Carcinoid crisis: answers for patients and care partners

A carcinoid crisis is a sudden change in your blood pressure or heart rate during surgery. It can happen in people who have a neuroendocrine tumor (NET) that is secreting excess hormones. Your blood pressure may drop very low or go very high. Your heart may beat very fast. Some people also have trouble breathing.

A crisis can be treated. Your surgery and anesthesia teams know how to spot it and how to stop it quickly.

Everyone having surgery for a neuroendocrine tumor (NET) should be asked about these symptoms. This one applies to all patients.

Flushing, diarrhea, and wheezing are signs of something called carcinoid syndrome. It happens when a NET releases hormones into your body.

Knowing whether you have carcinoid syndrome helps your team plan your care. People with carcinoid syndrome have a higher chance of a crisis during surgery. Your team should take extra steps to be ready. If you do not have these symptoms, your risk is lower, and you may need fewer tests.

If you have a NET of the small intestine or the lung, you should have this test, or a similar blood test, before surgery. It is recommended for everyone in this group, whether or not you have symptoms.

The test measures a substance called 5-HIAA. For the urine version, you collect your urine over 24 hours. Some centers use a blood test instead. Either one gives your team the same information.

The test shows whether your tumor is releasing hormones into your body. A high level is a sign of carcinoid syndrome, even in people who feel well and have no flushing or diarrhea. That is why the test is done for everyone with these tumor types, and not only for people with symptoms.

Your result, along with your symptoms, helps your team decide what else you need before surgery.

Not everyone needs this test. It is recommended for people who have carcinoid syndrome, based on symptoms or on the urine or blood test.

If your team orders an echocardiogram, it means they want to check your heart valves before surgery.

Here is why. If you have carcinoid syndrome, the hormones from your tumor can slowly damage the valves in your heart. This is called carcinoid heart disease. Many people with it feel completely fine and have no symptoms. But surgery and anesthesia put stress on the heart, and that stress can bring out a problem that was hidden before.

Being asked to have this test does not mean your team thinks something is wrong with your heart. It is a check, and most of the time the result is reassuring.

The echocardiogram is a picture of your heart taken with sound waves. It is painless and takes about half an hour.

This applies only to the smaller group of people whose echocardiogram shows damage to the heart valves. Most people who have the test will not need these extra steps.

If your test does show valve damage, you should see a heart doctor (cardiologist) first. You may need medicine to protect your heart.

If the valve damage is severe, you may need heart surgery to repair or replace the valve before your tumor operation. You will need time to recover from that. Going into surgery with an untreated heart problem is risky. Fixing your heart first makes your tumor operation safer.

No. Many people take these medicines to help control the growth of their tumor, not because their symptoms are severe.

Being on one of these medicines does not mean you are sicker than someone who is not. It also does not tell your team how likely you are to have a crisis. Your symptoms and your test results are what matter.

Yes. These medicines can be continued to control your symptoms and your tumor. Your team will tell you the timing.

That is not needed.

For many years, doctors gave octreotide before surgery hoping it would prevent a crisis. Newer studies show it does not reliably prevent one. Some teams may still choose to give it, but it is not necessary.

If your team does not give it, that does not mean they are ignoring the risk. What actually protects you is the preparation: the testing, the planning, and the close monitoring during surgery.

Usually not. Antidepressants (such as SSRIs, like fluoxetine, sertraline, citalopram, escitalopram, paroxetine, fluvoxamine) were once thought to be unsafe for people with NETs. Studies have not found this to be true.

Always tell your team every medicine you take and follow their instructions.

Yes. Common pain and anesthesia medicines, including morphine and fentanyl, are safe to use for people with NETs.

Before your operation begins, your surgeons, anesthesiologists, and nurses hold a short meeting called a time-out. For patients at risk of a crisis, they should go over three things:

• What a carcinoid crisis looks like
• How they will spot one and tell each other right away
• Exactly which treatments they will give, and in what order

This means the plan is in place before anything happens.

Here is the typical monitoring you may expect:

• An arterial line. A thin tube in an artery, usually at your wrist. It shows your blood pressure with every heartbeat instead of every few minutes. Sometimes it is placed while you are still awake, so your team can watch your blood pressure from the very start.
• Intravenous (IVs). These let your team give fluids and medicines quickly if they need to.
• A central line. A larger IV in your neck or chest. Not everyone needs one. It can be used if you have heart disease and might need medicines to support your heart.
• A urinary catheter. A tube in your bladder that measures your urine. Urine output tells your team how well your organs are getting blood.

You may wake up with these lines in place.

Your team should treat a crisis right away. The goal is to end it as fast as possible, because a crisis that lasts longer is linked to more problems after surgery.

Treatment should start with medicines called vasopressors. These bring your blood pressure back up quickly. Examples are vasopressin, phenylephrine, and norepinephrine.

Most crises are controlled with intravenous fluids and these medicines alone.

Not giving octreotide first does not mean you are not being treated. It means your team is giving you something that works faster.

Studies show that octreotide on its own usually does not fix the blood pressure problem. Almost everyone still needs vasopressors. If a team reaches for octreotide first, the crisis lasts longer before the medicines that actually work are given. Longer crises are linked to more problems afterward.

So your team should start with the medicines that end the crisis fastest. Octreotide can still be given afterward, as an added treatment, if needed. It can be a tool, just not the first one.

No. This is an old belief, and studies have shown it is not true.

Doctors once thought epinephrine and similar medicines could set off a carcinoid crisis, so they avoided them. Researchers then compared people having surgery for NETs who received these medicines with people who did not. There was no difference.

It is safe to give epinephrine (and other similar medicines like norepinephrine or phenylephrine) in patients with NETs or with carcinoid syndrome.

Probably not. A routine octreotide drip after surgery has not been shown to prevent a crisis.