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Carcinoid tumours

  • Carcinoid tumours are rare neuroendocrine lesions
  • Arise from amine precursor uptake and decarboxylation (APUD) cells
  • Approximately 1,000 are identified in the United Kingdom each year

Carcinoid tumour

  • Most primary tumours arise from the gastrointestinal tract
  • Commonest sites of primary tumours are appendix (30%), small bowel (20%)
  • Foregut tumours produce little 5HIAA - often produce other hormones (e.g. gastrin)
  • Midgut and hindgut tumours produce increased amounts of 5HIAA
  • When metastasis to liver these tumours produce the carcinoid syndrome

Clinical features

  • Carcinoid tumours produce vague right-sided abdominal discomfort
  • Symptoms have often been present for a number of years prior to diagnosis
  • Previous investigations have often been normal
  • Diagnosis is often only made after urgent surgery - usually due to obstruction

Small bowel carcinoid tumour

Picture provided by Jenny Wagener, Hawke's Bay District Hospital, New Zealand

  • In those with carcinoid syndrome symptoms include diarrhoea and flushing
  • Flushing affects face and neck lasting only several minutes
  • Often precipitated by alcohol or chocolate
  • May be associated with palpitations or hypotension
  • Examination is often normal
  • A right-sided abdominal mass or hepatomegaly may be present
  • Other features include telangiectasia, pellagra, tricuspid regurgitation.
  • Sclerotic bone secondaries occasionally occur

Facial flushing associated with carcinoid syndrome

Investigation

  • Diagnosis confirmed by increased 24-hour urinary 5HIAA excretion
  • Plasma chromogranin A may be increased
  • Radiological investigations are rarely helpful
  • Ultrasound or CT may demonstrate an abdominal mass or liver secondaries
  • 111In - octreotide scintigraphy may identify primary or secondary tumour

Treatment

  • Diagnosis is often made after resection of primary tumour
  • Liver metastases are rarely amenable to hepatic resection
  • Conventional chemotherapy has poor response rate
  • Symptomatic carcinoid syndrome can often be palliated by:
    • Somatostatin analogues (e.g. octreotide)
    • Embolisation of hepatic metastases

Prognosis

  • Prognosis is better than for adenocarcinomas at similar sites
  • Median survival is 5 to 8 years
  • For surgically resectable tumours 10-year survival rates of  more than 60% have been reported

Appendiceal carcinoid tumours

  • Most common tumour of the appendix
  • Found in 0.5% of appendicectomy specimens
  • Accounts for 85% of all appendiceal tumours
  • Usually an incidental finding found during appendicectomy
  • 75% occur at the tip, 15% in the middle and 10% at the base of the appendix
  • 80% are less than 1 cm in diameter
  • Only 5% are greater than 2 cm in diameter
  • Locoregional spread or metastases are rare especially if tumour is less than 2cm
  • Appendicectomy is adequate if tumour less than 1 cm
  • Right hemicolectomy needed if tumour greater than 1 cm
  • Prognosis is good with 5-year survival of 90-100%

Carcinoid tumour of the appendix

Picture provided by Mr M Maniman, Royal Berkshire County Hospital, Reading, United Kingdom

Bibliography

Bax N D S, Wood H F, Batchelor A et al. The clinical manifestations of carcinoid disease. World J Surg 1996; 20: 142-146.

Ganim R B,  Norton J A.  Recent advances in carcinoid pathogenesis, diagnosis and management.  Surg Oncol 2000;  9:  173-179.

Hauser H, Wolf G, Uranus S et al. Neuroendocrine tumours in various organ systems. Eur J Surg Oncol 1995; 21: 297-300.

Onaitis M W, Kirshbom P M, Hayward T Z et al. Gastrointestinal carcinoids: characterization by site of origin and hormone production. Ann Surg 2000; 232: 549-556.

 

 
 

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