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Carcinoid syndrome

Flush syndrome; Argentaffinoma syndrome

 

Carcinoid syndrome is a group of symptoms associated with carcinoid tumors. These are tumors of the small intestine, colon, appendix, and bronchial tubes in the lungs.

Causes

 

Carcinoid syndrome is the pattern of symptoms sometimes seen in people with carcinoid tumors. These tumors are rare, and often slow growing. Most carcinoid tumors are found in the gastrointestinal tract and lungs.

Carcinoid syndrome occurs in very few people with carcinoid tumors, after the tumor has spread to the liver or lung.

These tumors release too much of the hormone  serotonin , as well as several other chemicals. The hormones cause the blood vessels to open (dilate). This causes carcinoid syndrome.

 

Symptoms

 

The carcinoid syndrome is made up of of four main symptoms including:

  • Flushing (face, neck, or upper chest), such as widened blood vessels seen on the skin ( telangiectasias )
  • Difficulty breathing, such as wheezing
  • Diarrhea
  • Heart problems, such as leaking heart valves, slow heartbeat, low or high blood pressure

Sometimes symptoms are brought on by physical exertion, or eating or drinking things such as blue cheese, chocolate, or red wine.

 

Exams and Tests

 

Most of these tumors are found when tests or procedures are done for other reasons, such as during abdominal surgery.

If a physical exam is done, the health care provider may find signs of: 

  • Heart valve problems, such as murmur
  • Niacin-deficiency disease ( pellagra )

Tests that may be done include:

  • 5-HIAA levels in urine
  • Blood tests (including serotonin  and chromogranin blood test)
  • CT and MRI scan of the chest or abdomen
  • Echocardiogram
  • Octreotide radiolabeled scan

 

Treatment

 

Surgery to remove the tumor is usually the first treatment. It can permanently cure the condition if the tumor is completely removed.

If the tumor has spread to the liver, treatment involves transplantation of liver.

When the entire tumor cannot be removed, removing large portions of the tumor ("debulking") can help relieve the symptoms.

Octreotide (Sandostatin) or lanreotide (Somatuline) injections are given to people with advanced carcinoid tumors that cannot be removed with surgery.

People with carcinoid syndrome should avoid alcohol, large meals, and foods high in tyramine (aged cheeses, avocado, many processed foods), because they may trigger symptoms.

Some common medicines, like selective serotonin reuptake inhibitors (SSRIs), such as paroxetine (Paxil) and fluoxetine (Prozac), may make symptoms worse by increasing levels of serotonin. However, DO NOT stop taking these medicines unless your provider tells you to do so.

 

Support Groups

 

Learn more about carcinoid syndrome and get support from:

 

Outlook (Prognosis)

 

The outlook in people with carcinoid syndrome is sometimes different from the outlook in people who have carcinoid tumors without the syndrome.

Prognosis also depends on the site of tumor. In people with the syndrome, the tumor has usually spread to the liver. This lowers the survival rate. People with carcinoid syndrome are also more likely to have a separate cancer (second primary tumor) at the same time.

The outlook is more favorable thanks to new treatment methods.

 

Possible Complications

 

Complications of carcinoid syndrome may include:

  • Increased risk of falls and injury (from low blood pressure)
  • Bowel obstruction (from tumor)
  • Gastrointestinal bleeding
  • Heart valve failure

A fatal form of carcinoid syndrome, carcinoid crisis may occur as a side effect of surgery, anesthesia or chemotherapy.

 

When to Contact a Medical Professional

 

Call for an appointment with your provider if you have symptoms of carcinoid syndrome.

 

Prevention

 

Treating the tumor reduces the risk of carcinoid syndrome.

 

 

References

National Cancer Institute. Gastrointestinal carcinoid tumors treatment (PDQ) - health professional version. Updated July 8, 2015. www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq . Accessed September 22, 2016.

Öberg K. Neuroendocrine gastrointestinal and lung tumors (carcinoid tumors), the carcinoid syndrome, and related disorders. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology . 25th ed. Philadelphia, PA: Elsevier; 2016:chap 43.

 
  • Serotonin uptake - illustration

    Carcinoid syndrome is the pattern of symptoms that typically are exhibited by people with carcinoid tumors. The symptoms include bright red facial flushing, diarrhea, and occasionally wheezing. A specific type of heart valve damage can occur, as well as other cardiac problems. Carcinoid tumors secrete excessive amounts of the hormone serotonin. Surgery with complete removal of the tumor tissue is the ideal treatment. It can result in a permanent cure if it is possible to remove the tumor entirely.

    Serotonin uptake

    illustration

    • Serotonin uptake - illustration

      Carcinoid syndrome is the pattern of symptoms that typically are exhibited by people with carcinoid tumors. The symptoms include bright red facial flushing, diarrhea, and occasionally wheezing. A specific type of heart valve damage can occur, as well as other cardiac problems. Carcinoid tumors secrete excessive amounts of the hormone serotonin. Surgery with complete removal of the tumor tissue is the ideal treatment. It can result in a permanent cure if it is possible to remove the tumor entirely.

      Serotonin uptake

      illustration

    A Closer Look

     

      Talking to your MD

       

        Self Care

         

          Tests for Carcinoid syndrome

           

             

            Review Date: 8/15/2016

            Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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