Abdominal Cancers
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 for information about childhood renal cell carcinoma.)
Cancer of the Adrenal Cortex
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 for more information.)
Cancer of the Stomach
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 for more information.)
Cancer of the Pancreas
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. (Refer to the PDQ summary on adult Pancreatic Cancer Treatment for more
information.)
Colorectal Cancer
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 and Rectal Cancer Treatment for more information.)
Carcinoid Tumors
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 for more information.)
Gastrointestinal Stromal Cell Tumor
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.
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