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

• 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