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Unusual Cancers of Childhood (PDQ®)

Unusual Cancers of the Abdomen

Cancer of the Adrenal Cortex

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. Cancer of the adrenal cortex is also called adrenocortical carcinoma.

Childhood cancer of the adrenal cortex 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 cancer of the adrenal cortex is increased by having any of the following syndromes:

A tumor of the adrenal cortex may be functioning (makes more hormones than normal) or nonfunctioning (does not make hormones). The 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 cancer of the adrenal cortex.)

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 cancer of the adrenal cortex 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 is given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days.
  • High-dose dexamethasone suppression test: A test in which one or more high doses of dexamethasone is given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days.
  • Blood tests : Tests to measure the levels of testosterone or estrogen in the blood. A higher than normal amount of these hormones that 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. The cancer is harder to treat when the tumor is large or when the cancer has spread to other parts of the body when it was diagnosed. These tumors can spread to the kidneys, lungs, bones, and brain.

Treatment

Treatment for cancer of the adrenal cortex in children may include the following:

  • Surgery with or without chemotherapy.
  • A second surgery for tumors that come back and for tumors that spread to other parts of the body.

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.

Gastrointestinal (digestive) system anatomy; drawing shows the esophagus, liver, stomach, small intestine, and large intestine.
The esophagus and stomach are part of the upper gastrointestinal (digestive) system.

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:

Other conditions that are not stomach cancer may cause these same signs and symptoms.

Diagnostic and Staging Tests

Tests to diagnose and stage stomach cancer may include the following:

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 endoscope is 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:

Prognosis

Prognosis (chance of recovery) depends on whether the cancer has spread at the time of diagnosis.

Treatment

Treatment of stomach cancer in children may include the following:

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).

Anatomy of the pancreas; drawing shows the pancreas, stomach, spleen, liver, gallbladder, bile ducts, colon, and small intestine. An inset shows the head, body, and tail of the pancreas. The bile duct and pancreatic duct are also shown.
Anatomy of the pancreas. The pancreas has three areas: head, body, and tail. It is found in the abdomen near the stomach, intestines, and other organs.

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.

The risk of pancreatic cancer is increased by having Beckwith-Wiedemann syndrome or Cushing syndrome.

Signs and Symptoms

Most pancreatic tumors do not secrete hormones and there are no signs or symptoms of disease. This makes it difficult 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:

  • Weakness.
  • Feeling very tired.
  • Low blood sugar. This can cause blurred vision, headache, and feeling lightheaded, tired, weak, shaky, nervous, irritable, sweaty, confused, or hungry.
  • Coma.

Other signs and symptoms caused by tumors that make hormones include the following:

  • Watery diarrhea.
  • Abnormal sodium (salt) level in the blood: Having a low sodium level can cause confusion, sleepiness, muscle weakness, and seizures. Having a high sodium level may cause weakness, tiredness, confusion, paralysis, coma, and seizures.
  • A lump in the abdomen.
  • Weight loss for no known reason.
  • Pain in the abdomen.

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 organs and 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.
  • Percutaneous transhepatic cholangiography (PTC): A procedure used to x-ray the liver and bile ducts. A thin needle is inserted through the skin below the ribs and into the liver. Dye is injected into the liver or bile ducts and an x-ray is taken. If a blockage is found, a thin, flexible tube called a stent is sometimes left in the liver to drain bile into the small intestine or a collection bag outside the body. This test is done only if ERCP cannot be done.
  • 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

Treatment for children with pancreatic cancer may include the following:

See the PDQ summary on adult Pancreatic Cancer Treatment for more information.

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).

Gastrointestinal (digestive) system anatomy; shows esophagus, liver, stomach, colon, small intestine, rectum, and anus.
Anatomy of the lower digestive system, showing the colon and other organs.

Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests

Childhood colon cancer is often part of an inherited syndrome that causes the disease. 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 inherited certain conditions, such as:

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 tumor forms. 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:

Other conditions that are not colorectal cancer may cause these same signs and symptoms.

Tests to diagnose and stage colorectal cancer may include the following:

Other tests used to diagnose colorectal cancer include the following:

  • Colonoscopy : A procedure to look inside the rectum and colon for polyps, abnormal areas, 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:
  • 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 colon 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

Treatment for colorectal cancer in children may include the following:

Children with certain familial colon 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:

Carcinoid Tumors

Carcinoid tumors usually form in the lining of the stomach or intestines, but they can form in other organs, such as the lungs or liver. These tumors are usually small, slow-growing, and benign (not cancer). Some carcinoid tumors are malignant (cancer) and spread to other places in the body. Sometimes carcinoid 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.

Signs and Symptoms

Some carcinoid 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.
  • Diarrhea.

Other conditions that are not carcinoid 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 carcinoid tumors. They may include:

See the General Information section for a description of these tests and procedures.

Other tests used to diagnose carcinoid tumors include the following:

  • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for 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 a hormone made by carcinoid tumors. This test is used to help diagnose carcinoid syndrome.

Treatment

Treatment for carcinoid tumors in the appendix in children may include the following:

  • Surgery to remove the appendix, when the tumor is small and only in the appendix.
  • Surgery to remove the appendix, lymph nodes, and part of the large intestine, when the tumor is larger, has spread to nearby lymph nodes, and is in the appendix.
  • Surgery, chemotherapy, and/or radiation therapy for tumors that have spread.

Treatment for carcinoid tumors that have spread to the large intestine or stomach is the same as treatment for colorectal cancer.

For tumors that make hormones that cause symptoms, medicine can be given to help relieve the symptoms.

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 stomach or 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 genetic changes. 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.

Other conditions that are not anemia caused by GIST may cause these same signs and symptoms.

Treatment

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 and check nearby lymph nodes for signs of cancer. If cancer is in the lymph nodes, the lymph nodes are removed.
  • Watchful waiting for tumors that come back in the same place or cannot be removed, but do not cause signs or symptoms.
  • Updated: December 11, 2014