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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. These cells receive signals from the nervous system and then release hormones into the blood. Carcinoid tumors are most common in the gastrointestinal (GI) tract and the lungs.
The average age of people diagnosed with carcinoid tumors is the early 60s. Carcinoid tumors are more common in African-Americans than in whites. They are slightly more common in women than men. A GI carcinoid tumor makes you more likely to have other digestive system cancers.
Carcinoid Tumor 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 whites
- 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.
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
In about 1% 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.
Carcinoid syndrome symptoms may include:
- Flushing (redness or warm feeling) of the face and neck
- 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.
If your doctor suspects you have a carcinoid tumor or if one has been found during another procedure, the first step will be a thorough physical exam and medical history. 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 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:
- Esophagastrodoudenoscopy (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 body.
- Barium enema or lower GI series: You are given an enema that contains barium. X-rays are taken as it spreads into the colon. After the enema, air may be 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 though 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. Ultrasound or CT scan may be used to guide the needle.
- Surgery: A tissue sample is taken from the abdomen during surgery
Carcinoid tumor stages
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. The stages for carcinoid tumors are:
- 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.
(source: National Cancer Institute)
At MD Anderson, 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.
Our Carcinoid Tumor Treatments
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
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 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.
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: MD Anderson is among just a few cancer centers in the nation that are able to offer targeted therapies for some types of carcinoid tumors. These innovative new drugs stop the growth of cancer cells by interfering with certain proteins and receptors or blood vessels that supply the tumor with what it needs to grow.
Octreotide: This drug, which is given by injection, contains a substance similar to the hormone somatostain. 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.
Why choose MD Anderson for carcinoid tumor treatment?
MD Anderson’s Gastrointestinal Center gives you exemplary, customized carcinoid tumor care every step of the way. As one of the nation’s largest cancer centers, MD Anderson sees more patients with this complex type of cancer than most others, which translates into more successful outcomes for carcinoid tumors.
As one of the world’s largest cancer research centers, MD Anderson is leading the investigation into new methods of carcinoid tumor diagnosis and treatment. For instance, our researchers are studying carcinoid tumors on a molecular basis to try to find a cause.
Our status as a premier research center means we can offer clinical trials (research studies) of new treatments for carcinoid tumors. MD Anderson researchers have led the development of novel treatment strategies, including targeting of mTOR (a protein) and angiogenesis (growth of new blood vessels) in neuroendocrine tumors (including carcinoid tumors). MD Anderson researchers were involved from proof-of-concept single institution phase II to multi-national phase III studies.
I labeled the daily emails from our March trip to MD Anderson for my brother’s stem cell transplant “our adventure,” and that’s exactly what it turned out to be.
I was excited to be a stem cell donor for my brother, Dan, who has leukemia. But when the doctors performed a chest x-ray, they discovered a questionable spot on my lung. We hoped it was nothing, but scheduled a CT scan to make sure.
The scan revealed a tumor on my lung. I cried to my sister as I told her something was wrong.
“We’ve been here for a week, and you are the first person we have seen cry,” she said to me, telling me to remain calm, be positive and toughen up.
“All these people knew they had cancer, and now I might have cancer,” I responded.
Neuroendocrine tumor diagnosed by chance
Eleven days after I arrived in Houston, I was told I had a carcinoid tumor in my lung. This is so not happening to me, I thought. I had to tell Dan I couldn’t be his donor.
My husband arrived the next day, and the next two weeks we did follow up scans and test to make sure the tumor hadn’t metastasized.
This trip did not turn out the way it was supposed to. Or did it?
Carcinoid tumors, also called neuroendocrine tumors, are rare and slow growing. Between 11,000 and 12,000 new cases are diagnosed each year.
From talking to other lungnoids -- yes, that is what we call ourselves -- I’ve learned that a lot of us were diagnosed by chance. Many other lungnoids told me they were misdiagnosed for years since many doctors are not familiar with this type of cancer.
My carcinoid tumor surgery
My tumor was low grade and small. I was referred to James C. Yao, M.D. The best carcinoid tumor treatment option for me was surgery, but I had time to make a decision on where to have it. Should I have the surgery back in Florida or someplace else? My husband and I talked about it and decided to have the surgery at MD Anderson.
My surgery went well, and my blood work was good. I didn’t have to do chemo or radiation, and the chance of this cancer coming back is very slim because it was caught so early.
A few months later my brother Dan had his transplant. The stem cells came from my oldest sister, Molly. Dan was on the 17th floor while I was on the seventh floor. Keeping things in the family took on a new meaning.
I went to MD Anderson to save my brother’s life, but in the end, he saved mine.