Cancer of the Adrenal Cortex
Cancer of the Stomach
Cancer of the Pancreas
Colorectal Cancer
Carcinoid Tumors
Gastrointestinal Stromal Cell Tumor

Abdominal cancers include cancer of the adrenal cortex, stomach cancer, cancer of the pancreas, colorectal cancer, carcinoid tumors of the lung or intestine, and gastrointestinal stromal cell tumors. These abdominal cancers are discussed below. (Refer to the PDQ summary on Wilms Tumor and Other Childhood Kidney Tumors ¹ for information about childhood renal cell carcinoma.)

The adrenal cortex is the outside layer of the adrenal glands. The adrenal glands are a pair of organs near the front side edge of the kidney; their function is to produce hormones such as glucocorticoid and epinephrine. Cancers in this area are classified as carcinomas and adenomas. Adenomas are generally benign, whereas adrenocortical carcinomas frequently secrete hormones and may cause the patient to develop masculine traits, regardless of the patient’s gender. Pediatric patients with adrenocortical carcinoma often have Li-Fraumeni syndrome, an inherited condition that predisposes family members to multiple cancers, including breast cancer, rhabdomyosarcoma, and osteosarcoma (cancer of the bone).

These tumors can involve the kidneys, lungs, bones and brain. Surgical removal should be attempted but may not always be possible if the tumor has spread widely. Additional treatment may include the use of an artificial hormone that blocks the masculinizing effects of the tumor. The prognosis is generally excellent for patients who have small tumors that have been completely removed by surgery, but prognosis can be poor for patients who have large primary tumors or metastatic disease (disease that has spread to other parts of the body) at diagnosis. Tumor stage is an important factor affecting the chance of recovery for children with adrenocortical tumors. When possible, repeat surgery should be done for tumors that come back and for tumors that spread to the inferior vena cava (a large vein that empties into the heart). (Refer to the PDQ summary on adult Adrenocortical Carcinoma Treatment ² for more information.)

The frequency of, and death rate from, stomach cancer has declined worldwide over the past 50 years with the introduction of food preservation practices such as refrigeration. Symptoms of stomach cancer include vague upper abdominal pain, which can be associated with poor appetite, and weight loss. Many individuals become anemic but otherwise show no symptoms before the development of metastatic spread. Other symptoms may include nausea, vomiting, change in bowel habits, poor appetite and weakness, and Helicobacter pylori infection.

Treatment should include surgery. For individuals who cannot have a complete surgical removal of tissue, radiation therapy may be used along with chemotherapy. Prognosis depends on the extent of the disease at the time of diagnosis and the success of treatment that is appropriate for the clinical situation. Because of the rarity of stomach cancer in the pediatric age group, little information exists regarding treatment outcomes of children. (Refer to the PDQ summary on adult Gastric Cancer Treatment ³ for more information.)

Tumors of the pancreas (a gland in the abdomen that makes pancreatic juices and produces hormones) are rare in children and adolescents. Tumors included within the general category can arise at any site in the pancreas. Most pancreatic tumors do not secrete hormones, although some tumors secrete insulin, which can lead to symptoms of weakness, fatigue, hypoglycemia, and coma. If a tumor interferes with the normal function of the islet cells (cells in the pancreas that produce hormones), patients may have watery diarrhea or abnormalities of salt balance. At times, there is obstruction of the head of the pancreas, which is associated with jaundice and gastrointestinal bleeding.

Treatment includes various surgical procedures to remove the pancreas and duodenum or part of the pancreas. For pediatric patients, the effectiveness of radiation therapy is not known. Chemotherapy may be useful for treatment of localized or metastatic pancreatic carcinoma, although few cases have been successfully treated. Response rates and survival rates generally are not good. (Refer to the PDQ summary on adult Pancreatic Cancer Treatment ⁴ for more information.)

Cancer of the large bowel is rare in the pediatric age group: one person per one million younger than 20 years in the United States annually. In children, more than half of colon tumors begin on the right side, compared with adults, who have more colon tumors on the left side. Colon cancer in children is often linked to a family colorectal cancer syndrome, or inherited pattern. There is an increasing risk of colorectal cancer in members of families with a family history of intestinal polyps, which can lead to the development of multiple adenomatous polyps (benign tumors). Juvenile polyps are not associated with an increased incidence or risk of cancer.

Colorectal cancer usually presents with symptoms related to the site of the tumor. Changes in bowel habits are associated with tumors of the rectum or lower colon. Tumors of the right colon may cause more subtle symptoms but are often associated with an abdominal mass, weight loss, decreased appetite, and blood in the stool. Any tumor that causes complete obstruction of the large bowel can cause bowel perforation and spread of the tumor cells within the abdominal cavity.

Colorectal carcinoma is rarely diagnosed in a pediatric patient; however, vague gastrointestinal symptoms should alert the physician to investigate this possibility. Most patients present with evidence of metastatic disease (cancer that has spread to other body parts), either as gross tumor or as microscopic deposits in lymph nodes, on the surface of the bowel, or other organs within the abdomen. Complete surgical removal should be the primary aim of the surgeon, but in most instances, this is impossible; removal of large portions of tumor provides little benefit for the individuals with extensive metastatic disease. Most patients with microscopic metastatic disease generally develop gross metastatic disease, and few individuals with metastatic disease at diagnosis become long-term survivors.

Current therapy includes the use of radiation therapy for rectal and lower colon tumors, in conjunction with chemotherapy. (Refer to the PDQ summaries on adult Colon ⁵ and Rectal Cancer Treatment ⁶ for more information.)

Carcinoid tumors can involve the lining of the lung or the large or small bowel and may not be cancer. Most lung lesions are not cancerous. Treatment of metastatic carcinoid tumors of the large bowel or stomach becomes more complicated and requires treatment similar to that given for colorectal cancer. (Refer to the PDQ summary on Gastrointestinal Carcinoid Tumors Treatment ⁷ for more information.)

Gastrointestinal stromal cell tumor (GIST) usually begins in cells in the wall of the gastrointestinal tract. It may or may not be cancerous. This tumor is usually found in adults older than 40 years and is rare in children. GIST in children younger than 10 years is more common in girls. Symptoms of GIST include anemia caused by gastrointestinal bleeding. Most tumors in children are found in the stomach. A small number of children with GIST are found to have Carney complex, a rare, inherited disorder.Treatment of GIST is different for children than for adults, and includes surgery to remove the tumor. In children, unlike in adults, GIST is not caused by changes in DNA. When these changes are not found, treatment with imatinib mesylate (Gleevec), a new kind of cancer drug that blocks these changes, is not recommended as adjuvant treatment. Some adolescents and young adults with GIST have tumors that are caused by DNA changes and are treatable with imatinib mesylate. All patients with GIST should have their tumors examined for DNA changes.

Glossary Terms

The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

Having to do with the abdomen, which is the part of the body between the chest and the hips that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

Not normal. An abnormal lesion or growth may be cancerous, premalignant (likely to become cancer), or benign.

A noncancerous tumor that starts in gland-like cells of the epithelial tissue (thin layer of tissue that covers organs, glands, and other structures within the body).

Treatment given after the primary treatment to increase the chances of a cure. Adjuvant therapy may include chemotherapy, radiation therapy, hormone therapy, or biological therapy.

A small gland that makes steroid hormones, adrenaline, and noradrenaline. These hormones help control heart rate, blood pressure, and other important body functions. There are two adrenal glands, one on top of each kidney. Also called suprarenal gland.

Having to do with or made by the outer layer of the adrenal gland, which produces steroid hormones. There is an adrenal gland on top of each kidney.

A condition in which the number of red blood cells is below normal.

Not cancerous. Benign tumors may grow larger but do not spread to other parts of the body.

A noncancerous growth that does not invade nearby tissue or spread to other parts of the body.

The long, tube-shaped organ in the abdomen that completes the process of digestion. The bowel has two parts, the small bowel and the large bowel. Also called the intestine.

Glandular organ located on the chest. The breast is made up of connective tissue, fat, and breast tissue that contains the glands that can make milk. Also called mammary gland.

A term for diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord.

A rare cancer that forms in the outer layer of tissue of the adrenal gland (a small organ on top of each kidney that makes steroid hormones, adrenaline, and noradrenaline to control heart rate, blood pressure, and other body functions). Also called adrenocortical cancer and adrenocortical carcinoma.

A slow-growing type of tumor usually found in the gastrointestinal system (most often in the appendix), and sometimes in the lungs or other sites. Carcinoid tumors may spread to the liver or other sites in the body, and they may secrete substances such as serotonin or prostaglandins, causing carcinoid syndrome.

Cancer that begins in the skin or in tissues that line or cover internal organs.

A rare, inherited disorder marked by dark spots on the skin and tumors in the heart, endocrine glands, skin, and nerves. There are two types of Carney complex, which are caused by mutations (changes) in different genes.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

Treatment with drugs that kill cancer cells.

Having to do with the examination and treatment of patients.

Cancer that forms in the tissues of the colon (the longest part of the large intestine). Most colon cancers are adenocarcinomas (cancers that begin in cells that make and release mucus and other fluids).

Having to do with the colon or the rectum.

Cancer that develops in the colon (the longest part of the large intestine) and/or the rectum (the last several inches of the large intestine before the anus).

The process of identifying a disease by the signs and symptoms.

Frequent and watery bowel movements.

In medicine, a disturbance of normal functioning of the mind or body. Disorders may be caused by genetic factors, disease, or trauma.

Deoxyribonucleic acid. The molecules inside cells that carry genetic information and pass it from one generation to the next. Also called deoxyribonucleic acid.

Any substance, other than food, that is used to prevent, diagnose, treat or relieve symptoms of a disease or abnormal condition. Also refers to a substance that alters mood or body function, or that can be habit-forming or addictive, especially a narcotic.

The first part of the small intestine that connects to the stomach.

A hormone and neurotransmitter. Also called adrenaline.

A record of a person's current and past illnesses, and those of his or her parents, brothers, sisters, children, and other family members. A family history shows the pattern of certain diseases in a family, and helps to determine risk factors for those and other diseases.

A condition marked by extreme tiredness and inability to function due lack of energy. Fatigue may be acute or chronic.

GI. Refers to the stomach and intestines. Also called GI.

GIST. A type of tumor that usually begins in cells in the wall of the gastrointestinal tract. It can be benign or malignant. Also called GIST.

The stomach and intestines. The gastrointestinal tract is part of the digestive system, which also includes the salivary glands, mouth, esophagus, liver, pancreas, gallbladder, and rectum.

An organ that makes one or more substances, such as hormones, digestive juices, sweat, tears, saliva, or milk. Endocrine glands release the substances directly into the bloodstream. Exocrine glands release the substances into a duct or opening to the inside or outside of the body.

A compound that belongs to the family of compounds called corticosteroids (steroids). Glucocorticoids affect metabolism and have anti-inflammatory and immunosuppressive effects. They may be naturally produced (hormones) or synthetic (drugs).

H. pylori. A type of bacterium that causes inflammation and ulcers in the stomach or small intestine. People with H. pylori infections may be more likely to develop cancer in the stomach, including MALT (mucosa-associated lymphoid tissue) lymphoma. Also called H. pylori.

One of many chemicals made by glands in the body. Hormones circulate in the bloodstream and control the actions of certain cells or organs. Some hormones can also be made in the laboratory.

Abnormally low blood sugar.

A drug used to treat different types of leukemia and other cancers of the blood, gastrointestinal stromal tumors, skin tumors called dermatofibrosarcoma protuberans, and a rare condition called systemic mastocytosis. It is also being studied in the treatment of other types of cancer. Imatinib mesylate blocks the protein made by the bcr/abl oncogene. It is a type of tyrosine kinase inhibitor. Also called Gleevec and STI571.

The number of new cases of a disease diagnosed each year.

Invasion and multiplication of germs in the body. Infections can occur in any part of the body and can spread throughout the body. The germs may be bacteria, viruses, yeast, or fungi. They can cause a fever and other problems, depending on where the infection occurs. When the body’s natural defense system is strong, it can often fight the germs and prevent infection. Some cancer treatments can weaken the natural defense system.

A large vein that empties into the heart. It carries blood from the legs and feet and from organs in the abdomen and pelvis.

Transmitted through genes that have been passed from parents to their offspring (children).

A hormone made by the islet cells of the pancreas. Insulin controls the amount of sugar in the blood by moving it into the cells, where it can be used by the body for energy.

Having to do with the intestines.

The long, tube-shaped organ in the abdomen that completes the process of digestion. The intestine has two parts, the small intestine and the large intestine. Also called the bowel.

A pancreatic cell that produces hormones (e.g., insulin and glucagon) that are secreted into the bloodstream. These hormones help control the level of glucose (sugar) in the blood. Also called endocrine pancreas cell and islet of Langerhans cell.

A condition in which the skin and the whites of the eyes become yellow, urine darkens, and the color of stool becomes lighter than normal. Jaundice occurs when the liver is not working properly or when a bile duct is blocked.

One of a pair of organs in the abdomen. Kidneys remove waste from the blood (as urine), produce erythropoietin (a substance that stimulates red blood cell production), and play a role in blood pressure regulation.

The long, tube-like organ that is connected to the small intestine at one end and the anus at the other. The large intestine has four parts: cecum, colon, rectum, and anal canal. Partly digested food moves through the cecum into the colon, where water and some nutrients and electrolytes are removed. The remaining material, solid waste called stool, moves through the colon, is stored in the rectum, and leaves the body through the anal canal and anus.

An area of abnormal tissue. A lesion may be benign (noncancerous) or malignant (cancerous).

A rare, inherited predisposition to multiple cancers, caused by an alteration in the p53 tumor suppressor gene.

Restricted to the site of origin, without evidence of spread.

One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.

A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.

Having to do with metastasis, which is the spread of cancer from one part of the body to another.

Cancer that has spread from the place in which it started to other parts of the body.

Too small to be seen without a microscope.

A feeling of sickness or discomfort in the stomach that may come with an urge to vomit. Nausea is a side effect of some types of cancer therapy.

Blockage of a passageway.

A part of the body that performs a specific function. For example, the heart is an organ.

A cancer of the bone that usually affects the large bones of the arm or leg. It occurs most commonly in young people and affects more males than females. Also called osteogenic sarcoma.

A glandular organ located in the abdomen. It makes pancreatic juices, which contain enzymes that aid in digestion, and it produces several hormones, including insulin. The pancreas is surrounded by the stomach, intestines, and other organs.

A disease in which malignant (cancer) cells are found in the tissues of the pancreas. Also called exocrine cancer.

Fluid made by the pancreas. Pancreatic juices contain proteins called enzymes that aid in digestion.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

Having to do with children.

Medical doctor.

A growth that protrudes from a mucous membrane.

The original tumor.

The likely outcome or course of a disease; the chance of recovery or recurrence.

The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called radiotherapy and irradiation.

To become well and healthy again.

By or having to do with the rectum. The rectum is the last several inches of the large intestine and ends at the anus.

The percentage of patients whose cancer shrinks or disappears after treatment.

Cancer that forms in the soft tissues in a type of muscle called striated muscle. Rhabdomyosarcoma can occur anywhere in the body.

The part of the digestive tract that is located between the stomach and the large intestine.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

An organ that is part of the digestive system. The stomach helps digest food by mixing it with digestive juices and churning it into a thin liquid.

Cancer that forms in tissues lining the stomach. Also called gastric cancer.

The material in a bowel movement. Stool is made up of food that was not digested, bacteria, mucus, and cells from the intestines. Also called feces.

A doctor who removes or repairs a part of the body by operating on the patient.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

The percentage of people in a study or treatment group who are alive for a certain period of time after they were diagnosed with or treated for a disease, such as cancer. The survival rate is often stated as a five-year survival rate, which is the percentage of people in a study or treatment group who are alive five years after diagnosis or treatment. Also called overall survival rate.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

A set of symptoms or conditions that occur together and suggest the presence of a certain disease or an increased chance of developing the disease.

Treatment.

A group or layer of cells that work together to perform a specific function.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancerous), or malignant (cancerous). Also called neoplasm.

A blood vessel that carries blood to the heart from tissues and organs in the body.

To eject some or all of the contents of the stomach through the mouth.