Localized disease

A tumor is called localized when it is found only in the organ where it started. Treatment of localized carcinoid tumors is based mostly on their size. Experts sometimes disagree on the exact size for making treatment decisions, and there are some sizes where it has not been determined exactly what treatment is best.

Stomach

Small carcinoid tumors of the stomach can often be completely removed through an endoscope. For those larger than 1 centimeter (slightly less than ½ inch), an incision in the abdomen may be needed to remove the tumor and some surrounding stomach tissue.

Small intestine

Local excision is the usual treatment for carcinoid tumors in the small intestine that are smaller than 1 centimeter (slightly less than ½ inch). Surgery for larger tumors takes more surrounding tissue, as well as some surrounding blood vessels and lymph nodes.

Large intestine (other than appendix and rectum)

The usual treatment is local excision. If the carcinoid tumor is smaller than 1 centimeter (slightly less than ½ inch), excision can often be done through a colonoscope. If the tumor is larger, surgery is usually done through an incision in the skin.

Appendix

Nearly all cancer specialists agree that an appendectomy (surgical removal of the appendix) is the only treatment needed for carcinoid tumors that are smaller than 1.5 centimeters (slightly larger than ½ inch).

For tumors between 1.5 and 2 centimeters, most doctors believe that removing the appendix is all that is needed. But they also consider other factors, such as the patient's age, general health, and the patient's degree of worry about the possibility of the cancer coming back, to determine whether more treatment is needed.

Most specialists agree that more extensive surgery should be considered for tumors larger than 2 centimeters (about ¾ inch). For these tumors, removal of about a third of the colon next to the appendix (a hemicolectomy), along with nearby blood vessels and lymph nodes is an option for patients younger than 60 who are otherwise in good health. Because carcinoid tumors grow and spread slowly, people older than 60 or with other serious health problems (especially if these problems make surgery more risky) are not likely to benefit from more extensive surgery.

Rectum

Rectal carcinoid tumors that are smaller than 1 centimeter (slightly less than ½ inch) are usually treated by fulguration (destroying the cancer by burning it with an electrical current) or local excision.

For rectal carcinoid tumors between 1 and 2 centimeters, the best approach is often determined by how deeply the carcinoid tumor invades the wall of the rectum, as well as other details of each patient's medical situation. Deeply invasive tumors are often treated the same as larger tumors. Less invasive tumors may be treated by local excision. If local excision is used, careful follow-up to check for recurrence is needed.

Carcinoid tumors larger than 2 centimeters (about ¾ inch) have a higher risk of aggressive growth and spread, so they are removed by the same operations used for adenocarcinomas (the usual type of rectal cancer). This involves a low anterior resection if the carcinoid is in the upper part of the rectum. If the lower part is involved, abdominoperineal (AP) resection and colostomy are used. But this is a very complex area. Because many of these tumors will have already spread, it is not clear how much benefit such an extensive operation provides.

Regional spread

If possible, the primary (main) tumor and any spread to nearby tissues and lymph nodes should all be removed by surgery. If this can't be done, surgery should remove as much cancer as possible without causing severe side effects. Surgery should also be done to relieve symptoms such as intestinal blockage caused by the local growth of cancer. For example, surgery to redirect the flow of feces around a blocked area of intestine can be done by connecting adjacent areas of the intestine.

Distant spread

Cure in this situation is not usually possible, although treatment is not always needed right away, depending on how quickly the tumors are growing. The goal of surgery in this situation is to relieve symptoms and slow the course of the disease. For example, removing or bypassing areas blocked by cancer growth can relieve some symptoms. If distant metastases are not causing symptoms, treatment may not be needed, although chemotherapy or interferon-alfa may help delay the onset of symptoms in some patients.

If carcinoid syndrome is causing bothersome symptoms, treatment options include chemotherapy, immunotherapy, treatment with octreotide, or removing the metastatic tumors. If metastatic tumors cannot be removed by surgery without causing severe side effects, ablative methods can be used to destroy as much of the tumors as possible. These methods, used mostly for liver metastases, include chemoembolization, radiofrequency ablation, cryotherapy, and alcohol injection. Patients should also be advised to avoid alcoholic drinks, stress, strenuous exercise, spicy foods, and certain medicines that can make the symptoms of carcinoid syndrome worse.

Recurrent carcinoid tumors

Cancer is called recurrent when it come backs after treatment. Recurrence can be local (in or near the same place it started) or distant (spread to organs such as the lungs or bone). Patients with recurrent carcinoid tumors are treated with surgery to remove all signs of tumor whenever possible. This provides the best chance for a good long-term outcome. If surgery is not possible, the treatments described above for tumors that have spread to distant sites may be helpful.

Carcinoid heart disease

The substances released into the blood by some carcinoid tumors can damage the heart. Early symptoms are fatigue and shortness of breath. Eventually, patients get fluid in their legs and even their abdomen. The major cause is damage to the valves of the heart. Doctors can usually make the diagnosis by listening to the heart and by an ultrasound exam of the heart called an echocardiogram.

The main treatment is with octreotide to block the cancer’s secretion of the toxic substances. Drugs (diuretics) to strengthen the heart beat and to get rid of fluid can also be helpful. In some instances, heart surgery may be needed to replace the damaged valves.

Last Medical Review: 06/19/2009
Last Revised: 06/19/2009