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

If left untreated, patients with carcinoid syndrome may progress to develop flushing, diarrhea, and carcinoid heart disease (valvular heart disease and cardiac dysrythmias). Common complications of carcinoid tumor include increased risk of falls and injury (from hypotension), bowel obstruction, gastrointestinal bleeding, right-sided heart failure, and fibrosis of the tricuspid valve and pulmonary valve, and rarely the mitral valve in cases with left sided involvement. Prognosis is generally good and the 5-year survival rate of patients with carcinoid syndrome is approximately 69.7%.

Natural History

• If left untreated, patients with carcinoid syndrome may progress to develop flushing, diarrhea, and carcinoid heart disease (valvular heart disease and cardiac dysrythmias):
  • Pathologically, the cardiac valves become thickened because of fibrosis
  • Tricuspid and pulmonic valves are affected to a greater extent than the mitral and aortic valves
• They have a very slow growth rate compared to most malignant tumors

Complications

Common complications of carcinoid tumor include:

• Increased risk of falls and injury (from hypotension)
• Bowel obstruction
• Gastrointestinal bleeding
• Carcinoid heart disease develops in more than one-third of patients with carcinoid syndrome:

• Fibrosis of the tricuspid valve and pulmonary valve, rarely the mitral valve in cases with left sided involvement

• Tricuspid and pulmonic regurgitation ("TIPS" - Tricuspid Insufficiency, Pulmonic Stenosis)
• Pulmonary stenosis
• Mitral and aortic insufficiency
• Cardiac dysrhythmias

Prognosis

• Prognosis of carcinoid tumor is generally good and the 5-year survival rate of patients is approximately:

• Stomach-75.1%
• Small Intestine-76.1%
• Appendix-76.3%
• Rectum-87.5%

• Factors that determine the prognosis of patients with gastrointestinal carcinoid tumors include:

• Site of origin
• Size of the primary tumor
• Anatomical extent of disease

• Negative prognostic factors include:
  • Carcinoid heart disease
  • High concentrations of the tumor markers - urinary 5-HIAA and plasma chromogranin A
  • Metastasis to the liver
  • Carcinoid tumor in the thymus
  • Overexpression of the proliferation antigen Ki-67
  • Mutation in the p53 gene