ESMO E-Learning: Optimising Outcomes in Gastrointestinal Neuroendocrine Tumours

Learning Objectives

  • Understand the importance of the classification of neuroendocrine tumours.
  • Describe appropriate investigations in diagnosis of patients with neuroendocrine tumours.
  • Understand the importance of the multidisciplinary approach to the management of patients with a diagnosis of a neuroendocrine tumour.
  • Identify the treatment goals in managing patients with metastatic neuroendocrine tumours.
  • Describe treatment options for patients with advanced neuroendocrine tumours and be able to identify the sources of evidence-base. 
  • Have some knowledge of the chemotherapy used in the treatment of patients with advanced poorly-differentiated neuroendocrine tumours/carcinomas.
Title Duration Content CME Points CME Test
Optimising Outcomes in Gastrointestinal Neuroendocrine Tumours 44 min. 42 slides 1 Take Test
mcnamara-mairead
Mairéad McNamara
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In this E-Learning module, the author presents a classification and symptoms of neuroendocrine tumours (NETs), a multidisciplinary approach to diagnostic work-up, current treatment opportunities and future outlook. NETs are rare tumours but their incidence is rising, possibly due to the increased detection of asymptomatic disease. Because of the rarity of these tumours, not all doctors and local hospitals will have the full expertise to deal with this type of diagnosis and referral to a NET centre of excellence may be required. 

NETs often grow slowly and it may take years before symptoms appear and tumour is diagnosed. However, poorly-differentiated NETs may be fast-growing. Non-functioning NETs are discovered incidentally during surgery or on an investigation being carried out for other reasons; these NETs do not overproduce hormones and may not cause symptoms. Symptoms caused by NETs may be pain, nausea, change in bowel habits, and lung-related symptoms; functioning NETs may overproduce hormones resulting in hypoglycaemia, hyperglycaemia, diarrhoea, ulcers, or carcinoid syndromes, depending on hormone type. Somatostatin receptors are expressed in 70-90% of NETs. Result on somatostatin-Receptor scintigraphy is one factor that predicts effectiveness of somatostatin analogue therapy.

This E-learning module underlines the importance of a multidisciplinary team discussion and decisions in patients with NETs. The treatment depends on the primary site of NET, classification, size of the tumour, whether it has metastasised, and whether patient has carcinoid syndrome or overproduction of other hormones.

A watch and wait approach may be adopted in selected cases of incidental NETs. In terms of localised gastrointestinal NETs, the author presents the surgical options based on tumour site and recommendations for follow-up. In case of advanced gastrointestinal NETs, the treatment goals may be to remove the tumour by surgery (however, if the tumour has metastasised, this may not be possible), alleviate symptoms, control tumour growth, and maintain patient quality of life.

Initial therapy in advanced gastrointestinal NETs for hormone hypersecretion situations are somatostatin analogues and agents appropriate to the specific syndrome. In case of resectable hepatic metastases, surgery should be considered. In case of asymptomatic patients, the management strategies are observation alone or somatostatin analogues at disease progression or in case of symptoms.

In case of pancreatic NETs with progressive disease, for symptomatic patients the options are somatostatin analogues, everolimus or sunitinib; in case of symptomatic patients from tumour bulk the treatment strategy is somatostatin analogues with or without targeted therapy; in case of highly symptomatic patients chemotherapy is recommended; and in case of liver-predominant disease hepatic arterial embolisation.

In case of small bowel NETs and progressive disease, for symptomatic patients from tumour bulk, somatostatin analogues with or without chemotherapy are recommended; in case of liver-predominant disease hepatic arterial embolisation; and in refractory disease peptide receptor radioligand therapy (PRRT), if available.

All of the above treatment scenarios are illustrated in this module and supported by findings from the clinical trials. Furthermore, the author presents the latest findings from a clinical trial with everolimus versus placebo in patients with well-differentiated, advanced, progressive, non-functional NET of lung or gastrointestinal origin. The author also presents the study design for telotristat etiprate, a novel inhibitor of tryptophan hydroxylase, the rate-limiting Enzyme in serotonin biosynthesis; the role of chemotherapy in poorly-differentiated neuroendocrine carcinomas, as well as some ongoing studies in maintenance setting, sequencing of therapy, and combination therapy with PRRT and chemotherapy. 

Last update: 27 September 2016

The author has reported to have received travel and accommodation grants to attend conference from Ipsen and Speaker honorarium from Pfizer.