Experts at Brown Cancer Center provide exemplary, customized carcinoid tumor care every step of the way. As one of the region’s largest cancer centers, Brown Cancer Center sees more patients with this complex type of cancer than most others, which translates into more successful outcomes for carcinoid tumors.
Nearly 12,000 people in the United States are diagnosed each year with carcinoid tumors, which also are called neuroendocrine tumors. The number of people with carcinoid tumors has been increasing in recent years. This probably is because of the increase of endoscopy and CT scans to screen for or diagnose other issues.
This slow-growing type of cancer can start in any part of the body that has neuroendocrine cells.
The average age of people diagnosed with carcinoid tumors is the early 60s. Carcinoid tumors are more common in African-Americans than in Caucasians. They are slightly more common in women than men.
Risk factors
Anything that increases your chance of getting a carcinoid tumor is a risk factor. These include:
- Conditions that affect how the stomach makes digestive juices, including atrophic gastritis, pernicious anemia or Zollinger-Ellison syndrome.
- Family history of a rare syndrome called multiple endocrine neoplasia 1 (MEN1).
- Race: African-Americans are more likely to develop carcinoid tumors in certain areas of the body than Caucasians.
- Gender: Carcinoid tumors are slightly more common in women.
- Smoking tobacco
Not everyone with risk factors gets carcinoid tumors. However, if you have risk factors, you should discuss them with your doctor.
Symptoms
Most cancers have the same symptoms as other, less serious conditions. Still, it’s important to know the signs.
Carcinoid tumors usually do not have symptoms in the early stages. If you have symptoms, they may include:
- Abdominal pain
- Intestinal blockage, which cause nausea/vomiting, abdominal pain and change in bowel habits
- Intestinal bleeding, which may cause blood in the stool or black, tarry stool
- Anemia and fatigue
Carcinoid syndrome
In about 1 percent of cases, carcinoid tumors can spread to the liver. This may cause a group of symptoms called carcinoid syndrome.
Symptoms of carcinoid syndrome usually are vague. It may be diagnosed first as other similar and more common conditions. Symptoms may not be present all the time. They may be brought on or made worse by certain foods, activities or medical procedures.
Signs of carcinoid syndrome may include:
- Flushing (redness or warm feeling) of the face and neck
- Diarrhea
- Shortness of breath, fast heartbeat, tiredness or swelling of the feet and ankles
- Pain or full feeling in the abdomen
- Difficulty breathing or wheezing
These symptoms do not always mean you have a carcinoid tumor. However, it is important to discuss any symptoms with your doctor, since they may signal other health problems.
Diagnosis
Blood tests, imaging exams and even surgical procedures are used to check for cancer.
Your doctor will ask questions about your general health, your medical history and your family history and your smoking habits.
Tests to diagnose a carcinoid tumor, or to find out if it has spread may include one or more of the following. These tests also may be used to find out if treatment is working.
Blood and urine tests, which may include:
- Complete blood count (CBC)
- Blood chemistry studies to check certain substances in the blood
- 24-hour urine collection for 5-HIAA (metabolite of serotonin)
- Measurement of serotonin levels in blood
Imaging tests, which may include:
- Colonoscopy
- Esophagogastroduodenoscopy (upper endoscopy)
- Endoscopic ultrasound (EUS)
- CT or CAT (computed axial tomography) scans, including high-resolution CT scans
- MRI (magnetic resonance imaging) scans
- Octreotide scan or somatostatin receptor scintigraphy (OctreoScan™): This is the most common imaging test to diagnose carcinoid tumors. Octreotide, a radioactive substance, is injected into a vein. As it travels through the body, it attaches to carcinoid tumor cells that have somatostatin receptors. A radiation-measuring instrument shows where the octreotide has collected.
- Angiogram: In this test to examine blood vessels and flow, a dye is injected into a blood vessel. X-rays are taken as the dye moves through the vessel.
- Barium swallow or upper gastrointestinal (GI) series: You swallow a radioactive substance. X-rays are taken as it moves through the body.
- Barium enema or lower GI series: You are given an enema that contains sent into the colon to help spread the barium to get a more accurate image.
- Enteroclysis: A tiny tube is inserted through the mouth or nose into the small intestine. Barium contrast, as well as a substance that makes air in the intestines and causes them to expand, is put through the tube. X-rays are taken.
- I-131 MIBG scan: A chemical called MIBG with radioactive iodine is injected into a vein. As it moves through the body, images are taken with a special camera.
- Capsule endoscopy: You swallow a capsule with a light and tiny camera. As the capsule moves through the digestive system, it takes thousands of photographs. The doctor looks at the images on a computer.
- Double balloon enteroscopy: This test uses a special endoscope that is one tiny tube inside another. The doctor can biopsy any abnormal areas.
- Proctoscopy: A hollow tube (proctoscope) with a light on the end of it is inserted into the anus. The doctor can view the lining of the rectum and anus.
Biopsy: Methods to biopsy carcinoid tumors include:
- Needle biopsy: A needle is inserted into the tumor. An ultrasound or CT scan may be used to guide the needle.
- Surgery: A tissue sample is taken from the abdomen during surgery
Carcinoid tumor staging
If you are diagnosed with a carcinoid tumor, your doctor will determine the stage of the disease. Staging is a way of classifying cancer by how much disease is in the body when it is diagnosed. This helps the doctor plan the best way to treat the cancer.
Once the staging classification is determined, it stays the same even if treatment is successful or the cancer spreads.
Carcinoid tumor stages
Localized: Cancer is found only in the appendix, colon, rectum, small intestine, and/or stomach.
Regional: Cancer has spread from the appendix, colon, rectum, stomach, and/or small intestine to nearby tissues or lymph nodes.
Metastatic: Cancer has spread to other parts of the body.
Treatment
Common cancer treatments include chemotherapy, radiation treatment and surgery. Doctors select the treatment for carcinoid tumors based on your diagnosis and disease stage.
At Brown Cancer Center, your treatment for carcinoid tumor is personalized especially for you. A team of experts including oncologists, surgical oncologists, pathologists and others collaborate and communicate about your options before and during your therapy. Most carcinoid tumors grow slowly, and they often can be treated successfully.
If you are diagnosed with a carcinoid tumor, your doctor will discuss the best options to treat it. This depends on several factors, including:
- The tumor’s size and where it is located
- If the cancer has spread
- Your general health
- Your symptoms
Surgery is often the best option for small carcinoid tumors that have not spread. Chemotherapy and radiation therapy may be used to shrink tumors, although they often may not be successful.
One or more of the following therapies may be recommended to treat the cancer or help relieve symptoms.
Surgery is the most common procedure to treat carcinoid tumors. It may be used to treat the primary tumor and nearby lymph nodes where the cancer has spread. Surgery also may be done if the cancer has spread to the liver. Surgical removal of the tumor may help with carcinoid syndrome symptoms. Your doctor may suggest one of these types of surgery to treat a carcinoid tumor.
Bowel and colorectal resection: Removal of the intestine and lymph nodes near the primary carcinoid tumor(s). Lymph nodes along the vessels that supply the affected intestine (called the mesentery) are removed. Removal of the mesentery is at least as important as removing the primary tumor. This requires advanced imaging and surgical techniques to assure complete removal of cancer and preservation of good intestinal function.
Liver resection: Significant experience is needed to determine if liver surgery can and should be performed for carcinoid tumors since most are in both sides of the liver. Advanced planning and surgical techniques are important to ensure surgery is done only if you will benefit from it. This expertise also benefits many patients who would not be considered for surgery if standard approaches were used.
Appendectomy: Removal of the appendix, a common site of carcinoid tumors.
Radiofrequency ablation and cryoablation: These methods to destroy carcinoid tumors in the liver do not require surgical removal of the tumor (resection). They often are not as successful as surgery, but they may be helpful for some patients. Radiofrequency uses radio waves to heat tumors; cryoablation uses cold to freeze tumors. Each has advantages and disadvantages, and your doctor will decide if you can benefit from these treatments.
Radioembolization with Yittrium-90: This method involves injecting a solution containing tiny radiation beads into carcinoid tumors in the liver through an artery in the groin. This selectively kills the tumors that have spread to the liver and allows patients to avoid or delay taking more toxic treatments. Our interventional radiology experts who do this procedure are among the best in the nation.
Radiation therapy usually is not used to treat carcinoid tumors. It may help people who cannot have surgery, and it may help relieve pain if the cancer has spread.
Chemotherapy is not an effective treatment for carcinoid tumors in the bowel. However, it may be used for neuroendocrine tumors starting in the pancreas or aggressive fast-growing neuroendocrine tumors.
Targeted therapies: These innovative new drugs stop the growth of cancer cells by interfering with certain proteins and receptors.
Octreotide: This drug, which is given by injection, contains a substance similar to the hormone somatostatin. A long-acting version can be given once a month. Lanreotide is a similar drug. Although octreotide usually does not shrink carcinoid tumors, it may slow their growth and help relieve symptoms. Side effects may include insulin resistance.
Interferon: These natural substances activate the body’s immune system and sometimes slow the growth of carcinoid tumor cells.
