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Small intestine cancer

                                                                                             Description
                                              KEY TERMS                                       The small intestine is a long tube inside the
                                                                                         abdomen divided into three sections: the duodenum,
                          Autoimmune disease—A disease caused by the                     jejunum, and ileum. The function of the small intestine is
                          abnormal presence of antibodies against normal                 to break down food and to remove proteins, carbohy-
                          tissues of the body.                                           drates, fats, vitamins, and minerals. Obstruction of the
                                                                                         small intestine by cancer may impair normal passage and
                          Lymphocytes—White blood cells. Lymphocytes
                                                                                         digestion of food and nutrients.
                          play a vital role in the immune system.
                          Lymphoma—Any cancer of the tissues that make
                                                                                             Adenocarcinoma
                          up the lymphatic system.
                          Sicca symptoms—Dry mouth and eyes.                                  These malignancies most often start in the lining
                                                                                         of the small intestine, most frequently occurring in the
                                                                                         duodenum and jejunum, the sections closest to the
                                                                                         stomach. These tumors may obstruct the bowel, caus-
                         Iwamoto, Ryan R. “Xerostomia.” In Cancer Symptom Manage-        ing digestive problems. Adenocarcinoma is the most
                             ment, 2nd ed. Yarbro, Connie H., Margaret H. Frogge, and    common cancer of the small intestine, but only
                             Michelle Goodman, eds. Sudbury, MA: Jones and Bartlett
                                                                                         accounts for 2% of all tumors in the gastrointestinal
                             Publishers, 1999.
                                                                                         tract and 1% of all deaths related to cancer of the gas-
                         PERIODICALS                                                     trointestinal tract. Carcinomas of the small intestine
                         Bell, Mary, et al. “Sjögren’s Syndrome: A Critical Review of    may appear at multiple sites.
                              Clinical Management.” The Journal of Rheumatology 26,
                              no. 9 (2001): 2051-9.
                                                                                             Lymphoma
                         Daniels, Troy E. “Evaluation, Differential Diagnosis, and
                              Treatment of Xerostomia.” Current Opinion in Rheuma-            This fairly uncommon cancer is typically a non-
                              tology 27 (2001): 6-9.                                     Hodgkin’s type that starts in the lymph tissue of the
                         Fox, Robert I., and Paul Michelson. “Approaches to the Treat-   small intestine. (The body’s immune system is com-
                              ment of Sjögren’s Syndrome.” Current Opinion in
                                                                                         prised of lymph tissue, which assists in fighting infec-
                              Rheumatology 27 (2000): 15-20.
                                                                                         tions.) Malignant lymphoma is not often found as a soli-
                         Fox, Robert I. “Update in Sjögren Syndrome.” Current Opin-
                                                                                         tary lesion.
                              ion in Rheumatology 12 (2000): 391-8.
                         OTHER
                                                                                             Sarcoma
                         MGH Virtual Brain Tumor Center. 22 June 2001 <http://brain.
                                                                                              Sarcoma malignancies of the small intestine are usu-
                            mgh.harvard.edu>.
                                                                                         ally leiomyosarcoma. They most often occur in the
                                                                                         smooth muscle lining of the ileum, the last section of the
                                                            Belinda Rowland, Ph.D.
                                                                                         small intestine. Liposarcoma and angiosarcoma occur
                         Skin cancer see individual cancers: Basal                       more rarely in the small intestine.
                           cell carcinoma; Bowen’s disease;
                                                                                             Carcinoid tumors
                           Melanoma; Merkel cell carcinoma;
                           Squamous cell carcinoma of the skin,                               Carcinoid tumors are most often found in the ileum.
                           Trichilemmal carcinoma                                        In approximately 50% of cases, they appear in multiples.

                                                                                             Demographics
                                                                                             Approximately 50% of small intestine cancers are
                                                                                         adneocarcinomas; 20% are lymphomas; 20% are carci-
                                 Small intestine cancer                                  noid; and about 10% are sarcomas.
                             Definition
                                                                                             Causes and symptoms
                              Cancer of the small intestine is a rare disease that
                         results when abnormal, malignant cells divide out of con-            The causes of this cancer are not known, but factors
                         trol. Cancers in this location consist primarily of adeno-      that contribute to its development include exposure to
                         carcinoma, lymphoma, sarcoma, and carcinoid tumors.             carcinogens such as chemicals, radiation, and viruses. In


                         984                                                                              G A L E E N C Y C L O P E D I A O F CA N C E R
Small intestine cancer
addition, smoking and a poor diet may contribute to the
incidence of small intestine cancer. The incidence of can-
cer is higher in obese individuals.
    Often cancer of the small intestine does not initially
produce any symptoms. Gastrointestinal bleeding is per-
haps the most common symptom. A doctor should be
consulted if any of these symptoms are present:
• involuntary weight loss
• a lump in the abdominal region
• blood in the stool
• pain or cramping in the abdominal region

     Diagnosis
     Evaluation begins by taking a patient’s medical his-
tory and conducting a physical examination. If a patient
experiences symptoms, a doctor may suggest the follow-
ing tests:
• Upper gastrointestinal x ray/upper GI series: To allow
  the stomach to be seen easier on an x ray, the patient        Colored scanning electron micrograph (SEM) of cancer
                                                                cells in the intestine. (© Quest, Science Source/Photo
  drinks a liquid called barium. This test can be conduct-
                                                                Researchers, Inc. Reproduced by permission.)
  ed in either a doctor’s office or a radiology department
  at a hospital.
                                                                • Localized: The cancer has not spread beyond the wall
• CT scan (computed tomography): A computerized x                 of the organ it developed in.
  ray that takes a picture of the abdomen.
                                                                • Regional spread: The cancer has spread from the organ
• MRI scan (magnetic resonance imaging): A imaging
                                                                  it started in to other tissues such as muscle, fat, liga-
  technique that uses magnetic waves to take a picture of
                                                                  ments, or lymph nodes.
  the abdomen.
                                                                • Distant spread: The cancer has spread to tissues or
• Ultrasound: An imaging technique that uses sound
                                                                  organs outside of where it originated such as the liver,
  waves to locate tumors.
                                                                  bones, or lungs.
• Endoscopy: An endoscope is a thin, lighted tube which
                                                                     Treatment options for small intestine cancer most
  is placed down the throat to reach the first section of the
                                                                often include surgery, and possibly radiation therapy,
  small intestine (duodenum). During this procedure, the
                                                                chemotherapy, and/or biological therapy. Cancer of the
  doctor may take a biopsy, in which a small piece of tis-
                                                                small intestine is treatable and sometimes curable de-
  sue is removed for examination of cancereous cells
                                                                pending on the histology. Removing the cancer through
  under a microscope.
                                                                surgery is the most common treatment. If the tumor is
     If small intestine cancer is evident, more tests will      large, a small portion may be removed if resection of the
be conducted to determine if cancer has spread to other         small intestine is possible. For larger tumors, surgery
parts of the body.                                              requires removing a greater amount of the surrounding
                                                                normal intestinal tissue, in addition to some surrounding
     Treatment team                                             blood vessels and lymph nodes.
     Cancer treatment often requires a team of specialists           Radiation therapy kills cancer cells and reduces the
and may include a surgeon, medical oncologist, radiation        size of tumors through the use of high-energy x rays.
oncologist, nurse, physical therapist, occupational thera-      Radiation therapy may come from an external source
pist, dietitian, and or a social worker.                        using a machine or an internal source. Internal-based
                                                                therapy involves the use of radioisotopes to administer
     Clinical staging, treatments, and prognosis                radiation through thin plastic tubes to the area of the
                                                                body where cancer cells are found. Side effects of radia-
     As with many other types of cancer, malignancies of
                                                                tion therapy include:
the small intestine can be classified as localized, regional
                                                                • fatigue
spread, or distant spread.

G A L E E N C Y C L O P E D I A O F CA N C E R                                                                       985
Small intestine cancer
                         • loss of appetite (anorexia)                                       Treatment of lymphoma of the small intestine may
                                                                                         consist of:
                         • nausea and vomiting
                                                                                         • surgical removal of the cancer and lymph nodes in
                         • Diarrhea
                                                                                           close proximity to it
                         • gas
                                                                                         • Surgery accompanied by radiation therapy or adjuvant
                         • bloating                                                        chemotherapy. If the disease is localized to the bowel
                                                                                           wall, then surgical resection alone or combined
                         • mild temporary, sunburn-like skin changes
                                                                                           chemotherapy should be considered. If the disease has
                         • difficulty tolerating milk products                             extended to the regional lymph nodes, then surgical
                                                                                           resection and combination chemotherapy is suggested
                              Chemotherapy kills cancer cells with drugs taken
                                                                                           at the time of diagnosis.
                         orally or by injection in a vein or muscle. It is referred to
                         as a systemic treatment due to fact that it travels through     • For extensive lymphoma or lymphoma that cannot be
                         the bloodstream and kills cancer cells outside the small          removed surgically, chemotherapy with or without
                         intestine. Adjuvant chemotherapy may be given follow-             additional radiation therapy is frequently used to reduce
                         ing surgery to ensure all cancer cells are killed. Some           the risk of recurrence.
                         side effects of chemotherapy are:
                                                                                            Treatment of leiomyosarcoma of the small intestine
                         • nausea and vomiting                                           may consist of:
                         • loss of appetite                                              • surgical removal of the cancer
                         • temporary hair loss (alopecia)                                • When cancer cannot be removed by resection, surgical
                                                                                           bypass of the tumor is recommended to allow food to pass.
                         • mouth sores
                                                                                         • radiation therapy
                         • fatigue, as a result of a low red blood cell count
                                                                                         • For unresectable metastatic disease, surgery, radiation
                         • higher likelihood of infection or bleeding due to low
                                                                                           therapy, or chemotherapy is suggested in order to alle-
                           white blood cell counts and low blood platelets, respec-
                                                                                           viate symptoms.
                           tively
                                                                                         • For unresectable primary or metastatic disease, a clini-
                              Radiation and chemotherapy are seldom beneficial
                                                                                           cal trial evaluating the benefits of new anticancer drugs
                         in small intestinal cancers.
                                                                                           (chemotherapy) and biological therapy.
                              Utilizing the body’s immune system, biological ther-
                                                                                              For recurrent small intestine cancer, treatment may
                         apy stimulates the body to combat cancer. Natural mate-
                                                                                         consist of the following measures, if the cancer has
                         rials from the body or other laboratory-produced agents
                                                                                         returned to one area of the body only:
                         are designed to boost, guide, or restore the body’s ability
                                                                                         • surgical removal of the cancer
                         to fight disease.
                                                                                         • symptom relief using chemotherapy or radiation therapy
                              Treatment options for small interstine cancers are
                         based on the type of cells found—adenocarcinoma, lym-           • a clinical trial using radiation and drug therapy (with or
                         phoma, sarcoma, or carcinoid tumor—rather than the                without chemotherapy) to elicit greater sensitivity to
                         clinical staging system.                                          radiation using radiosensitizers
                            Treatment of adenocarcinoma of the small intestine                For recurrent metastatic adenocarcinoma or leiomy-
                         may consist of:                                                 osarcoma, there is no standard effective chemotherapy
                                                                                         treatment. Patients should be regarded as candidates for
                         • surgical removal of the tumor
                                                                                         clinical studies assessing new anticancer drugs or biolog-
                         • If the cancer cannot be removed by resection of the
                                                                                         ical agents.
                           small intestine, surgery may be performed to bypass the
                                                                                              For carcinoid tumors at least than 1 cm in size, surgi-
                           cancer to allow food to travel through the intestine.
                                                                                         cal removal of the tumor and surrounding tissue is possi-
                         • symptom relief with radiation therapy
                                                                                         ble. Carcinoid tumors often grow and spread slowly, there-
                         • chemotherapy or biological therapy in a clinical trial        fore, approximately half are found at an early or localized
                           setting                                                       stage. By the time of sugery, 80% of the tumors over 2 cm
                                                                                         in diameter have metastasized locally or to the liver.
                         • a clinical trial involving radiation and drug therapy
                           (with or without chemotherapy) to elicit greater sensi-            The prognosis or likelihood of recovery depends on
                           tivity to radiation using radiosensitizers                    the type of cancer, the overall health of the patient, and


                         986                                                                               G A L E E N C Y C L O P E D I A O F CA N C E R
Small intestine cancer
whether the cancer has spread to other regions or is only
                                                                                    KEY TERMS
localized in the small intestine. A cure depends on the
ability to remove the cancer completely with surgery.
Adenocarcinoma is most common in the duodenum,                  Adenocarcinoma—A cancer that starts in glandu-
however, patient survival is less likely for individuals        lar tissue.
with cancer is in this area compared with those patients
                                                                Angiosarcoma—A malignant tumor that develops
with tumors in the jejunum or ileum due to reduced rates
                                                                either from blood vessels or from lymphatic ves-
of surgery to remove cancer. Between 1985-1995, there
                                                                sels.
were 4,995 cases of adenocarcinoma of the small intes-
                                                                Carcinogen—A substance that causes cancer.
tine reported to the National Cancer Database. Of these
malignancies, 55% occurred in the duodenum, 13% in              Carcinoid—A tumor that develops from neuroen-
the ileum, 18% in the jejunum, and 14% were in unspeci-         docrine cells.
fied areas. The National Cancer Database reported a
                                                                Leiomyosarcoma—A cancerous tumor of smooth
median survival of 19.7 months for these patients with an
                                                                (involuntary) muscle tissue.
overall 5-year disease survival rate of 30.5%. For
                                                                Liposarcoma—A cancerous tumor of fat tissue.
resectable adenocarcinoma, the National Cancer Institute
reports an overall five-year survival rate of only 20%,         Lymphoma—A cancer of the lymphatic tissue.
whereas resectable leiomyosarcoma’s survival rate is
                                                                Malignant—Cancerous; a tumor or growth that
reported at approximately 50%. One study found the
                                                                often destroys surrounding tissue and spreads to
overall rate of metastatic spread of leiomyosarcoma
                                                                other parts of the body.
ranged from 24–50%; this cancer most often spread to
                                                                Metastasis—The spread of cancer from the origi-
the liver. Five-year survival in 705 patients with
                                                                nal site to other body parts.
leiomyosarcoma was reported at 28%. Surgery is the pre-
ferred treatment for smooth muscle tumors. Little benefit       Radiation therapy—Also called radiotherapy, it
was found for irradiation or chemotherapy, or for these         uses high-energy rays to kill cancer cells.
therapies combined. Patients over 75 years of age have a
                                                                Sarcoma—A malignant tumor of the soft tissue
significantly poorer survival rate than younger people. In
                                                                including fat, muscle, nerve, joint, blood vessel,
addition, patients with poorly differentiated tumors have
                                                                and deep skin tissues.
a poorer prognosis than those with moderately or well-
                                                                Staging—Performing exams and tests to learn the
differentiated tumors. Survival rate decreases with pro-
                                                                extent of the cancer within the body, especially
gression of disease by stage: localized 47.6%; regional
                                                                whether the disease has spread from the original
31%; distant 5.2%.
                                                                site to other parts of the body.

     Alternative and complementary therapies
     Bovine and shark cartilage is currently being
                                                               not take herbal remedies without consulting their physi-
explored in clinical trials for antitumor properties, but as
                                                               cians, particularly if they intend to combine the herbs
of mid-2001 there is not enough evidence to warrant its
                                                               with prescription drugs. Herb and drug combinations can
use. Some popular herbs that are purported to have thera-
                                                               sometimes result in toxic interactions.
peutic effects in cancer treatment include echinacea, gar-
lic, ginseng, and ginger. Laboratory studies have shown
that echinacea has the potential to control the growth of          Coping with cancer treatment
cancerous cells, but more studies are needed to confirm
                                                                    Pain is a common problem for people with some
efficacy in humans. In addition, dosage and toxicity lev-
                                                               types of cancer, especially when the cancer grows and
els still need to be established. Some studies suggest that
                                                               presses against other organs and nerves. Pain may also be
diets high in garlic reduce the risk of stomach,
                                                               a side effect of treatment. However, pain can generally be
esophageal, and colon cancers. There is still debate
                                                               relieved or reduced with prescription medicines or over-
regarding the best form of garlic to take—whole raw gar-
                                                               the-counter drugs as recommended by the doctor. Other
lic or garlic in tablet form; aged or fresh garlic; garlic
                                                               ways to reduce pain, such as relaxation exercises, may
with odor or “deodorized” garlic. Ginger is often recom-
                                                               also be useful. It is important for patients to report pain to
mended for its beneficial effects on the digestive system,
                                                               their doctors, so that steps can be taken to help relieve it.
but evidence has not confirmed efficacy in cancer treat-
ment. Ginseng in excessive amounts can be very toxic,              Depression may affect approximately 15–25% of
causing vomiting, bleeding, and death. Patients should         cancer patients, particularly if the prognosis for recovery

G A L E E N C Y C L O P E D I A O F CA N C E R                                                                         987
Small intestine cancer
                                                                                         can lead to cancer is called a carcinogen. Carcinogens
                                        QU E S T I O N S                                 include certain chemicals, certain types of radiation, and
                                    TO A S K T H E D O C TO R                            viruses. Asbestos is one substance that is suspected of con-
                                                                                         tributing to the development of small intestinal cancer.
                                                                                         Although the precise causes of cancer are not known, a
                          • Is there any evidence the cancer has spread?
                                                                                         variety of factors are known to contribute to the develop-
                          • What is the stage of the disease?
                                                                                         ment of cancer including tobacco smoke, and poor dietary
                          • What are my treatment choices?                               habits such as high-fat diet. Eating a diet rich in fruits and
                                                                                         vegetables and low in fat may reduce the likelihood of
                          • What new treatments are being studied?
                                                                                         cancer. Studies have demonstrated that individuals who
                          • Would a clinical trial be appropriate for me?
                                                                                         were protected from cancer ate a greater variety of foods
                          • What are the expected benefits of each kind of               and nutrients compared to those with cancer. Several
                            treatment?                                                   fruits, vitamins, and minerals were found particularly pro-
                                                                                         tective against intestinal cancer including vitamin B6,
                          • What are the risks and possible side effects of
                                                                                         folate, niacin, and iron. Some studies have linked eating
                            each treatment?
                                                                                         large amounts of salt-cured, salt-pickled, and smoked
                          • How often will I have treatments?
                                                                                         foods to cancers of the digestive system. Other studies
                          • How long will treatment last?                                have linked stomach cancers, specifically intestinal cancer,
                                                                                         to a lack of fruits, vegetables, and fiber in the diet. For pre-
                          • Will I have to change my normal activities?
                                                                                         vention of cancer, it is important to avoid carcinogens
                          • What is the treatment likely to cost?
                                                                                         (smoking, chemicals) and known risk factors, and to pur-
                          • Is infertility a side effect of cancer treatment?            sue a healthy lifestyle which includes moderate alcohol
                            Can anything be done about it?                               intake, regular exercise, a low-fat diet, and a diet rich in
                                                                                         fruits and vegetables. Modifying genetic predispositions
                          • What is my prognosis?
                                                                                         through risk factor reduction can also assist in prevention.

                                                                                              Special concerns
                         is poor. A number of antidepressant medications are
                                                                                              Due to the side effects of radiation and chemothera-
                         available from physicians to alleviate feelings of depres-
                                                                                         py, individuals must make a deliberate effort to eat as
                         sion. Counseling with a psychologist or psychiatrist also
                                                                                         nutritiously as possible. Those who experience pain, nau-
                         may help patients deal with depression.
                                                                                         sea, or diarrhea may want to discuss treatments options
                                                                                         with their doctor to ease these side effects.
                             Clinical trials
                                                                                              Eating well during cancer treatment means getting
                              As of 2001, Glivec (STI-571 or imatinib mesylate)          enough calories and protein to help prevent weight loss
                         is in clinical trials for treatment of gastrointestinal stro-   and maintain strength. Eating nutritiously may also help
                         mal tumors, as well as for leukemia and glioblastoma, a         an individual feel better.
                         type of brain tumor. An open trial (GIST trial SWOG-
                         S0033) led by Southwest Oncology Group will test those          Resources
                         individuals with metastatic or recurrent disease using
                                                                                         BOOKS
                         two doses of the drug.
                                                                                         Coit, D.G. “Cancer of the Small Intestine.” In Cancer: Princi-
                              Clinical trials may be suitable for patients suffering          ples and Practice of Oncology, edited by V.T. DeVita, Jr.,
                         from small intestine cancer. The principal investigator              S. Hellman, and S.A. Rosenberg. Philadelphia: Lippin-
                         should be contacted regarding participation in appropri-             cott-Raven Publishers, 1997, pp. 1128–1143.
                         ate trials. For information about cancer trials, patients       Kelsen, David, Bernard Levin, and Joel Tepper. Principles and
                                                                                              Practice of Gastrointestinal Oncology. Philadelphia: Lip-
                         can visit the National Cancer Institute web site at <http://
                                                                                              pincott Williams & Wilkins Publishers, 2001.
                         cancertrials.nci.nih.gov>.
                                                                                         “Small Intestine.” In American Joint Committee on Cancer:
                                                                                              AJCC Cancer Staging Manual. Philadelphia: Lippincott-
                             Prevention                                                       Raven Publishers, 1997, pp. 77–81.
                              Most people who develop cancer do not have inherit-        PERIODICALS
                         ed genetic abnormalities. Their genes have been damaged         Howe, J.R., et al. “The American College of Surgeons Com-
                         after birth by substances in their environment. A substance        mission on Cancer and the American Cancer Society.
                         that damages deoxyribonucleic acid (DNA) in a way that             Adenocarcinoma of the Small Bowel: Review of the


                         988                                                                                G A L E E N C Y C L O P E D I A O F CA N C E R
Smoking cessation
     National Cancer Data Base, 1985-1995.” Cancer                     • Withdrawal symptoms. Nicotine, the substance in tobac-
     86(1999): 2693-2706.                                                co that gives smokers a pleasurable feeling, is as addic-
                                                                         tive as heroin or cocaine. Withdrawal from nicotine may
ORGANIZATIONS
                                                                         produce depression, anger, fatigue, headaches, prob-
The National Cancer Institute (NCI). For information contact the
                                                                         lems with sleep or concentration, or increased appetite
     Public Inquiries Office: Building 31, Room 10A31, 31 Cen-
                                                                         for food. These symptoms usually start several hours
     ter Drive, MSC 2580, Betheseda, MD 20892-2580 USA.
                                                                         after the last cigarette. They may last for several days or
     (301) 435-3848 or 1-800-4-CANCER. <http://cancer.gov/
                                                                         several weeks.
     publications/> or <http://cancertrials.nci.nih.gov> or <http://
     cancernet.nci.nih.gov>.                                           • Weight gain. Many people, particularly women, gain
National Center for Complementary and Alternative Medicine               between two and 10 pounds after giving up smoking.
     (NCCAM), 31 Center Dr., Room #5B-58, Bethesda, MD                   This mild weight gain, however, is not nearly as great a
     20892-2182. (800) NIH-NCAM. Fax: (301) 495-4957.
                                                                         danger to health as continuing to smoke. Getting more
     <http://nccam.nih.gov>.
                                                                         exercise can help.
                                                                       • Stress. Many smokers started to smoke as a way to cope
                          Crystal Heather Kaczkowski, MSc.
                                                                         with stress and tension. Finding other methods—exer-
                                                                         cise, meditation, biofeedback, massage, and others, can
                                                                         reduce the temptation to smoke when stress arises.
                                                                       • Side effects of nicotine replacement products. Smokers
                                                                         who are using these products to help them quit may
         Smoking cessation                                               experience headaches, nausea, sore throat, or long-term
                                                                         dependence. Side effects can often be reduced or elimi-
     Definition
                                                                         nated by using a lower dosage of the product or switch-
     Smoking cessation is the medical term for quitting                  ing to another form of nicotine replacement.
smoking. It is a vital part of cancer prevention because
smoking is the single most preventable cause of death
                                                                           Treatments
from cancer. As early as 1982, the Surgeon General
reported that tobacco causes more cancer deaths in the                     Nicotine replacement therapy
United States than any other factor–30% of all cancer
                                                                            Nicotine replacement therapy gives the smoker a
deaths, including 87% of deaths from lung cancer.
                                                                       measured supply of nicotine without the other harmful
Although people think of smoking most often in connec-
                                                                       chemicals in tobacco. It reduces the physical craving for
tion with lung cancer, smoking is also associated with
                                                                       cigarettes so that the smoker can handle the psychologi-
cancers of the mouth, throat, voice box (larynx), esopha-
                                                                       cal aspects of quitting more effectively.
gus, pancreas, kidney, and bladder. Women who smoke
                                                                            As of 2001, the Food and Drug Administration
increase their risk of cancer of the cervix. Quitting smok-
                                                                       (FDA) had approved four forms of nicotine replacement
ing, however, significantly reduces the risk of cancer; 15
                                                                       therapy:
years after quitting, a former smoker’s risk is almost as
low as that of someone who has never smoked.                           • Transdermal patches. Patches, which are non-prescrip-
                                                                         tion items, supply measured doses of nicotine through
                                                                         the skin. The doses are lowered over a period of weeks,
     Description
                                                                         thus helping the smoker to reduce the need for nicotine
     Smoking cessation covers several different                          gradually.
approaches, ranging from medications and psychothera-
                                                                       • Nicotine gum. Nicotine gum provides a fast-acting nico-
py to special classes and programs. Smoking is a habit
                                                                         tine replacement that is absorbed through the mouth tis-
difficult to break because it involves many different
                                                                         sues. The smoker chews the gum slowly and then keeps
aspects of a person’s emotions and social life as well as
                                                                         it against the inside of the cheek for 20 to 30 minutes.
physical addiction to nicotine. Most people who quit
                                                                         The gum is also available without prescription.
smoking successfully use a combination of treatments or
                                                                       • Nasal spray. Nicotine nasal spray provides nicotine
techniques for quitting.
                                                                         through the tissues that line the nose. It acts much more
                                                                         rapidly than the patches or gum, but requires a doctor’s
     Special concerns                                                    prescription.
    People who are trying to quit smoking are often con-               • Inhalers. Nicotine inhalers are plastic tubes containing
cerned about:                                                            nicotine plugs. The plug gives off nicotine vapor when

G A L E E N C Y C L O P E D I A O F CA N C E R                                                                                989

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Small Intestine Cancer

  • 1. Small intestine cancer Description KEY TERMS The small intestine is a long tube inside the abdomen divided into three sections: the duodenum, Autoimmune disease—A disease caused by the jejunum, and ileum. The function of the small intestine is abnormal presence of antibodies against normal to break down food and to remove proteins, carbohy- tissues of the body. drates, fats, vitamins, and minerals. Obstruction of the small intestine by cancer may impair normal passage and Lymphocytes—White blood cells. Lymphocytes digestion of food and nutrients. play a vital role in the immune system. Lymphoma—Any cancer of the tissues that make Adenocarcinoma up the lymphatic system. Sicca symptoms—Dry mouth and eyes. These malignancies most often start in the lining of the small intestine, most frequently occurring in the duodenum and jejunum, the sections closest to the stomach. These tumors may obstruct the bowel, caus- Iwamoto, Ryan R. “Xerostomia.” In Cancer Symptom Manage- ing digestive problems. Adenocarcinoma is the most ment, 2nd ed. Yarbro, Connie H., Margaret H. Frogge, and common cancer of the small intestine, but only Michelle Goodman, eds. Sudbury, MA: Jones and Bartlett accounts for 2% of all tumors in the gastrointestinal Publishers, 1999. tract and 1% of all deaths related to cancer of the gas- PERIODICALS trointestinal tract. Carcinomas of the small intestine Bell, Mary, et al. “Sjögren’s Syndrome: A Critical Review of may appear at multiple sites. Clinical Management.” The Journal of Rheumatology 26, no. 9 (2001): 2051-9. Lymphoma Daniels, Troy E. “Evaluation, Differential Diagnosis, and Treatment of Xerostomia.” Current Opinion in Rheuma- This fairly uncommon cancer is typically a non- tology 27 (2001): 6-9. Hodgkin’s type that starts in the lymph tissue of the Fox, Robert I., and Paul Michelson. “Approaches to the Treat- small intestine. (The body’s immune system is com- ment of Sjögren’s Syndrome.” Current Opinion in prised of lymph tissue, which assists in fighting infec- Rheumatology 27 (2000): 15-20. tions.) Malignant lymphoma is not often found as a soli- Fox, Robert I. “Update in Sjögren Syndrome.” Current Opin- tary lesion. ion in Rheumatology 12 (2000): 391-8. OTHER Sarcoma MGH Virtual Brain Tumor Center. 22 June 2001 <http://brain. Sarcoma malignancies of the small intestine are usu- mgh.harvard.edu>. ally leiomyosarcoma. They most often occur in the smooth muscle lining of the ileum, the last section of the Belinda Rowland, Ph.D. small intestine. Liposarcoma and angiosarcoma occur Skin cancer see individual cancers: Basal more rarely in the small intestine. cell carcinoma; Bowen’s disease; Carcinoid tumors Melanoma; Merkel cell carcinoma; Squamous cell carcinoma of the skin, Carcinoid tumors are most often found in the ileum. Trichilemmal carcinoma In approximately 50% of cases, they appear in multiples. Demographics Approximately 50% of small intestine cancers are adneocarcinomas; 20% are lymphomas; 20% are carci- Small intestine cancer noid; and about 10% are sarcomas. Definition Causes and symptoms Cancer of the small intestine is a rare disease that results when abnormal, malignant cells divide out of con- The causes of this cancer are not known, but factors trol. Cancers in this location consist primarily of adeno- that contribute to its development include exposure to carcinoma, lymphoma, sarcoma, and carcinoid tumors. carcinogens such as chemicals, radiation, and viruses. In 984 G A L E E N C Y C L O P E D I A O F CA N C E R
  • 2. Small intestine cancer addition, smoking and a poor diet may contribute to the incidence of small intestine cancer. The incidence of can- cer is higher in obese individuals. Often cancer of the small intestine does not initially produce any symptoms. Gastrointestinal bleeding is per- haps the most common symptom. A doctor should be consulted if any of these symptoms are present: • involuntary weight loss • a lump in the abdominal region • blood in the stool • pain or cramping in the abdominal region Diagnosis Evaluation begins by taking a patient’s medical his- tory and conducting a physical examination. If a patient experiences symptoms, a doctor may suggest the follow- ing tests: • Upper gastrointestinal x ray/upper GI series: To allow the stomach to be seen easier on an x ray, the patient Colored scanning electron micrograph (SEM) of cancer cells in the intestine. (© Quest, Science Source/Photo drinks a liquid called barium. This test can be conduct- Researchers, Inc. Reproduced by permission.) ed in either a doctor’s office or a radiology department at a hospital. • Localized: The cancer has not spread beyond the wall • CT scan (computed tomography): A computerized x of the organ it developed in. ray that takes a picture of the abdomen. • Regional spread: The cancer has spread from the organ • MRI scan (magnetic resonance imaging): A imaging it started in to other tissues such as muscle, fat, liga- technique that uses magnetic waves to take a picture of ments, or lymph nodes. the abdomen. • Distant spread: The cancer has spread to tissues or • Ultrasound: An imaging technique that uses sound organs outside of where it originated such as the liver, waves to locate tumors. bones, or lungs. • Endoscopy: An endoscope is a thin, lighted tube which Treatment options for small intestine cancer most is placed down the throat to reach the first section of the often include surgery, and possibly radiation therapy, small intestine (duodenum). During this procedure, the chemotherapy, and/or biological therapy. Cancer of the doctor may take a biopsy, in which a small piece of tis- small intestine is treatable and sometimes curable de- sue is removed for examination of cancereous cells pending on the histology. Removing the cancer through under a microscope. surgery is the most common treatment. If the tumor is If small intestine cancer is evident, more tests will large, a small portion may be removed if resection of the be conducted to determine if cancer has spread to other small intestine is possible. For larger tumors, surgery parts of the body. requires removing a greater amount of the surrounding normal intestinal tissue, in addition to some surrounding Treatment team blood vessels and lymph nodes. Cancer treatment often requires a team of specialists Radiation therapy kills cancer cells and reduces the and may include a surgeon, medical oncologist, radiation size of tumors through the use of high-energy x rays. oncologist, nurse, physical therapist, occupational thera- Radiation therapy may come from an external source pist, dietitian, and or a social worker. using a machine or an internal source. Internal-based therapy involves the use of radioisotopes to administer Clinical staging, treatments, and prognosis radiation through thin plastic tubes to the area of the body where cancer cells are found. Side effects of radia- As with many other types of cancer, malignancies of tion therapy include: the small intestine can be classified as localized, regional • fatigue spread, or distant spread. G A L E E N C Y C L O P E D I A O F CA N C E R 985
  • 3. Small intestine cancer • loss of appetite (anorexia) Treatment of lymphoma of the small intestine may consist of: • nausea and vomiting • surgical removal of the cancer and lymph nodes in • Diarrhea close proximity to it • gas • Surgery accompanied by radiation therapy or adjuvant • bloating chemotherapy. If the disease is localized to the bowel wall, then surgical resection alone or combined • mild temporary, sunburn-like skin changes chemotherapy should be considered. If the disease has • difficulty tolerating milk products extended to the regional lymph nodes, then surgical resection and combination chemotherapy is suggested Chemotherapy kills cancer cells with drugs taken at the time of diagnosis. orally or by injection in a vein or muscle. It is referred to as a systemic treatment due to fact that it travels through • For extensive lymphoma or lymphoma that cannot be the bloodstream and kills cancer cells outside the small removed surgically, chemotherapy with or without intestine. Adjuvant chemotherapy may be given follow- additional radiation therapy is frequently used to reduce ing surgery to ensure all cancer cells are killed. Some the risk of recurrence. side effects of chemotherapy are: Treatment of leiomyosarcoma of the small intestine • nausea and vomiting may consist of: • loss of appetite • surgical removal of the cancer • temporary hair loss (alopecia) • When cancer cannot be removed by resection, surgical bypass of the tumor is recommended to allow food to pass. • mouth sores • radiation therapy • fatigue, as a result of a low red blood cell count • For unresectable metastatic disease, surgery, radiation • higher likelihood of infection or bleeding due to low therapy, or chemotherapy is suggested in order to alle- white blood cell counts and low blood platelets, respec- viate symptoms. tively • For unresectable primary or metastatic disease, a clini- Radiation and chemotherapy are seldom beneficial cal trial evaluating the benefits of new anticancer drugs in small intestinal cancers. (chemotherapy) and biological therapy. Utilizing the body’s immune system, biological ther- For recurrent small intestine cancer, treatment may apy stimulates the body to combat cancer. Natural mate- consist of the following measures, if the cancer has rials from the body or other laboratory-produced agents returned to one area of the body only: are designed to boost, guide, or restore the body’s ability • surgical removal of the cancer to fight disease. • symptom relief using chemotherapy or radiation therapy Treatment options for small interstine cancers are based on the type of cells found—adenocarcinoma, lym- • a clinical trial using radiation and drug therapy (with or phoma, sarcoma, or carcinoid tumor—rather than the without chemotherapy) to elicit greater sensitivity to clinical staging system. radiation using radiosensitizers Treatment of adenocarcinoma of the small intestine For recurrent metastatic adenocarcinoma or leiomy- may consist of: osarcoma, there is no standard effective chemotherapy treatment. Patients should be regarded as candidates for • surgical removal of the tumor clinical studies assessing new anticancer drugs or biolog- • If the cancer cannot be removed by resection of the ical agents. small intestine, surgery may be performed to bypass the For carcinoid tumors at least than 1 cm in size, surgi- cancer to allow food to travel through the intestine. cal removal of the tumor and surrounding tissue is possi- • symptom relief with radiation therapy ble. Carcinoid tumors often grow and spread slowly, there- • chemotherapy or biological therapy in a clinical trial fore, approximately half are found at an early or localized setting stage. By the time of sugery, 80% of the tumors over 2 cm in diameter have metastasized locally or to the liver. • a clinical trial involving radiation and drug therapy (with or without chemotherapy) to elicit greater sensi- The prognosis or likelihood of recovery depends on tivity to radiation using radiosensitizers the type of cancer, the overall health of the patient, and 986 G A L E E N C Y C L O P E D I A O F CA N C E R
  • 4. Small intestine cancer whether the cancer has spread to other regions or is only KEY TERMS localized in the small intestine. A cure depends on the ability to remove the cancer completely with surgery. Adenocarcinoma is most common in the duodenum, Adenocarcinoma—A cancer that starts in glandu- however, patient survival is less likely for individuals lar tissue. with cancer is in this area compared with those patients Angiosarcoma—A malignant tumor that develops with tumors in the jejunum or ileum due to reduced rates either from blood vessels or from lymphatic ves- of surgery to remove cancer. Between 1985-1995, there sels. were 4,995 cases of adenocarcinoma of the small intes- Carcinogen—A substance that causes cancer. tine reported to the National Cancer Database. Of these malignancies, 55% occurred in the duodenum, 13% in Carcinoid—A tumor that develops from neuroen- the ileum, 18% in the jejunum, and 14% were in unspeci- docrine cells. fied areas. The National Cancer Database reported a Leiomyosarcoma—A cancerous tumor of smooth median survival of 19.7 months for these patients with an (involuntary) muscle tissue. overall 5-year disease survival rate of 30.5%. For Liposarcoma—A cancerous tumor of fat tissue. resectable adenocarcinoma, the National Cancer Institute reports an overall five-year survival rate of only 20%, Lymphoma—A cancer of the lymphatic tissue. whereas resectable leiomyosarcoma’s survival rate is Malignant—Cancerous; a tumor or growth that reported at approximately 50%. One study found the often destroys surrounding tissue and spreads to overall rate of metastatic spread of leiomyosarcoma other parts of the body. ranged from 24–50%; this cancer most often spread to Metastasis—The spread of cancer from the origi- the liver. Five-year survival in 705 patients with nal site to other body parts. leiomyosarcoma was reported at 28%. Surgery is the pre- ferred treatment for smooth muscle tumors. Little benefit Radiation therapy—Also called radiotherapy, it was found for irradiation or chemotherapy, or for these uses high-energy rays to kill cancer cells. therapies combined. Patients over 75 years of age have a Sarcoma—A malignant tumor of the soft tissue significantly poorer survival rate than younger people. In including fat, muscle, nerve, joint, blood vessel, addition, patients with poorly differentiated tumors have and deep skin tissues. a poorer prognosis than those with moderately or well- Staging—Performing exams and tests to learn the differentiated tumors. Survival rate decreases with pro- extent of the cancer within the body, especially gression of disease by stage: localized 47.6%; regional whether the disease has spread from the original 31%; distant 5.2%. site to other parts of the body. Alternative and complementary therapies Bovine and shark cartilage is currently being not take herbal remedies without consulting their physi- explored in clinical trials for antitumor properties, but as cians, particularly if they intend to combine the herbs of mid-2001 there is not enough evidence to warrant its with prescription drugs. Herb and drug combinations can use. Some popular herbs that are purported to have thera- sometimes result in toxic interactions. peutic effects in cancer treatment include echinacea, gar- lic, ginseng, and ginger. Laboratory studies have shown that echinacea has the potential to control the growth of Coping with cancer treatment cancerous cells, but more studies are needed to confirm Pain is a common problem for people with some efficacy in humans. In addition, dosage and toxicity lev- types of cancer, especially when the cancer grows and els still need to be established. Some studies suggest that presses against other organs and nerves. Pain may also be diets high in garlic reduce the risk of stomach, a side effect of treatment. However, pain can generally be esophageal, and colon cancers. There is still debate relieved or reduced with prescription medicines or over- regarding the best form of garlic to take—whole raw gar- the-counter drugs as recommended by the doctor. Other lic or garlic in tablet form; aged or fresh garlic; garlic ways to reduce pain, such as relaxation exercises, may with odor or “deodorized” garlic. Ginger is often recom- also be useful. It is important for patients to report pain to mended for its beneficial effects on the digestive system, their doctors, so that steps can be taken to help relieve it. but evidence has not confirmed efficacy in cancer treat- ment. Ginseng in excessive amounts can be very toxic, Depression may affect approximately 15–25% of causing vomiting, bleeding, and death. Patients should cancer patients, particularly if the prognosis for recovery G A L E E N C Y C L O P E D I A O F CA N C E R 987
  • 5. Small intestine cancer can lead to cancer is called a carcinogen. Carcinogens QU E S T I O N S include certain chemicals, certain types of radiation, and TO A S K T H E D O C TO R viruses. Asbestos is one substance that is suspected of con- tributing to the development of small intestinal cancer. Although the precise causes of cancer are not known, a • Is there any evidence the cancer has spread? variety of factors are known to contribute to the develop- • What is the stage of the disease? ment of cancer including tobacco smoke, and poor dietary • What are my treatment choices? habits such as high-fat diet. Eating a diet rich in fruits and vegetables and low in fat may reduce the likelihood of • What new treatments are being studied? cancer. Studies have demonstrated that individuals who • Would a clinical trial be appropriate for me? were protected from cancer ate a greater variety of foods • What are the expected benefits of each kind of and nutrients compared to those with cancer. Several treatment? fruits, vitamins, and minerals were found particularly pro- tective against intestinal cancer including vitamin B6, • What are the risks and possible side effects of folate, niacin, and iron. Some studies have linked eating each treatment? large amounts of salt-cured, salt-pickled, and smoked • How often will I have treatments? foods to cancers of the digestive system. Other studies • How long will treatment last? have linked stomach cancers, specifically intestinal cancer, to a lack of fruits, vegetables, and fiber in the diet. For pre- • Will I have to change my normal activities? vention of cancer, it is important to avoid carcinogens • What is the treatment likely to cost? (smoking, chemicals) and known risk factors, and to pur- • Is infertility a side effect of cancer treatment? sue a healthy lifestyle which includes moderate alcohol Can anything be done about it? intake, regular exercise, a low-fat diet, and a diet rich in fruits and vegetables. Modifying genetic predispositions • What is my prognosis? through risk factor reduction can also assist in prevention. Special concerns is poor. A number of antidepressant medications are Due to the side effects of radiation and chemothera- available from physicians to alleviate feelings of depres- py, individuals must make a deliberate effort to eat as sion. Counseling with a psychologist or psychiatrist also nutritiously as possible. Those who experience pain, nau- may help patients deal with depression. sea, or diarrhea may want to discuss treatments options with their doctor to ease these side effects. Clinical trials Eating well during cancer treatment means getting As of 2001, Glivec (STI-571 or imatinib mesylate) enough calories and protein to help prevent weight loss is in clinical trials for treatment of gastrointestinal stro- and maintain strength. Eating nutritiously may also help mal tumors, as well as for leukemia and glioblastoma, a an individual feel better. type of brain tumor. An open trial (GIST trial SWOG- S0033) led by Southwest Oncology Group will test those Resources individuals with metastatic or recurrent disease using BOOKS two doses of the drug. Coit, D.G. “Cancer of the Small Intestine.” In Cancer: Princi- Clinical trials may be suitable for patients suffering ples and Practice of Oncology, edited by V.T. DeVita, Jr., from small intestine cancer. The principal investigator S. Hellman, and S.A. Rosenberg. Philadelphia: Lippin- should be contacted regarding participation in appropri- cott-Raven Publishers, 1997, pp. 1128–1143. ate trials. For information about cancer trials, patients Kelsen, David, Bernard Levin, and Joel Tepper. Principles and Practice of Gastrointestinal Oncology. Philadelphia: Lip- can visit the National Cancer Institute web site at <http:// pincott Williams & Wilkins Publishers, 2001. cancertrials.nci.nih.gov>. “Small Intestine.” In American Joint Committee on Cancer: AJCC Cancer Staging Manual. Philadelphia: Lippincott- Prevention Raven Publishers, 1997, pp. 77–81. Most people who develop cancer do not have inherit- PERIODICALS ed genetic abnormalities. Their genes have been damaged Howe, J.R., et al. “The American College of Surgeons Com- after birth by substances in their environment. A substance mission on Cancer and the American Cancer Society. that damages deoxyribonucleic acid (DNA) in a way that Adenocarcinoma of the Small Bowel: Review of the 988 G A L E E N C Y C L O P E D I A O F CA N C E R
  • 6. Smoking cessation National Cancer Data Base, 1985-1995.” Cancer • Withdrawal symptoms. Nicotine, the substance in tobac- 86(1999): 2693-2706. co that gives smokers a pleasurable feeling, is as addic- tive as heroin or cocaine. Withdrawal from nicotine may ORGANIZATIONS produce depression, anger, fatigue, headaches, prob- The National Cancer Institute (NCI). For information contact the lems with sleep or concentration, or increased appetite Public Inquiries Office: Building 31, Room 10A31, 31 Cen- for food. These symptoms usually start several hours ter Drive, MSC 2580, Betheseda, MD 20892-2580 USA. after the last cigarette. They may last for several days or (301) 435-3848 or 1-800-4-CANCER. <http://cancer.gov/ several weeks. publications/> or <http://cancertrials.nci.nih.gov> or <http:// cancernet.nci.nih.gov>. • Weight gain. Many people, particularly women, gain National Center for Complementary and Alternative Medicine between two and 10 pounds after giving up smoking. (NCCAM), 31 Center Dr., Room #5B-58, Bethesda, MD This mild weight gain, however, is not nearly as great a 20892-2182. (800) NIH-NCAM. Fax: (301) 495-4957. danger to health as continuing to smoke. Getting more <http://nccam.nih.gov>. exercise can help. • Stress. Many smokers started to smoke as a way to cope Crystal Heather Kaczkowski, MSc. with stress and tension. Finding other methods—exer- cise, meditation, biofeedback, massage, and others, can reduce the temptation to smoke when stress arises. • Side effects of nicotine replacement products. Smokers who are using these products to help them quit may Smoking cessation experience headaches, nausea, sore throat, or long-term dependence. Side effects can often be reduced or elimi- Definition nated by using a lower dosage of the product or switch- Smoking cessation is the medical term for quitting ing to another form of nicotine replacement. smoking. It is a vital part of cancer prevention because smoking is the single most preventable cause of death Treatments from cancer. As early as 1982, the Surgeon General reported that tobacco causes more cancer deaths in the Nicotine replacement therapy United States than any other factor–30% of all cancer Nicotine replacement therapy gives the smoker a deaths, including 87% of deaths from lung cancer. measured supply of nicotine without the other harmful Although people think of smoking most often in connec- chemicals in tobacco. It reduces the physical craving for tion with lung cancer, smoking is also associated with cigarettes so that the smoker can handle the psychologi- cancers of the mouth, throat, voice box (larynx), esopha- cal aspects of quitting more effectively. gus, pancreas, kidney, and bladder. Women who smoke As of 2001, the Food and Drug Administration increase their risk of cancer of the cervix. Quitting smok- (FDA) had approved four forms of nicotine replacement ing, however, significantly reduces the risk of cancer; 15 therapy: years after quitting, a former smoker’s risk is almost as low as that of someone who has never smoked. • Transdermal patches. Patches, which are non-prescrip- tion items, supply measured doses of nicotine through the skin. The doses are lowered over a period of weeks, Description thus helping the smoker to reduce the need for nicotine Smoking cessation covers several different gradually. approaches, ranging from medications and psychothera- • Nicotine gum. Nicotine gum provides a fast-acting nico- py to special classes and programs. Smoking is a habit tine replacement that is absorbed through the mouth tis- difficult to break because it involves many different sues. The smoker chews the gum slowly and then keeps aspects of a person’s emotions and social life as well as it against the inside of the cheek for 20 to 30 minutes. physical addiction to nicotine. Most people who quit The gum is also available without prescription. smoking successfully use a combination of treatments or • Nasal spray. Nicotine nasal spray provides nicotine techniques for quitting. through the tissues that line the nose. It acts much more rapidly than the patches or gum, but requires a doctor’s Special concerns prescription. People who are trying to quit smoking are often con- • Inhalers. Nicotine inhalers are plastic tubes containing cerned about: nicotine plugs. The plug gives off nicotine vapor when G A L E E N C Y C L O P E D I A O F CA N C E R 989