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]

Overview

Prognosis varies from individual to individual. It ranges from a 95% 5 year survival for localized disease to a 20% 5 year survival for those with liver metastases. The average survival time from the start of octreotide treatment has increased to about 12 years.

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

The outlook in patients with carcinoid syndrome is different from the outlook in patients who have carcinoid tumors without the syndrome. In people with the syndrome, the tumor has usually spread to the liver, which lowers the survival rate. People with carcinoid syndrome are also more likely to have a separate cancer (second primary tumor) at the same time. The outlook is more favorable with new treatment methods, such as Sandostatin.