American Joint Cancer Committee/Union Internationale Contre le Cancer 2009 and WHO 2010 recommendations for grading and staging of NET; surgery of the primary tumour in distant metastasised disease; surgery of metastatic liver disease and impact on survival; somatostatin analogues for symptom control and for tumour control; selective internal radiation therapy with 90Y-microspheres as novel local ablative therapy in liver metastases; peptide receptor radionuclide therapy; novel chemotherapy regimens (eg, temozolomide) and novel targeted therapies (eg, sunitinib and everolimus).
- gastroenteropancreatic system
- islet cell tumour
- neuroendocrine carcinoma
- neuroendocrine tumour
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Competing interests CJA has received research contracts (Ipsen, Novartis), lecture honorarium (Ipsen, Novartis, Pfizer) and advisory board honorarium (Ipsen, Novartis). BG declares no competing interests.
Provenance and peer review Not commissioned; externally peer reviewed.
Amendment After this manuscript was accepted for publication, the clinical phase 3 study of sunitinib in patients with neuroendocrine tumors of the pancreas and the clinical phase 3 study of everolimus in patients with neuroendocrine tumors of the pancreas were both been published as follows:
Raymond E, Dahan L, Raoul JL, et al. Sunitinib malate for the treatment of pancreatic neuroendocrine tumors. N Engl J Med 2011;364:501–13.
Yao JC, Shah MH, Ito T, et al. Everolimus for advanced pancreatic neuroendocrine tumors. N Engl J Med 2011;364:514–23.