Carcinoid tumors and carcinoid heart disease

KEY POINTS

Carcinoid tumors are rare neuroendocrine tumors, arising from the small bowel (75% to 80%) or lung (20% to 30%).
Carcinoid tumors secrete biologically active substances, including serotonin, histamine, tachykinins, kallikrein, and prostaglandins and they account for the presentation of carcinoid syndrome (flushing, diarrhea, and bronchospasm).
Carcinoid syndrome typically occurs in patients with small-bowel primaries and hepatic metastases.
Carcinoid syndrome therapy should be optimized prior to any cardiac or noncardiac procedure owing to the risk of intraprocedural carcinoid crisis. All patients with carcinoid syndrome should receive preprocedural octreotide, and intraprocedural hypotension should be treated with further doses of intravenous octreotide.
Carcinoid heart disease occurs in approximately 20% of patients with carcinoid syndrome and has characteristic echocardiographic appearance, generally affecting the tricuspid and pulmonary valves.
Cardiac surgical intervention is the only current effective treatment option for carcinoid heart disease, and surgical outcomes have improved over the past three decades.

Introduction

Carcinoid tumors are rare neuroendocrine tumors affecting 1.2 to 2.1 people per 100,000 people years. These tumors most commonly arise from the small bowel, typically ileum and appendix, in 75% to 80% of cases, and lungs in 20% to 30% of cases. Other less-common sites include ovaries and kidneys. The tumor secretes multiple biologically active substances, including serotonin, histamine, tachykinins, kallikrein, and prostaglandins. The overall 10-year survival with small bowel neuroendocrine tumors are 82% without and 42% with metastatic disease.

Carcinoid syndrome

Signs and symptoms of carcinoid syndrome result from the secretion of vasoactive hormones and include flushing, diarrhea, and bronchospasm. This is typically seen with hepatic metastases in cases of small-bowel primary tumors, as hormone may be cleared hepatically prior to entering the systemic circulation.

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