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

The presence of liver metastasis is associated with a particularly poor prognosis among patients with carcinoid tumor. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor. Common complications of carcinoid tumor include gastrointestinal bleeding, right heart failure, bowel obstruction, hypotension, and tricuspid insufficiency and pulmonary stenosis.

Natural History

• They have a very slow growth rate compared to most malignant tumors.

• Carcinoid heart disease develops in more than one-third of patients with carcinoid syndrome. Pathologically, the cardiac valves become thickened because of fibrosis, and the tricuspid and pulmonic valves are affected to a greater extent than the mitral and aortic valves.

Complications

Common complications of carcinoid tumor include:

• Increased risk of falls and injury (from hypotension)
• Bowel obstruction (from tumor)
• Gastrointestinal bleeding
• Right 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

Prognosis is generally good, and the 5-year survival rate of patients with carcinoid syndrome is approximately 69.7%.

• Factors that determine the clinical course and outcome of patients with gastrointestinal 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.
• Thymic carcinoid tumor are generally thought to carry a poor prognosis due to high rates of recurrence and metastases.