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Unusual Cancers of Childhood

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.

The adrenal cortex makes important hormones that do the following:

Risk Factors, 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 testosterone may cause both male and female children to develop masculine traits, such as body hair or a deep voice. (See the PDQ summary on adult Adrenocortical Carcinoma Treatment for more information on the symptoms of cancer of the adrenal cortex.)

Tests that examine the adrenal gland are used to diagnose and stage cancer of the adrenal cortex. The tests and procedures used to diagnose 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:

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:

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 stomach and esophagus are part of the upper digestive system.
The stomach and esophagus are part of the upper digestive system.

Symptoms and Diagnostic and Staging Tests

Many patients will have anemia (a lower than normal number of red blood cells), but have no symptoms before the cancer spreads. Stomach cancer may cause any of the following signs and symptoms. Check with your doctor if any of the following problems occur:

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

Tests that examine the stomach and esophagus are used to diagnose and stage stomach cancer. They may include:

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

Other tests used to diagnose stomach cancer include 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. The pancreas has three areas: head, body, and tail. It is found in the abdomen near the stomach, intestines, and other organs.
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:

Symptoms and Diagnostic and Staging Tests

Most pancreatic tumors do not secrete hormones. Pancreatic tumors that do secrete hormones may cause symptoms. The symptoms depend on the type of hormone being made.

If the tumor secretes insulin, symptoms that may occur include the following:

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

If cancer is in the head of the pancreas, the bile duct or blood flow to the stomach may be blocked and the following symptoms may occur:

Check with your doctor if any of these problems occur. Other conditions that are not pancreatic cancer may cause these same symptoms.

Tests that examine the pancreas are used to diagnose and stage pancreatic cancer. They may include:

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

Other tests used to diagnose pancreatic cancer include the following:

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 first 6 feet of the large intestine are called the large bowel or colon. The last 6 inches are the rectum and the anal canal. The anal canal ends at the anus (the opening of the large intestine to the outside of the body).


Anatomy of the lower digestive system, showing the colon and other organs.
Anatomy of the lower digestive system, showing the colon and other organs.

Risk Factors, 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. This gene is also linked to an increased risk of brain and liver tumors. Colon polyps that form in children who do not have an inherited syndrome are not linked to an increased risk of cancer.

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 doctor if any of the following problems occur:

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

Tests that examine the colon and rectum are used to diagnose and stage colorectal cancer. They may include:

Other tests used to diagnose colorectal cancer include the following:

Prognosis

The prognosis (chance of recovery) depends on the following:

Treatment

Treatment for colorectal cancer in children may include the following:

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.

Symptoms and Diagnostic and Staging Tests

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 symptoms called carcinoid syndrome. Carcinoid syndrome caused by the hormone somatostatin may cause any of the following signs and symptoms. Check with your doctor if any of the following problems occur:

Other conditions that are not carcinoid tumors may cause these same symptoms.

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:

Treatment

Treatment for carcinoid tumors in children may include the following:

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 children older than 10 years.

Risk Factors 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. Symptoms of anemia include the following:

Other conditions that are not anemia caused by GIST may cause these same 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: