Neuroendocrine Tumours

Neuroendocrine Neoplasms = (NENs)

Types

• Well-differentiated neuroendocrine tumours (NETs)

  • Traditionally called carciinoid tumours

  • 5 year survival 67%

• Poorly differentiated neuroendocrine carcinomas (NECs)

  • Resembled small or large cell carcinomas

  • Poor outcome

Pancreas

• Insulinoma = episodic hypocglycaemia, confusion, visual change, unusual behaviour, dipahoresis, tremor, amnesia.

• Gastrinomas = Peptic ulcer disease, diarrhoea

• Glucagonoma = necrolytic migrating erythema, diabetes, anaemia, weight loss, diarrhoea, neuropsychiatric symptoms

• VIPoma = watery diarrhoea, hypokalemia, hypochloria.

Carcinoid

• 40 secretory products in carcinoid syndrome

• Tryptophan

  • 1% of tryptophan converted to serotonin normally

  • In carcinoid this is up to 70%

• Histamine

  • Some convert to histamine instead

  • Flushing, pruritus

• Kallikrein

  • Leads to Bradykinin

  • Flushing, increased intestinal motility

• Prostaglandins

  • Stimulate intestinal motility

• Tachykinin

  • SubstanceP + A + Neuropeptide K

  • Flushing and diarrhoea

Carcinoid Signs

• Venous telangectasia

• Diarrhoea

• Bronchospasm

• Cardiac valvular lesions

• Flushing

Lung NECs

• Non hormonal symptoms

  • Obstruction

  • Haemoptysis

  • Cough, wheeze

  • Chest pain

  • Recurrent pneumonia

• Carcinoid

  • As above

• Cushing’s

  • ACTH secreting

• Acromegaly

  • GHRH secreting

• DIPNECH

  • Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia

  • Respiratory symptoms

Resource:

• Neuroendocrine Tumours - Up To Date