Treatment of gastrointestinal carcinoid tumors by stage

Localized disease

A tumor is 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

Treatment of carcinoid tumors of the stomach depends on the level of a hormone called gastrin that is measured before surgery. Certain conditions linked to high gastrin levels are also linked to getting many carcinoid tumors of the stomach. Patients with these conditions who have stomach carcinoid tumors are treated differently from patients without these conditions.

For patients with high gastrin levels, small carcinoid tumors of the stomach are often treated by removing them completely through an endoscope. The other option is to watch the tumors closely (by doing repeat endoscopy), and only removing them if they start growing. For tumors larger than 2 cm (slightly less than an inch), an incision in the abdomen may be needed to remove the tumor and some surrounding stomach tissue.

For patients who don’t have high gastrin levels, more extensive surgery is needed. Often, a piece of the stomach is removed (a partial gastrectomy) along with nearby lymph nodes.

Small intestine

Some small tumors in the duodenum (the first part of the small intestine) can be removed through the endoscope (endoscopic resection). Otherwise, treatment is the same as for other small intestine carcinoid tumors: local excision for small tumors and small bowel resection (removal of a piece of intestine as well as some surrounding blood vessels and lymph nodes) for larger tumors.

Large intestine (other than appendix and rectum)

The usual treatment is hemicolectomy (removal of a section of colon along with nearby lymph nodes and blood vessels). Because many patients have more than one carcinoid tumor, the surgeon will often check the rest of the colon for other tumors during surgery. For very small tumors, sometimes the tumor can be removed without surgery using a colonoscope.

Appendix

Most often, an appendectomy (surgical removal of the appendix) is the only treatment needed for carcinoid tumors that are 2 cm (a little less than an inch) or smaller. Still, 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, might also be used to determine whether more treatment is needed.

Tumors larger than 2 cm are more likely to have already spread to nearby tissues and lymph nodes, so for these tumors more extensive surgery is usually recommended. This means removal of about a third of the colon next to the appendix (a hemicolectomy), along with nearby blood vessels and lymph nodes. This procedure may not be recommended for patients who are older or have other serious health problems (especially if these problems make surgery more risky), because the benefit may not outweigh the risks.

Rectum

Rectal carcinoid tumors that are smaller than 2 cm (slightly less than an inch) can be removed through an endoscope or local excision through the anus.

For rectal carcinoid tumors between 1 and 2 cm, the best approach depends on how deeply the carcinoid tumor has grown into the wall of the rectum, as well as other details of each patient's medical situation. If the tumor has grown into the thick muscle layer of the rectum (the muscularis propria) or deeper, it needs to be treated like it is a larger tumor.

Carcinoid tumors larger than 2 cm (and those that have grown deep into the wall of the rectum) have a higher risk of growing and spreading, so they are removed by the same operations used for adenocarcinomas (the usual type of rectal cancer). This operation is 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.

Regional spread

Regional spread means that the cancer has either spread to nearby lymph nodes or it has grown through the wall of the organ where it started to invade nearby tissues such as fat, ligaments, and muscle.

If possible, the primary (main) tumor and any areas of cancer spread should all be removed by surgery. Nearby lymph nodes should be removed and checked for signs of cancer spread. This provides the best chance of cure. 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 stool around a blocked area of intestine can be done by connecting adjacent areas of the intestine.

Distant spread

A 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 symptoms in some patients.

If carcinoid syndrome is causing bothersome symptoms, treatment options include chemotherapy, immunotherapy, treatment with octreotide (Sandostatin), 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, radioembolization, 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 in the “Distant spread” section 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 heartbeat and to get rid of fluid can also help. In some instances, heart surgery may be needed to replace the damaged valves.