Unusual Cancers of Childhood Treatment (PDQ®)–Patient Version
Unusual Cancers of the Abdomen
Adrenocortical Carcinoma
There are two adrenal glands. The adrenal glands are small and shaped like a triangle. One adrenal gland sits on top of each kidney. Each adrenal gland has two parts. The outer layer of the adrenal gland is the adrenal cortex. The center of the adrenal gland is the adrenal medulla. Adrenocortical carcinoma is also called cancer of the adrenal cortex.
Childhood adrenocortical carcinoma occurs most commonly in patients younger than 6 years or in the teen years, and more often in females.
The adrenal cortex makes important hormones that do the following:
- Balance the water and salt in the body.
- Help keep blood pressure normal.
- Help control the body's use of protein, fat, and carbohydrates.
- Cause the body to have male or female characteristics.
Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests
The risk of adrenocortical carcinoma is increased by having a certain mutation (change) in a gene or any of the following syndromes:
A tumor of the adrenal cortex may be functioning (makes more hormones than normal) or nonfunctioning (does not make extra hormones). Most tumors of the adrenal cortex in children are functioning tumors. The extra hormones made by functioning tumors may cause certain signs or symptoms of disease and these depend on the type of hormone made by the tumor. For example, extra androgen hormone may cause both male and female children to develop masculine traits, such as body hair or a deep voice, grow faster, and have acne. Extra estrogen hormone may cause the growth of breast tissue in male children. (See the PDQ summary on adult Adrenocortical Carcinoma Treatment for more information on the signs and symptoms of adrenocortical carcinoma.)
The tests and procedures used to diagnose and stage adrenocortical carcinoma depend on the patient's symptoms. They may include:
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose adrenocortical carcinoma include the following:
- Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of cortisol or 17-ketosteroids. A higher than normal amount of these substances in the urine may be a sign of disease in the adrenal cortex.
- Low-dose dexamethasone suppression test: A test in which one or more small doses of dexamethasone are given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days. This test is done to check if the adrenal gland is making too much cortisol.
- High-dose dexamethasone suppression test: A test in which one or more high doses of dexamethasone are given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days. This test is done to check if the adrenal gland is making too much cortisol or if the pituitary gland is telling the adrenal glands to make too much cortisol.
- Blood hormone studies: A procedure in which a blood sample is checked to measure the amounts of certain hormones released into the blood by organs and tissues in the body. 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 blood may be checked for testosterone or estrogen. A higher than normal amount of these hormones may be a sign of adrenocortical carcinoma.
- Adrenal angiography : A procedure to look at the arteries and the flow of blood near the adrenal gland. A contrast dye is injected into the adrenal arteries. As the dye moves through the blood vessel, a series of x-rays are taken to see if any arteries are blocked.
- Adrenal venography : A procedure to look at the adrenal veins and the flow of blood near the adrenal glands. A contrast dye is injected into an adrenal vein. As the contrast dye moves through the vein, a series of x-rays are taken to see if any veins are blocked. A catheter (very thin tube) may be inserted into the vein to take a blood sample, which is checked for abnormal hormone levels.
Prognosis
The prognosis (chance of recovery) is good for patients who have small tumors that have been completely removed by surgery. For other patients, the prognosis depends on the following:
- Size of the tumor.
- Whether there are changes in certain genes.
- Whether the tumor has spread to other parts of the body, including the lymph nodes.
- Child's age.
- Whether the covering around the tumor broke open during surgery to remove the tumor.
- Whether the child has developed masculine traits.
These tumors can spread to the kidneys, lungs, bones, and brain.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of adrenocortical carcinoma in children may include the following:
- Surgery to remove the tumor with or without chemotherapy.
Treatment of recurrent adrenocortical carcinoma 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 Adrenocortical Carcinoma Treatment for more information.
Stomach (Gastric) Cancer
Stomach cancer is a disease in which malignant (cancer) cells form in the lining of the stomach. The stomach is a J-shaped organ in the upper abdomen. It is part of the digestive system, which processes nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) in foods that are eaten and helps pass waste material out of the body. Food moves from the throat to the stomach through a hollow, muscular tube called the esophagus. After leaving the stomach, partly-digested food passes into the small intestine and then into the large intestine.
The risk of stomach cancer is increased by having an infection with Helicobacter pylori (H. pylori) bacterium, which is found in the stomach.
Signs and Symptoms
Many patients will have anemia (a lower than normal number of red blood cells), but have no signs or symptoms before the cancer spreads. Stomach cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
- Stomach pain.
- Loss of appetite.
- Weight loss for no known reason.
- Nausea.
- Vomiting.
- Constipation or diarrhea.
- Weakness.
Other conditions that are not stomach cancer may cause these same signs and symptoms.
Diagnostic and Staging Tests
- Physical exam and history.
- X-ray of the abdomen.
- Blood chemistry studies.
- CT scan.
- Biopsy.
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose stomach cancer include the following:
- Upper endoscopy : A procedure to look inside the esophagus, stomach, and duodenum (first part of the small intestine) to check for abnormal areas. An endoscopeis passed through the mouth and down the throat into the esophagus. 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.
- Barium swallow : A series of x-rays of the esophagus and stomach. The patient drinks a liquid that contains barium (a silver-white metallic compound). The liquid coats the esophagus and stomach, and x-rays are taken. This procedure is also called an upper GI series.
- 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.
Prognosis
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of stomach cancer in children may include the following:
- Surgery to remove the cancer and some healthy tissue around it.
- Surgery to remove as much of the cancer as possible, followed by radiation therapyand/or chemotherapy.
Treatment of recurrent stomach 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.
See the PDQ summary on adult Gastric Cancer Treatment for more information.
Pancreatic Cancer
Pancreatic cancer is a disease in which malignant (cancer) cells form in the tissues of the pancreas. The pancreas is a pear-shaped gland about 6 inches long. The wide end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. Many different kinds of tumors can form in the pancreas. Some tumors are benign (not cancer).
The pancreas has two main jobs in the body:
- To make juices that help digest (break down) food. These juices are secreted into the small intestine.
- To make hormones that help control the sugar and salt levels in the blood. These hormones are secreted into the bloodstream.
There are four types of pancreatic cancer in children:
- Solid pseudopapillary tumor of the pancreas. This is the most common type of pancreatic tumor. It most commonly affects females that are older adolescents and young adults. The tumors have both cyst -like and solid parts. Solid pseudopapillary tumor of the pancreas is unlikely to spread to other parts of the body and the prognosis is very good.
- Pancreatoblastoma. It usually occurs in children aged 10 years or younger. Children with Beckwith-Wiedemann syndrome and familial adenomatous polyposis (FAP) syndrome have an increased risk of developing pancreatoblastoma. These tumors may make adrenocorticotropic hormone (ACTH) and antidiuretic hormone (ADH). Pancreatoblastoma may spread to the liver, lungs, and lymph nodes. The prognosis for children with pancreatoblastoma is good.
- Islet cell tumors. These tumors are not common in children and can be benign or malignant. Islet cell tumors may occur in children with multiple endocrine neoplasia type 1 (MEN1) syndrome. The most common types of islet cell tumors are insulinomasand gastrinomas. These tumors may make hormones, such as insulin and gastrin, that cause signs and symptoms.
- Pancreatic carcinoma. Pancreatic carcinoma is very rare in children. The two types of pancreatic carcinoma are acinar cell carcinoma and ductal adenocarcinoma.
Signs and Symptoms
In children, some pancreatic tumors do not secrete hormones and there are no signs and symptoms of disease. This makes it hard to diagnose pancreatic cancer early.
Pancreatic tumors that do secrete hormones may cause signs and symptoms. The signs and symptoms depend on the type of hormone being made.
If the tumor secretes insulin, signs and symptoms that may occur include the following:
- Low blood sugar. This can cause blurred vision, headache, and feeling lightheaded, tired, weak, shaky, nervous, irritable, sweaty, confused, or hungry.
- Changes in behavior.
- Seizures.
- Coma.
If the tumor secretes gastrin, signs and symptoms that may occur include the following:
Signs and symptoms caused by tumors that make other types of hormones may include the following:
- Watery diarrhea.
- Dehydration (feeling thirsty, making less urine, dry skin and mouth, headaches, dizziness, or feeling tired).
- Low sodium (salt) level in the blood (confusion, sleepiness, muscle weakness, and seizures).
- Weight loss or gain for no known reason.
- Round face and thin arms and legs.
- Feeling very tired and weak.
- High blood pressure.
- Purple or pink stretch marks on the skin.
If cancer is in the head of the pancreas, the bile duct or blood flow to the stomach may be blocked and the following signs may occur:
Check with your child’s doctor if you see any of these problems in your child. Other conditions that are not pancreatic cancer may cause these same signs and symptoms.
Diagnostic and Staging Tests
Tests to diagnose and stage pancreatic cancer may include the following:
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose pancreatic cancer include the following:
- Endoscopic ultrasound (EUS): A procedure in which an endoscope is inserted into the body, usually through the mouth or rectum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal tissues or organsand make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography.
- Endoscopic retrograde cholangiopancreatography (ERCP): A procedure used to x-ray the ducts (tubes) that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Sometimes pancreatic cancer causes these ducts to narrow and block or slow the flow of bile, causing jaundice. An endoscope (a thin, lighted tube) is passed through the mouth, esophagus, and stomach into the first part of the small intestine. A catheter (a smaller tube) is then inserted through the endoscope into the pancreatic ducts. A dye is injected through the catheter into the ducts and an x-ray is taken. If the ducts are blocked by a tumor, a fine tube may be inserted into the duct to unblock it. This tube, called a stent, may be left in place to keep the duct open. Tissue samples may also be taken and checked under a microscope for signs for cancer.
- Somatostatin receptor scintigraphy : A type of radionuclide scan used to find pancreatic tumors. A very small amount of radioactive octreotide (a hormone that attaches to carcinoid tumors) is injected into a vein and travels through the bloodstream. 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 used to diagnose islet cell tumors.
- Laparoscopy : A surgical procedure to look at the organs inside the abdomen to check for signs of disease. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as removing organs or taking tissue samples to be checked under a microscope for signs of disease.
- Laparotomy : A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of solid pseudopapillary tumor of the pancreas in children may include the following:
- Surgery to remove the tumor.
- Chemotherapy for tumors that cannot be removed by surgery or have spread to other parts of the body.
Treatment of pancreatoblastoma in children may include the following:
- Surgery to remove the tumor. A Whipple procedure may be done for tumors in the head of the pancreas.
- Chemotherapy may be given to shrink the tumor before surgery. More chemotherapy may be given after surgery for large tumors, tumors that cannot be removed by surgery, and tumors that have spread to other parts of the body.
- Chemotherapy may be given if the tumor does not respond to treatment or comes back.
Treatment of islet cell tumors in children may include drugs to treat symptoms caused by hormones and the following:
- Surgery to remove the tumor.
- Chemotherapy and targeted therapy for tumors that cannot be removed by surgery or that have spread to other parts of the body.
Treatment of recurrent pancreatic carcinoma 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 summaries on adult Pancreatic Cancer Treatment and adult Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment for more information on pancreatic tumors.
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:
- Attenuated familial adenomatous polyposis.
- Familial adenomatous polyposis (FAP).
- Lynch syndrome.
- Li-Fraumeni syndrome.
- MYH-associated polyposis.
- Turcot syndrome.
- Cowden syndrome.
- Juvenile polyposis syndrome.
- Peutz-Jeghers syndrome.
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:
Other conditions that are not colorectal cancer may cause these same signs and symptoms.
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 when it has not spread.
- Radiation therapy and chemotherapy for tumors in the rectum or lower colon.
- Combination chemotherapy.
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.
See the following PDQ summaries on adult cancer for more information:
Neuroendocrine Tumors (Carcinoid Tumors)
Neuroendocrine tumors (including carcinoid tumors) usually form in the lining of the stomach or intestines, 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. Sometimes 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
Some neuroendocrine tumors release hormones and other substances. If the tumor is in the liver, high amounts of these hormones may remain in the body and cause a group of signs and symptoms called carcinoid syndrome. Carcinoid syndrome caused by the hormone somatostatin 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 and neck.
- 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:
- 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.
- 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 a hormone 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:
- Radioembolization.
- Somatostatin analogue therapy (octreotide or lanreotide).
- Peptide receptor radionuclide therapy.
- Targeted therapy (sunitinib or 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:
- Feeling tired.
- 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.
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.
Treatment for children whose tumors do not show genetic changes may include the following:
- Surgery to remove the tumor.
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.
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