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

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. (For more information, see the Renal Cell Cancer section in the PDQ summary on Wilms Tumor and Other Childhood Kidney Tumors.)

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). (See the PDQ summary on adult Adrenocortical Carcinoma Treatment for more information.)

Cancer that begins in the stomach is rare in children. 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. (See the PDQ summary on adult Gastric Cancer Treatment 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. Pancreatoblastoma may be treated with combination chemotherapy given before or after surgery. Response rates and survival rates generally are not good. (See the PDQ summary on adult Pancreatic Cancer Treatment 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. (See the PDQ summaries on adult Colon and Rectal Cancer Treatment 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. (See the PDQ summary on Gastrointestinal Carcinoid Tumors Treatment 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 and is rare in children. GIST is more common in girls, and usually appears in patients over the age of 10 years. Most children with GIST have tumors in the stomach and develop anemia caused by gastrointestinal bleeding. A small number of children with GIST are found to have one of two rare genetic disorders, Carney triad or Carney Stratakis syndrome.

GIST in children is not the same as the disease in adults. Patients should be seen at centers that specialize in the treatment of GIST and all tumors should be tested for genetic changes. A small number of children have tumors with genetic changes similar to those found in adult patients. These children are treated with a type of drug called a tyrosine kinase inhibitor that blocks these changes.

Treatment for most children whose tumors do not show these genetic changes includes surgery to remove the tumor when possible. If the disease comes back in the same place or cannot be removed but is not causing symptoms, watchful waiting may be recommended. If the disease progresses or causes symptoms, tyrosine kinase inhibitor therapy may be an option. However, there is not enough evidence to recommend tyrosine kinase inhibitor therapy as standard adjuvant treatment in children.

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