Carcinoid Syndrome

Carcinoid syndrome is a rare medical condition that occurs when a carcinoid tumor, a type of neuroendocrine tumor, secretes excessive amounts of serotonin and other chemicals into the bloodstream.

Causes and Risk Factors

Carcinoid tumors are typically found in the gastrointestinal tract, lungs, or thymus gland. When these tumors metastasize to the liver, they can produce large amounts of serotonin, which is then released into the systemic circulation. The liver normally breaks down serotonin, but when the tumor has spread to the liver, it can no longer perform this function effectively.

Symptoms

The symptoms of carcinoid syndrome can vary depending on the location and size of the tumor, as well as the amount of serotonin produced. Common symptoms include:

  • Flushing: a sudden, intense reddening of the skin, often accompanied by a feeling of warmth or burning
  • Diarrhea: frequent, watery stools that can lead to dehydration and electrolyte imbalances
  • Wheezing and shortness of breath: due to the constriction of airways in response to serotonin release
  • Heart valve abnormalities: the constant exposure to high levels of serotonin can cause scarring and thickening of the heart valves, leading to problems with blood flow
  • Weight loss and fatigue: due to the malabsorption of nutrients and the body's increased energy expenditure

Diagnosis

The diagnosis of carcinoid syndrome is typically made through a combination of physical examination, medical history, laboratory tests, and imaging studies. These may include:

  • 24-hour urine test: to measure the levels of serotonin metabolites in the urine
  • Blood tests: to evaluate liver function, electrolyte levels, and other markers of tumor activity
  • Imaging studies: such as CT scans, MRI scans, or PET scans to locate the primary tumor and assess for metastasis
  • Biopsy: to confirm the presence of a carcinoid tumor and determine its histological characteristics

Treatment

The treatment of carcinoid syndrome depends on the extent of disease, the location of the primary tumor, and the patient's overall health. Options may include:

  • Surgery: to remove the primary tumor and any metastatic lesions
  • Chemotherapy: to slow the growth of the tumor and reduce symptoms
  • Targeted therapies: such as somatostatin analogs, which can help control symptoms by reducing serotonin production
  • Interferon therapy: to stimulate the immune system's response to the tumor
  • Liver-directed therapies: such as embolization or radiofrequency ablation, to reduce the tumor burden in the liver

Prognosis

The prognosis for patients with carcinoid syndrome varies depending on the extent of disease and the effectiveness of treatment. With proper management, many patients can experience significant improvement in symptoms and quality of life.

Article last updated on: 6th June 2025.
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