Advances in the management of neuroendocrine tumours


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Martyn Caplin

The Royal Free Hospital, London, UK

Abstract

Neuroendocrine Tumours (NETs) are relatively rare tumours however in the last 20 years there has been a doubling in the reported incidence to 5-6 per 100,000 population. NETs are classified according to site of origin and whether they secrete peptides. They are graded according to size and mitotic/proliferation (Ki67) index. The best serological marker is chromogranin A.

The most sensitive imaging modality for metastatic NETs is the Indium-111 pentetreotide scan (OctreoScan) however new PET agents such as Gallium-68 octreotate PET imaging offers the prospect of even greater sensitivity. First line therapy for NETs is surgery however most patients by time of presentation have advanced disease. There is increasing evidence for debulking surgery. The mainstay of symptomatic therapy for NETs particularly in carcinoid syndrome has been somatostatin analogues especially once monthly preparations Sandostatin LAR and Somatuline Autogel.

The first randomised study of anti-tumour effect was presented 2009 demonstrating benefit of Sandostatin LAR over placebo in midgut NETs. There are multiple therapeutic modalities considered for patients with progressive NETs and the choice should be based on tumour origin and biology. Options include chemotherapy, interferon, (chemo)embolisation, radiofrequency ablation, radionuclide targeted agents e.g. Yttrium-90 DOTA Octreotide or Lutetium-177 DOTA Octreotate and new biologicals.

In 2009 the first study of Sunitinib (Sutent), an oral tyrosine kinase inhibitor with action against VEGFR and PDGFR, demonstrated benefit over placebo in time to progression for pancreatic neuroendocrine tumours with slowly progressive disease. There are ongoing studies in other tyrosine kinase inhibitors as well as agents targeting Epidermal Growth Factor Receptor and mTOR inhibitors.

The management of patients with NETs is fast evolving. It is important to build as much evidence base as possible hence patients should be seen in specialist centres and entered into national studies such as those coordinated by NCRI / UK & Ireland NET Society.