Carcinoid syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]

Natural History

Carcinoid tumours originate from neuroendocrine cells (Enterochromaffin or amine precursor uptake and decarboxylase [APUD] cells), which embryologically are of neural crest origin. Gastrointestinal carcinoids derive from cells that migrate from the neural crest to the foregut, midgut and hindgut. They are normally found throughout the gastrointestinal tract from mouth to anus, with the highest concentration of cells in the appendix and small intestine 49. The pancreas contains a large number of these cells, the biliary tree only a few and the liver normally contains none

Complications

• Increased risk of falls and injury (from hypotension)
• Bowel obstruction (from tumor)
• Gastrointestinal bleeding
• Right-sided heart failure
• Serotonin causes fibrosis of the tricuspid valve and pulmonary valve, more rarely the mitral valve in cases with left sided involvement
  • "TIPS" - Tricuspid Insufficiency, Pulmonic Stenosis (fibrosis of tricuspid and pulmonary valves)

Prognosis

• Factors that determine the clinical course and outcome of patients with GI carcinoid tumors are complex and multifaceted and include the following:

• The site of origin
• The size of the primary tumor
• The anatomical extent of disease

• Elevated expression of the proliferation antigen Ki-67 and the tumor suppressor protein p53 have been associated with poorer prognosis.
• Adverse clinical prognostic indicators include:

• Carcinoid syndrome
• Carcinoid heart disease
• High concentrations of the tumor markers urinary 5-HIAA and plasma chromogranin A.

• In people with the carcinoid syndrome, the tumor has usually spread to the liver, which lowers the survival rate
• The outlook is more favorable with new treatment methods, such as sandostatin..