Esophageal cancer is a disease in which malignant (cancer) cells form in the tissues of the esophagus.

The esophagus is the hollow, muscular tube that moves food and liquid from the throat to the stomach. The wall of the esophagus is made up of several layers of tissue, including mucous membrane, muscle, and connective tissue.

The two most common forms of esophageal cancer are named after the type of cells where the cancer started.

• Squamous cell carcinoma: Most esophageal tumors in children begin in the thin, flat cells that line the inside of the esophagus (called squamous cell carcinoma of the esophagus) and spread outward through the other layers as the tumor grows.
• Adenocarcinoma: Some esophageal tumors begin in the mucus-secreting glands of the esophagus (called adenocarcinoma of the esophagus).

Esophageal tumors may be benign (not cancer) or malignant (cancer).

Certain factors affect prognosis (chance of recovery).

Prognosis depends on the following:

• The type of esophageal cancer (squamous cell or adenocarcinoma).
• Whether the cancer was completely removed by surgery.
• Whether the cancer has spread to other areas of the body.
• Whether the cancer has just been diagnosed or has recurred (come back).

Esophageal cancer is hard to cure because it usually cannot be completely removed by surgery.

Having gastroesophageal reflux or Barrett esophagus may increase the risk of esophageal cancer.

Anything that increases a person's chance of getting a disease is called a risk factor. Not every child with one or more of these risk factors will develop esophageal cancer, and it will develop in some children who don't have any known risk factors. Talk with your child's doctor if you think your child may be at risk.

Risk factors for esophageal cancer include the following:

• Having gastroesophageal reflux.
• Having Barrett esophagus.
• Swallowing chemicals, which may burn the esophagus.

Signs and symptoms of esophageal cancer include trouble swallowing and weight loss.

These and other signs and symptoms may be caused by esophageal cancer or by other conditions.

Check with your child's doctor if your child has any of the following:

• Trouble swallowing.
• Weight loss.
• Feeling tired and weak.
• Indigestion and heartburn.
• Reflux.
• Nausea or vomiting.

Tests that examine the esophagus are used to diagnose esophageal cancer.

In addition to asking about your child's personal and family health history and doing a physical exam, your child's doctor may perform the following tests and procedures.

• Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
• PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.

• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of the esophagus. This procedure is also called nuclear magnetic resonance imaging (NMRI).
• Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
• 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.
• Esophagoscopy: A procedure to look inside the esophagus to check for abnormal areas. An esophagoscope is inserted through the mouth or nose and down the throat into the esophagus. An esophagoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. A biopsy is usually done during an esophagoscopy. Sometimes a biopsy shows changes in the esophagus that are not cancer but may lead to cancer.
• Bronchoscopy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
• Thoracoscopy: A surgical procedure to look at the organs inside the chest to check for abnormal areas. An incision (cut) is made between two ribs and a thoracoscope is inserted into the chest. A thoracoscope 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 cancer. Sometimes this procedure is used to remove part of the esophagus or lung.
• Immunohistochemistry: A laboratory test that uses antibodies to check for certain antigens (markers) in a sample of a patient’s cells. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to the antigen in the sample of the patient’s cells, the enzyme or dye is activated, and the antigen can then be seen under a microscope.

There is no standard staging system for childhood esophageal cancer.

The process used to find out if cancer has spread from the esophagus to nearby areas or to other parts of the body is called staging. There is no standard staging system for childhood esophageal cancer. The results of tests and procedures done to diagnose esophageal cancer are used to help make decisions about treatment.

Sometimes esophageal cancer recurs (comes back) after treatment. The cancer may come back in the esophagus or in other parts of the body after it has been treated.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

• Tissue. The cancer spreads from where it began by growing into nearby areas.
• Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
• Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

• Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
• Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if esophageal cancer spreads to the lung, the cancer cells in the lung are actually esophageal cancer cells. The disease is metastatic esophageal cancer, not lung cancer.

There are different types of treatment for children with esophageal cancer.

Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.

Children with esophageal cancer should have their treatment planned by a team of doctors who are experts in treating childhood cancer.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health professionals who are experts in treating children with cancer and who specialize in certain areas of medicine. This may include the following specialists and others:

• Pediatrician.
• Pediatric surgeon.
• Radiation oncologist.
• Pathologist.
• Pediatric nurse specialist.
• Social worker.
• Rehabilitation specialist.
• Psychologist.
• Child-life specialist.

Three types of standard treatment are used:

Surgery

Surgery to remove as much of the tumor as possible is done.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. In esophageal cancer, a plastic or metal tube is passed through the mouth and into the esophagus. A machine that is outside the body has a special tool that is placed in the tube to send radiation toward the area of the body with cancer.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy).

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapy is being studied for the treatment of childhood esophageal cancer that has recurred (come back).

Treatment for childhood esophageal cancer may cause side effects.

Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:

• Physical problems, such as narrowing of the esophagus.
• Changes in mood, feelings, thinking, learning, or memory.
• Second cancers (new types of cancer) or other conditions.

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer may be repeated to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment of Childhood Esophageal Cancer

Treatment of newly diagnosed esophageal cancer in children may include the following:

• Radiation therapy given through a plastic or metal tube placed through the mouth into the esophagus.
• Chemotherapy.
• Surgery to remove as much as the tumor as possible. The entire tumor can rarely be removed.

Treatment of Recurrent Childhood Esophageal Cancer

Treatment of recurrent esophageal 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.