Carcinoid syndrome

Other Names: Carcinoid tumor syndrome

Carcinoid syndrome refers to a group of symptoms that are associated with carcinoid tumors (rare, slow-growing tumors that occur most frequently in the gastroinestinal tract or lungs).

Presentation of the carcinoid syndrome.

Symptoms

Affected people may experience skin flushing, abdominal pain, diarrhea, difficulty breathing, rapid heart rate, low blood pressure, skin lesions on the face (telangiectasias), and wheezing.

In later stages, carcinoid syndrome may damage the heart valves, resulting in symptoms of congestive heart failure.

Cause

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.

Diagnosis

With a certain degree of clinical suspicion, the most useful initial test is the 24-hour urine levels of 5-HIAA (5-hydroxyindoleacetic acid), the end product of serotonin metabolism.

Patients with carcinoid syndrome usually excrete more than 25 mg of 5-HIAA per day.

Imaging

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

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

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.

Surgical resection of tumor and chemotherapy (5-FU and doxorubicin)

Carcinoid syndrome Resources
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NIH genetic and rare disease info

• NIH info on Carcinoid syndrome

Carcinoid syndrome is a rare disease.