Neuroendocrine Tumours

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Neuroendocrine Tumours Current Treatments

• Mark WJ Strachan
• Metabolic Unit, Western General Hospital,
  Edinburgh

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NETs Can Arise in Many Different Places
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Spectrum of Malignancy
Benign
Malignant
Appendiceal carcinoids Insulinomas Gastric
carcinoids
Non-functioning pancreatic NETs Gastrinomas Glucag
onomas Small bowel carcinoids
Small cell lung cancer
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Hormone-Producing Glands
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Hormones Bind to Receptors
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NET hormones

• Carcinoids
• Serotonin
• Pancreatic NETs
• Gastrin
• Insulin
• Glucagon
• VIP

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Serotonin

• Manufactured mainly in the bowel and the brain
• Released in response to noxious foods
• Causes diarrhoea and vomiting
• Can improve mood and reduce appetite
• Also is a growth factor for cells
• Metabolised to 5-HIAA, which can be measured in a
  24 hour urine sample

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Kallikrein

• Vasoactive hormone
• Helps reduce blood pressure by cause dilation of
  blood vessels
• Causes flushing

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Clinical Features of Carcinoid Syndrome

• Diarrhoea - Serotonin
• Flushing - Kallikrein
• Wheeze probably Serotonin
• Heart valve problems - Serotonin

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NET hormones

• Chromogranin A and B
• Small Bowel Carcinoids
• Serotonin
• Kallikrein
• Pancreatic NETs
• Most are non-functional
• Gastrin
• Insulin
• Glucagon
• VIP

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Treatment Options for NETs

• Observation
• Surgery
• Somatostatin analogue therapy (if Carcinoid
  syndrome)
• Chemotherapy
• Radiotherapy
• (Chemo) Embolisation procedure
• Radio-Frequency Ablation
• Targeted Radionuclide Therapy -
  MIBG/radio-labelled somatostatin analogue
• Interferon
• Sunitinib or Everolimus pancreatic NETs

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Factors that Determine which Treatments We Use

• Surgery
• Can the tumour be removed completely cured?
• Is there a tumour mass causing a local problem
  that surgery will help?
• (Does removal of the primary tumour slow the
  progression of secondary deposits?)
• (Liver transplantation?)

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Factors that Determine which Treatments We Use

• Hormones
• Does the person have hormone-related symptoms?
• Measure 5-HIAA in urine and pancreas hormones in
  blood

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Octreotide Scanning
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Factors that Determine which Treatments We Use

• Hormones
• Does the person have hormone-related symptoms
• What is the extent of the cancer and how quickly
  is it growing?
• Can get a clue from the biopsy specimen number
  of cells dividing
• Change over interval scans

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Treatment Options for NETs

• Observation
• Surgery
• Somatostatin analogue therapy (if Carcinoid
  syndrome)
• Chemotherapy
• Radiotherapy
• (Chemo) Embolisation procedure
• Radio-Frequency Ablation
• Targeted Radionuclide Therapy -
  MIBG/radio-labelled somatostatin analogue
• Interferon
• Sunitinib or Everolimus pancreatic NETs

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Treatment Options for NETs

• Observation
• Surgery
• Somatostatin analogue therapy (if Carcinoid
  syndrome)
• Chemotherapy
• Radiotherapy
• (Chemo) Embolisation procedure
• Radio-Frequency Ablation
• Targeted Radionuclide Therapy -
  MIBG/radio-labelled somatostatin analogue
• Interferon
• Sunitinib or Everolimus pancreatic NETs

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Conventional Chemotherapy

• Crude therapy that targets cells which are
  multiplying rapidly
• Given over several cycles with close monitoring
  of tumour response

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Radiotherapy
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Treatment Options for NETs

• Observation
• Surgery
• Somatostatin analogue therapy (if Carcinoid
  syndrome)
• Chemotherapy
• Radiotherapy
• (Chemo) Embolisation procedure
• Radio-Frequency Ablation
• Targeted Radionuclide Therapy -
  MIBG/radio-labelled somatostatin analogue
• Interferon
• Sunitinib or Everolimus pancreatic NETs

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Ablation and Embolisation

• Can be given in most large centres
• Only targets cancer deposits in the liver
• Destructive therapy so potential for rapid
  release of hormones from the dying cells
• This can cause major swings in blood pressure

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Radionuclide Therapy
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Peripheral Uptake of Radioactive Label
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Radiation Crossfire
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Radionuclide Therapy

• MIBG therapy Glasgow, Dundee, Aberdeen
• DOTA-Octreotate London
• Need for isolation
• Bone marrow toxicity can lower blood cell counts
• Can affect liver and kidney function

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Treatment Options for NETs

• Observation
• Surgery
• Somatostatin analogue therapy (if Carcinoid
  syndrome)
• Chemotherapy
• Radiotherapy
• (Chemo) Embolisation procedure
• Radio-Frequency Ablation
• Targeted Radionuclide Therapy -
  MIBG/radio-labelled somatostatin analogue
• Interferon
• Sunitinib or Everolimus pancreatic NETs

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Summary

• NETs are rare
• Lots of different treatment options which need
  to be tailored to the individual
• More treatments are on the way..