Other Names: Carcinoid tumor syndrome
In later stages, carcinoid syndrome may damage the heart valves, resulting in symptoms of congestive heart failure.
The condition occurs when the carcinoid tumor secretes serotonin or other chemicals into the bloodstream. Only 10% of people with carcinoid tumors develop carcinoid syndrome; most have advanced stage carcinoid tumors that have spread to the liver.
Patients with carcinoid syndrome usually excrete more than 25 mg of 5-HIAA per day.
Usually, on a CT scan, a spider-like/crab-like change is visible in the mesentery due to the fibrosis from the release of serotonin. 8F-FDG PET/CT, which evaluate for increased metabolism of glucose, may also aid in localizing the carcinoid lesion or evaluating for metastases. hromogranin A and platelets serotonin are increased.
Prognosis varies from individual to individual. It ranges from a 95% 5-year survival for localized disease to an 80% 5-year survival for those with liver metastases.The average survival time from the start of octreotide treatment has increased to about 12 years.
Treatment generally involves addressing the underlying carcinoid tumor and medications to alleviate symptoms. The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.
- Octreotide (Brand name: Sandostatin LAR)Reduction of growth hormone and IGF-1 (somatomedin C) in acromegaly.
- Telotristat etiprate (Brand name: Xermelo) Treatment of carcinoid syndrome diarrhea in combination with somatostatin analog (SSA) therapy in adults inadequately controlled by SSA therapy.
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NIH genetic and rare disease info
Carcinoid syndrome is a rare disease.