Carcinoid Tumors

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Questions to Ask Your Doctor About Your Cancer
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Diagnosis

Diagnosis of carcinoid tumors involves urine and blood tests, imaging tests (e.g., x-ray, CT scan, MRI scan), and may involve exploratory surgery. In some cases, physicians administer a drug (e.g., calcium gluconate, pentagastrin, alcohol) to provoke flushing and help in the diagnosis. These drugs are administered under careful observation in a hospital because they may cause serious side effects.

When a carcinoid is suspected, the levels of serotonin or its by-products (usually 5-hydroxyindoleacetic acid [5-HIAA]) in the patient's urine are measured over a 24-hour period. High levels of other peptides (e.g., neuropeptide, neurotensin, secretin) may indicate a carcinoid tumor as well. Blood tests also may be used to measure the plasma level of these substances.

Serotonin-rich foods (e.g., bananas, avocados, pineapple, eggplant, walnuts) can interfere with the results of this test. Three days prior to undergoing the test, the patient should eliminate these foods from the diet. Drugs such as guaifenesin (present in cough medicines), acetaminophen (e.g., Tylenol®), methocarbamol (muscle relaxant), and phenothiazines (tranquilizers) also can interfere with test results and should be discontinued prior to testing.

Imaging tests used to diagnose carcinoid tumors depend on the location of the tumor and whether or not the tumor has spread (metastasized). Tests include x-rays, computed tomography (CT scan), magnetic resonance imaging (MRI scan), and positron-emission tomography (PET scan).

CT scan uses x-rays to produce detailed images of abdominal organs and lymph nodes. In some cases, a contrast agent (dye) is used to detect metastasis. MRI scan uses magnetic fields to produce a cross-sectional image and detect tumors. PET scan is used to detect cell growth by producing detailed images of physical and chemical changes in the body.

Colonoscopy and upper and lower (barium enema) GI series may be used to detect carcinoid tumors of the GI tract. In colonoscopy, the physician inserts a very thin tube containing a light and a lens (called a colonoscope) into the rectum and colon to check for tumors. Small instruments can be inserted through the colonoscope to remove tissue for testing (called biopsy).

In an upper GI series (also called a barium swallow), the patient swallows a solution containing the mineral barium and x-rays of the upper tract (e.g., esophagus, stomach) are taken. In a lower GI series (also called a barium enema), the patient receives an enema with a solution containing barium and x-rays of the lower tract (e.g., intestines) are taken.

Because most carcinoids have receptors for the hormone somatostatin, angiography and radionuclide scan can be used to detect approximately 90% of tumors. These tests involve injecting a radioactive form of somatostatin into the bloodstream and using nuclear scanning to locate the tumor and determine its growth.

Exploratory surgery of the abdomen may be performed to locate a carcinoid tumor and determine its size.

Treatment

Surgical removal (resection) of the tumor is the preferred method of treatment for carcinoid tumors. If the entire tumor cannot be removed, treatment may include removing as much of the tumor as possible (debulking), chemotherapy, biological therapy, and radiation therapy. In cases of disease that has spread (i.e., metastatic disease), chemotherapy and/or radiation may be used to relieve symptoms (called palliative treatment).

Surgery
Surgery to treat carcinoid tumors depends on the size and location of the tumor and whether or not the tumor has metastasized. Surgical options may include removal of the tumor (local excision), removal of the tumor and part of the organ (resection), removal of the tumor using electric current (fulguration), or removal of the tumor by freezing the tissue (cryosurgery).

Surgical procedures include the following:

• Removal of all or part of the stomach (gastrectomy)
• Removal of the appendix and part of the colon (hemicolectomy)
• Removal of part of the colon (partial cecectomy)
• Removal of part of the liver (partial hepatectomy)
• Removal of the appendix (simple appendectomy)

Carcinoid tumors that have metastasized to the liver may be treated using hepatic artery ligation or hepatic artery embolization. Hepatic artery ligation involves cutting and tying off the main artery that supplies blood to the liver (i.e., the hepatic artery) to slow tumor growth. Hepatic artery embolization uses drugs or radiation to reduce or block the flow of blood to the liver and destroy cancer cells.

Palliative Treatment
Treatment used to relieve symptoms (palliative treatment) for carcinoid tumors may include chemotherapy, biological therapy, and radiation.

Chemotherapy involves using drugs to destroy cancer cells. Chemotherapy drugs may be used alone, or in combination, and are usually administered through an IV (intravenously). Chemotherapy relieves symptoms in less than 30% of cases of metastatic carcinoid tumors, usually for less than 1 year. Drugs that may be used include the following:

• Cisplatin (Platinol®)
• Dacarbazine (DTIC®)
• Doxorubicin (Doxil®)
• Etoposide (Vepesid®, Toposar®)
• 5-fluorouracil (Adrucil®, Efudex®)

Chemotherapy may cause severe side effects such as fatigue, hair loss, nausea vomiting, and low blood counts (e.g., anemia, neutropenia, thrombocytopenia).

Biological therapy, also called immunotherapy, involves using the body’s immune system to fight cancer. It reduces the size of carcinoid tumors in as many as 17% of patients and may slow the growth of metastatic tumors and prolong survival. Biological agents that are used include somatostatin analogues (e.g., octreotide [Sandostatin®], lanreotide) and interferon (Roferon®).

Biological agents usually are administered via injection and side effects include cough, headache, low blood count (e.g., anemia, neutropenia), and weakness.

Radiation therapy involves using high-energy x-rays to destroy cancer cells and shrink tumors. Radiation may come from a machine outside the body (called external beam radiation) or from radioactive "seeds" placed into the body (called brachytherapy or internal radiation therapy).

Side effects of radiation include fatigue, swelling (edema), and skin irritations.

Other types of palliative treatment include the following:

• Antidiarrheal agents (e.g., loperamide [Imodium®], diphenoxylate [Motofen®, Lomotil®])
• Avoiding situations and substances that provoke flushing (e.g., stress, alcohol)
• Diuretics (to treat symptoms of heart damage)
• Oral bronchodilators (to treat wheezing)
• Serotonin receptor antagonists (to treat persistent symptoms of carcinoid syndrome)

Prognosis

Most carcinoids grow slowly. The 5-year survival rate following surgical removal (resection) of a carcinoid tumor that has not metastasized is 70—90%. In approximately 45% of patients, metastases are present at the time of diagnosis. Patients with metastatic disease often live 10—15 years. Carcinoid tumors that metastasize to the liver have a poor prognosis.

New on the Horizon

All advances in the treatment of malignant tumors come from laboratory research. This information is then translated into treatments appropriate for clinical trials. Such advancements can only be made with the voluntary participation of patients in clinical trials, however.

New and emerging treatments for carcinoid syndrome include therapy directed against cellular receptors, cellular enzymes, cellular antigens, and genes. Patients diagnosed with carcinoid syndrome can ask their physician about available research protocols and inquire into the possibility of participating in experimental treatment.

Expert advice and assistance may be obtained by contacting Dr. Lowell Anthony, M.D., Director of Gastroenterology and Neuroendocrine Oncology at Louisiana State University. Dr. Anthony has devoted his career to the understanding and treatment of carcinoid tumors and malignant carcinoid syndrome.

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