- Carcinoid syndrome is a group of symptoms that occur in patients who have Carcinoid Tumors.
- Carcinoid Tumors are not very common. These cancers most often develop in intestine or lungs and arise from cells in the body that produce certain hormones. Initially, when the cancer is within the intestine, these substances are removed from circulation by the liver. However, once the cancer has spread here, the liver can no longer remove them. The hormones then travel throughout the body and cause the symptoms associated with carcinoid syndrome. These symptoms include flushing, Diarrhea, and low
blood pressure. Medications are available that can treat
some of the symptoms with varying degrees of success.
However, treating the symptoms does not change the long-term
outlook, which is more dependent on the size of the tumor,
its location, and whether or not it has spread to the liver.
- For more details regarding carcinoid syndrome, please see the section "Carcinoid Tumor."
- In people with intestinal Carcinoid Tumors,
carcinoid syndrome only occurs in those people whose cancers
have spread to the liver.
- People with Carcinoid Tumors that
develop outside the intestinal tract can have the symptoms
of carcinoid syndrome even without it having spread to the
- Carcinoid Tumors
release large amount of hormones that cause the various
symptoms associated with carcinoid syndrome.
- The classic symptoms of carcinoid syndrome are flushing of the skin, Diarrhea, and damage
to the valves of the heart.
- The flushing associated with
carcinoid syndrome may involve just the face or the entire
body. The symptoms can last anywhere from a few minutes to
several hours. Also, there may be excessive tearing,
excessive saliva production, or swelling of the face.
- Some people develop Hives with the flushing,
while others develop wheezing.
- Early on during the course of the
disease, the symptoms may only occur at certain times.
However, as the disease progresses, the symptoms can occur
more frequently and last longer. The symptoms can become
- Certain factors, such as stress,
certain foods, and alcohol, can also trigger the symptoms.
- Less common symptoms include
wheezing, a drop in blood pressure, and prominent
capillaries (the smallest blood vessels).
- The Diarrhea that is
associated with this disease can occur even when the person
- Heart valve damage usually affects the right side of the heart and can cause leaking tricuspid valves, narrowed pulmonary valves, and right heart failure. In people with Carcinoid Tumors that
develop in the lungs, they can have problems with the valves
on the left side of the
- Approximately 5 percent of people with Carcinoid Tumors
develop the symptoms of carcinoid syndrome.
- The chance of developing symptoms is dependent on the site of the cancer. For example, up to 60 percent of those with small bowel carcinoids develop symptoms, while only about 1 percent of those with Carcinoid Tumors that develop in the appendix have the symptoms. Almost no one with rectal Carcinoid Tumors will
have the symptoms of carcinoid syndrome.
- Carcinoid syndrome is caused by the release of hormones from Carcinoid Tumor
- Usually, in cases of intestinal Carcinoid Tumors,
carcinoid syndrome only occurs after the cancer has spread
to the liver.
- However, for Carcinoid Tumors that
develop outside the intestinal tract (such as the lungs,
thymus, etc.), the syndrome can occur even if the cancer has
not spread to the
- Often, there is a delay in the
diagnosis of carcinoid syndrome. This is because, initially,
the symptoms are vague and only occur sporadically. Also,
the symptoms of carcinoid syndrome can be caused by a number
of other diseases.
- Additionally, the characteristic
symptoms of carcinoid syndrome only occur after the cancer
has spread to the liver.
- If the person has symptoms of carcinoid syndrome, then blood and urine tests for 5-HIAA (5-hyroxyindolacetic acid) can be done. Carcinoid Tumors
secret a substance known as serotonin. This substance is
then converted to 5-HIAA and excreted in the urine.
- Since most people with carcinoid
syndrome will have an elevated urine 5-HIAA level, this the
most useful test.
- Some people with carcinoid syndrome
may have normal urinary 5-HIAA levels. In these cases, they
may need to have their blood serotonin levels measured to
make the diagnosis.
- Also, in some cases of gastric Carcinoid Tumors,
very little 5-HIAA is formed. Therefore, other lab tests
such as 5-HTP levels, urine serotonin levels, or histamine
levels may need to be checked.
- Sometimes, diagnosis is unclear as
when the person complains of flushing, but the doctor has
not seen it. In such cases, medicines, such as alcohol,
pentagastrin, or extremely low doses of epinephrine can be
given to cause flushing.
- Bananas, plantains, pineapples,
kiwi fruits, walnuts, plums, pecans, avocados, guaifenesin,
and acetaminophen can elevate 5-HIAA levels. Aspirin and
Levodopa can decrease 5-HIAA levels. Therefore, these
variables must be taken into account when checking 5-HIAA
- The risk factor for developing carcinoid syndrome is having a Carcinoid Tumor.
Also, as stated above, the chance of developing the symptoms
of carcinoid syndrome depend on the location of the cancer
and whether or not it has spread to the liver.
- Treatment depends on whether or not carcinoid syndrome
is present and the severity of the symptoms.
- Because most people with carcinoid syndrome have
metastatic disease to the liver, surgery is not really an
- If symptoms are not too severe, they can be controlled
with certain medications.
- For Diarrhea, medicines
such as Loperamide, Diphenoxylate/atropine,
Cyproheptadine, or Methysergide can be used.
- For flushing, treatment is given if the symptoms are
frequent or severe. Medications that can be used include
anti-histamines (such as Diphenhydramine) along with
anti-ulcer medications (such as ranitidine). These two
medicines in combination can help reduce the flushing.
- Another medicine called Phenoxybenzamine can also be
used to help reduce the flushing.
- People with wheezing and trouble breathing caused by
lung carcinoids can be treated with steroids and medicines
such as theophylline. Inhalers such as Albuterol should be
avoided because they can make the symptoms worse.
- One of the most effective drugs used in treating people with carcinoid syndrome is Octreotide. It can reduce the wheezing, flushing, and Diarrhea associated
with the disease in over 75% of the cases. It can also be
used to treat the low blood pressure that can sometimes
occur with this disease. It does not treat any heart valve
problems associated with the disease and it is not clear
whether it decreases the fibrosis caused by the disease.
The other problem with Octreotide is that it has to be
given either intravenously or by injections several times
- Surgical removal of the cancer is the best treatment for
tumors from the appendix or large intestine that are less
than 2 centimeters in size.
- Carcinoid Tumors from other parts of the intestinal tract are also surgically removed if the cancer has not spread. However, remember, the diagnosis of Carcinoid Tumors is usually delayed. The vast majority of people have liver involvement by the time the diagnosis is made. In a few cases, where only one metastatic lesion is found in the liver, removal of the cancer and the metastatic liver lesion results in some improvement of survival. However, in the majority of cases, surgery may temporarily reduce symptoms but it does not change the long-term outlook. Thus, in most cases, surgery is usually not performed for intestinal Carcinoid Tumors.
- In some people with carcinoid syndrome due to a tumor in
the lung or other site outside the intestine, they may be
cured if the tumor is removed.
- There are also other treatments that have been tried for Carcinoid Tumors. The
problem is that these cancers generally do not respond to
chemotherapy or to radiation therapy. Therefore, these are
not very good options.
- Certain therapies do reduce the size of the tumor as
well as the symptoms of carcinoid syndrome. However, these
treatments do not really change the long-term prognosis.
- Hepatic artery embolization - in this procedure, the
blood supply to the tumor is cut off by blocking the
artery. In 90% of people, this will reduce the symptoms
temporarily. Complications of this treatment include pain,
fever, and problems with liver function. Also, symptoms of
carcinoid syndrome may increase temporarily after the
- Local radiation therapy - this is often not very
effective because these tumors are often resistant to
- Selective hepatic artery chemotherapy infusion - in this procedure, an angiogram is performed to see which artery is feeding the cancer. Then, chemotherapy is given into that specific artery in an attempt to destroy the cancer. However, as mentioned above, Carcinoid Tumors
are often very resistant to chemotherapy.
- Newer treatments are being studied. Hopefully, research
will result in more options in the future.
- In some people with bronchial Carcinoid Tumors as well as Carcinoid Tumors
outside the intestinal tract, surgical removal is an option
even if they have carcinoid syndrome. Radiation therapy and
chemotherapy are not options because these treatments
usually do not work. .
- Once the person has developed flushing, survival on
average is about 2 to 3 years. Also, the higher the urine
5-HIAA levels, the worse the prognosis.
- Heart valve damage
- Dehydration if the Diarrhea is severe
- Heart failure depending on the
extent of the valve damage
- Trouble breathing
- Angina or chest pain if the blood
pressure drops significantly
- Abdominal pain or Intestinal Obstruction if the lining of the abdominal cavity
becomes thick and hard
- There is really no way to prevent
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