Carcinoid

Book an Appointment

Overview

Carcinoid tumors develop from neuroendocrine cells in the gastrointestinal tract (90%), pancreas, pulmonary bronchi, and rarely the genitourinary tract. More than 95% of all gastrointestinal carcinoids originate in only 3 sites: the appendix, ileum, and rectum.

Although carcinoids are often benign or only locally invasive, those affecting the ileum and bronchus are frequently malignant. Malignant tumors range between well differentiated and poorly differentiated, with corresponding variability in aggressiveness. They typically spread to the liver and/or regional lymph nodes, although other metastatic sites are possible.

Carcinoids can be

Endocrinologically inert

Endocrinologically active (produce hormones)

The most common endocrinologic syndrome arising from carcinoid tumors is carcinoid syndrome; however, most patients with carcinoid tumors do not develop carcinoid syndrome. The likelihood that a tumor will be endocrinologically active varies with its site of origin, being highest for tumors originating in the ileum and proximal colon (40 to 50%). The likelihood is lower with bronchial carcinoids, lower still with appendiceal carcinoids, and essentially zero with rectal carcinoids.

Endocrinologically inert carcinoids are suspected because of their symptoms and signs (eg, pain, luminal bleeding, gastrointestinal obstruction). They can be detected by angiography, CT, or MRI. Small-bowel carcinoids may exhibit filling defects or other abnormalities on barium x-rays. Definitive diagnosis and grading are determined histologically after biopsy or resection.

Staging evaluation typically includes imaging with MRI (probably preferred over CT scan) and sometimes imaging with somatostatin receptor-based imaging techniques, which can be useful in detecting endocrinologically inert tumors as well.

Treatment of nonmetastatic carcinoid tumors is usually surgical resection. The type of surgery depends on the location and the size of the tumor.


Symptoms

Skin flushing. The skin on your face and upper chest feels hot and changes color — ranging from pink to purple. ...

Facial skin lesions. Purplish areas of spiderlike veins may appear on your nose and upper lip.

Diarrhea. ...

Difficulty breathing. ...

Rapid heartbeat.

Causes

Carcinoid syndrome is caused by a carcinoid tumor that secretes serotonin or other chemicals into your bloodstream. Carcinoid tumors occur most often in the gastrointestinal tract, including your stomach, small intestine, appendix, colon and rectum.


Risk factors

Doctors don't know for sure why people get them. But a few things may put you at a higher risk.



Genetic disease. You may get carcinoid tumors if you have an illness called multiple endocrine neoplasia type 1 (MEN1). It's a disease that's passed down through your family. About 10% of these tumors are due to MEN1.


Another condition that can raise your risk for them is neurofibromatosis type 1.


Race. More African-Americans than whites get carcinoid tumors in the GI tract.



Gender. Women are slightly more likely than men to have this type of cancer.


Age. Most people are diagnosed with carcinoid tumors in their 40s or 50s.


Conditions. You're more likely to get a tumor in your stomach if you have a disease like pernicious anemia or Zollinger-Ellison syndrome, which changes the amount of acid your stomach makes.

Older age. Older adults are more likely to be diagnosed with a carcinoid tumor than are younger people or children.

Sex. Women are more likely than men to develop carcinoid tumors.

Family history. A family history of multiple endocrine neoplasia, type 1 (MEN 1), increases the risk of carcinoid tumors.


Calendar Schedule

Have a medical question?

We are available to help you with all your questions and concerns.

Complications

Having carcinoid syndrome can cause the following complications:


Carcinoid heart disease. Some people with carcinoid syndrome develop carcinoid heart disease. Carcinoid syndrome causes problems with the heart valves, making it difficult for them to function properly. As a result, the heart valves may leak.


Signs and symptoms of carcinoid heart disease include fatigue and shortness of breath. Carcinoid heart disease can eventually lead to heart failure. Surgical repair of damaged heart valves may be an option.


Carcinoid crisis. Carcinoid crisis causes a severe episode of flushing, low blood pressure, confusion and breathing difficulty. Carcinoid crisis can occur in people with carcinoid tumors when they are exposed to certain triggers, including anesthetic used during surgery. Carcinoid crisis can be fatal. Your doctor may give you medications before surgery to reduce the risk of carcinoid crisis.


Carcinoid syndrome causes problems with the heart valves, making it difficult for them to function properly. As a result, the heart valves may leak. Signs and symptoms of carcinoid heart disease include fatigue and shortness of breath. Carcinoid heart disease can eventually lead to heart failure.


Prevention

At this time, there is no known way to prevent gastrointestinal carcinoid tumors. Since smoking might increase the risk of carcinoid tumors of the small intestine, not starting or quitting smoking may reduce the risk for this disease.