Unusual Cancers of Childhood Treatment (PDQ®)–Patient Version
Colorectal Cancer
Colorectal cancer is a disease in which malignant (cancer) cells form in the tissues of the colon or the rectum. The colon is part of the body’s digestive system. The digestive system removes and processes nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) from foods and helps pass waste material out of the body. The digestive system is made up of the esophagus, stomach, and the small and large intestines. The colon (large bowel) is the first part of the large intestine and is about 5 feet long. Together, the rectum and anal canal make up the last part of the large intestine and are 6-8 inches long. The anal canal ends at the anus (the opening of the large intestine to the outside of the body).
Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests
Childhood colorectal cancer may be part of an inherited syndrome. Some colorectal cancers in young people are linked to a gene mutation that causes polyps (growths in the mucous membrane that lines the colon) to form that may turn into cancer later.
The risk of colorectal cancer is increased by having certain inherited conditions, such as:
- Familial adenomatous polyposis (FAP).
- Attenuated FAP.
- MUTYH-associated polyposis.
- Lynch syndrome.
- Oligopolyposis.
- Change in the NTHL1 gene.
- Juvenile polyposis syndrome.
- Cowden syndrome.
- Peutz-Jeghers syndrome.
- Neurofibromatosis type 1 (NF1).
Colon polyps that form in children who do not have an inherited syndrome are not linked to an increased risk of cancer.
Signs and symptoms of childhood colorectal cancer usually depend on where the tumorforms. Colorectal cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
- Tumors of the rectum or lower colon may cause pain in the abdomen, constipation, or diarrhea.
- Tumors in the part of the colon on the left side of the body may cause:
- Tumors in the part of the colon on the right side of the body may cause:
- Pain in the abdomen.
- Blood in the stool.
- Constipation or diarrhea.
- Nausea or vomiting.
- Weight loss for no known reason.
Other conditions that are not colorectal cancer may cause these same signs and symptoms.
- Physical exam and health history.
- X-ray of the chest.
- CT scan of the chest, abdomen, and pelvis.
- PET scan.
- MRI.
- Bone scan.
- Biopsy.
Other tests used to diagnose colorectal cancer include the following:
- Colonoscopy: A procedure to look inside the rectum and colon for polyps, abnormalareas, or cancer. A colonoscope is inserted through the rectum into the colon. A colonoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove polyps or tissue samples, which are checked under a microscope for signs of cancer.
- Barium enema: A series of x-rays of the lower gastrointestinal tract. A liquid that contains barium (a silver-white metallic compound) is put into the rectum. The barium coats the lower gastrointestinal tract and x-rays are taken. This procedure is also called a lower GI series.
- Fecal occult blood test: A test to check stool (solid waste) for blood that can only be seen with a microscope. Small samples of stool are placed on special cards and returned to the doctor or laboratory for testing.
- Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
- The number of red blood cells, white blood cells, and platelets.
- The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
- The portion of the blood sample made up of red blood cells.
- Kidney function test: A test in which blood or urine samples are checked for the amounts of certain substances released by the kidneys. A higher or lower than normal amount of a substance can be a sign that the kidneys are not working the way they should. This is also called a renal function test.
- Liver function test: A blood test to measure the blood levels of certain substances released by the liver. A high or low level of certain substances can be a sign of liver disease.
- Carcinoembryonic antigen (CEA) assay: A test that measures the level of CEA in the blood. CEA is released into the bloodstream from both cancer cells and normal cells. When found in higher than normal amounts, it can be a sign of colorectal cancer or other conditions.
Prognosis
The prognosis (chance of recovery) depends on the following:
- Whether the entire tumor was removed by surgery.
- Whether the cancer has spread to other parts of the body, such as the lymph nodes, liver, pelvis, or ovaries.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of colorectal cancer in children may include the following:
- Surgery to remove the tumor if it has not spread.
- Radiation therapy and chemotherapy for tumors in the rectum or lower colon.
- Combination chemotherapy, for advanced colorectal cancer.
- Immunotherapy with immune checkpoint inhibitors (ipilimumab and nivolumab).
Treatment of recurrent colorectal cancer in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
Children with certain familial colorectal cancer syndromes may be treated with:
- Surgery to remove the colon before cancer forms.
- Medicine to decrease the number of polyps in the colon.
Neuroendocrine Tumors (Carcinoid Tumors)
Neuroendocrine cells can act like nerve cells or hormone-making cells. The cells are scattered throughout organs such as the lungs (tracheobronchial) or digestive tract.
Neuroendocrine tumors (including carcinoid tumors) usually form in the lining of the stomach or intestines (including the appendix), but they can form in other organs, such as the pancreas, lungs, or liver. These tumors are usually small, slow-growing, and benign(not cancer). Some neuroendocrine tumors are malignant (cancer) and spread to other places in the body.
Most neuroendocrine tumors in children form in the appendix (a pouch that sticks out from the first part of the large intestine near the end of the small intestine). The tumor is often found during surgery to remove the appendix.
See the Tracheobronchial tumors section of this summary for information on tracheobronchial carcinoid tumors.
Signs and Symptoms
Signs and symptoms of neuroendocrine tumors depend on where the tumor forms. Neuroendocrine tumors in the appendix may cause the following signs and symptoms:
Neuroendocrine tumors that are not in the appendix may release hormones and other substances. Carcinoid syndrome caused by the hormone serotonin and other hormones, may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
- Redness and a warm feeling in the face, neck, and upper chest.
- A fast heartbeat.
- Trouble breathing.
- Sudden drop in blood pressure (restlessness, confusion, weakness, dizziness, and pale, cool, and clammy skin).
- Diarrhea.
Other conditions that are not neuroendocrine tumors may cause these same signs and symptoms.
Diagnostic and Staging Tests
Tests that check for signs of cancer are used to diagnose and stage neuroendocrine tumors. They may include:
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose neuroendocrine tumors include the following:
- Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of certain substances, such as hormones. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. The urine sample is checked to see if it contains 5-HIAA (a breakdown product of the hormone serotonin which may be made by carcinoid tumors). This test is used to help diagnose carcinoid syndrome.
- Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find tumors. A very small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.
Prognosis
The prognosis for neuroendocrine tumors in the appendix in children is usually excellent after surgery to remove the tumor. Neuroendocrine tumors that are not in the appendix are usually larger or have spread to other parts of the body at the time of diagnosis and do not respond well to chemotherapy. Larger tumors are more likely to recur (come back).
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of neuroendocrine tumors in the appendix in children may include the following:
- Surgery to remove the appendix.
Treatment of neuroendocrine tumors that have spread to the large intestine, pancreas, or stomach is usually surgery. Treatment of tumors that cannot be removed by surgery, multiple tumors, or tumors that have spread may include the following:
- Embolization.
- Somatostatin analogue therapy (octreotide or lanreotide).
- Peptide receptor radionuclide therapy.
- Targeted therapy with a tyrosine kinase inhibitor (sunitinib) or an mTOR inhibitor(everolimus).
Treatment of recurrent neuroendocrine tumors in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the PDQ summary on adult Gastrointestinal Carcinoid Tumors Treatment for more information.
Gastrointestinal Stromal Tumors
Gastrointestinal stromal cell tumors (GIST) usually begin in cells in the wall of the stomachor intestines. GISTs may be benign (not cancer) or malignant (cancer). Childhood GISTs are more common in girls, and usually appear in the teen years.
Risk Factors and Signs and Symptoms
GISTs in children are not the same as GISTs in adults. Patients should be seen at centers that specialize in the treatment of GISTs and the tumors should be tested for geneticchanges. A small number of children have tumors with genetic changes like those found in adult patients. The risk of GIST is increased by the following genetic disorders:
Most children with GIST have tumors in the stomach and develop anemia caused by bleeding. Signs and symptoms of anemia include the following:
- Tiredness.
- Dizziness.
- A fast or irregular heartbeat.
- Shortness of breath.
- Pale skin.
A lump in the abdomen or a blockage of the intestine (crampy pain in the abdomen, nausea, vomiting, diarrhea, constipation, and swelling of the abdomen) are also signs of GIST.
Other conditions that are not anemia caused by GIST may cause these same signs and symptoms.
Diagnostic and Staging Tests
- Physical exam and health history.
- MRI.
- CT scan.
- PET scan.
- X-ray of the abdomen.
- Biopsy.
- Fine-needle aspiration: The removal of tissue using a thin needle.
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose GIST include the following:
- Endoscopy: A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth or anus. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment for children who have tumors with genetic changes like those found in adult patients is targeted therapy with a tyrosine kinase inhibitor (imatinib or sunitinib).
Treatment for children whose tumors do not show genetic changes may include the following:
- Surgery to remove the tumor. More surgery may be needed if intestinal obstruction or bleeding occurs.
Treatment of recurrent GIST in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
- A clinical trial of a new chemotherapy drug.
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