Unusual Cancers of Childhood Treatment (PDQ®)—Patient Version - National Cancer Institute

Unusual Cancers of Childhood Treatment (PDQ®)–Patient Version

Unusual Cancers of the Head and Neck

Nasopharyngeal Cancer

See the PDQ summary on Childhood Nasopharyngeal Cancer Treatment for more information.

Esthesioneuroblastoma

See the PDQ summary on Childhood Esthesioneuroblastoma Treatment for more information.

Thyroid Tumors

See the PDQ summary on Childhood Thyroid Cancer Treatment for more information.

Oral Cavity Cancer

See the PDQ summary on Childhood Oral Cavity Cancer Treatment for more information.

Salivary Gland Tumors

See the PDQ summary on Childhood Salivary Gland Tumors Treatment for more information.

Laryngeal Cancer and Papillomatosis

See the PDQ summary on Childhood Laryngeal Tumors Treatment for more information.

Midline Tract Cancer with NUT Gene Changes (NUT Midline Carcinoma)

See the PDQ summary on Childhood Midline Tract Carcinoma with NUT Gene Changes Treatment for more information.

Unusual Cancers of the Chest

Breast Cancer

Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. Breast cancer may occur in both male and female children.

Breast cancer is the most common cancer among females aged 15 to 39 years. Breast cancer in this age group is more aggressive and more difficult to treat than in older women. Treatments for younger and older women are similar. Younger patients with breast cancer may have genetic counseling (a discussion with a trained professional about inherited diseases) and testing for familial cancer syndromes. Also, the possible effects of treatment on fertility should be considered.

Most breast tumors in children are fibroadenomas, which are benign (not cancer). Rarely, these tumors become large phyllodes tumors (cancer) and begin to grow quickly. If a benign tumor begins to grow quickly, a fine-needle aspiration (FNA) biopsy or an excisional biopsy will be done. The tissues removed during the biopsy will be viewed under a microscope by a pathologist to check for signs of cancer.

Risk Factors, Signs, and Diagnostic and Staging Tests

The risk of breast cancer is increased by the following:

Having a personal history of a type of cancer that may spread to the breast, such as leukemia, rhabdomyosarcoma, soft tissue sarcoma, or lymphoma.
Past treatment for another cancer, such as Hodgkin lymphoma, with radiation therapyto the breast or chest.

Breast cancer may cause any of the following signs. Check with your child’s doctor if your child has any of the following:

A lump or thickening in or near the breast or in the underarm area.
A change in the size or shape of the breast.
A dimple or puckering in the skin of the breast.
A nipple turned inward into the breast.
Scaly, red, or swollen skin on the breast, nipple, or areola (the dark area of skin that is around the nipple).
Dimples in the breast that look like the skin of an orange, called peau d’orange.

Other conditions that are not breast cancer may cause these same signs.

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

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

Another test used to diagnose breast cancer is the mammogram (an x-ray of the breast). When treatment for another cancer included radiation therapy to the breast or chest, it is important to have a mammogram and MRI of the breast to check for breast cancer. These should be done beginning at age 25, or 10 years after finishing radiation therapy, whichever is later.

Treatment

For information about the treatments listed below, see the Treatment Option Overviewsection.

Treatment of breast cancer in children may include the following:

Watchful waiting for benign tumors.
Surgery to remove the tumor, but not the whole breast. Radiation therapy may also be given.

Treatment of recurrent breast 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 Breast Cancer Treatment for more information on the treatment of adolescents and young adults with breast cancer.

Lung Cancer

Lung cancer begins in the tissue of the lung. The lungs are a pair of cone-shaped breathing organs in the chest. The lungs bring oxygen into the body as you breathe in. They release carbon dioxide, a waste product of the body’s cells, as you breathe out. Each lung has sections called lobes. The left lung has two lobes. The right lung is slightly larger and has three lobes. Two tubes called bronchi lead from the trachea (windpipe) to the right and left lungs. Tiny air sacs called alveoli and small tubes called bronchioles make up the inside of the lungs.

In children, most lung tumors are malignant (cancer). The most common lung tumors are tracheobronchial tumors and pleuropulmonary blastoma.

Tracheobronchial Tumors

Tracheobronchial tumors begin in the cells that line the surface of the lung. Most tracheobronchial tumors in children are benign and occur in the trachea or large bronchi(large airways of the lung). Sometimes, a slow-growing tracheobronchial tumor becomes cancer that may spread to other parts of the body.

Respiratory anatomy; drawing shows right lung with upper, middle, and lower lobes; left lung with upper and lower lobes; and the trachea, bronchi, lymph nodes, and diaphragm. Inset shows bronchioles, alveoli, artery, and vein. — Anatomy of the respiratory system, showing the trachea and both lungs and their lobes and airways. Lymph nodes and the diaphragm are also shown. Oxygen is inhaled into the lungs and passes through the thin membranes of the alveoli and into the bloodstream (see inset).

Signs and Symptoms

Tracheobronchial tumors may cause any of the following signs and symptoms. Check with your child's doctor if your child has any of the following:

Coughing.
Wheezing.
Trouble breathing.
Spitting up blood from the airways or lung.
Frequent infections in the lung, such as pneumonia.

Other conditions that are not tracheobronchial tumors may cause these same signs and symptoms. For example, symptoms of tracheobronchial tumors are a lot like the symptoms of asthma, and that can make it hard to diagnose the tumor.

Diagnostic and Staging Tests

Tests to diagnose and stage tracheobronchial tumors may include the following:

Physical exam and history.
X-ray of the chest.
CT scan.

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

A biopsy of the abnormal area is usually not done because it can cause severe bleeding.

Other tests used to diagnose tracheobronchial tumors include the following:

Bronchography: 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. A contrast dye is put through the bronchoscope to make the larynx, trachea, and airways show up more clearly on x-ray film.
Octreotide scan : A type of radionuclide scan used to find carcinoid and other types of 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.

Prognosis

The prognosis (chance of recovery) for children with tracheobronchial cancer is very good, unless the child has rhabdomyosarcoma.

Treatment

For information about the treatments listed below, see the Treatment Option Overviewsection.

The treatment of tracheobronchial tumors depends on the type of cell the cancer formed from. Treatment of tracheobronchial tumors in children may include the following:

Surgery to remove the tumor. Sometimes a type of surgery called a sleeve resection is used. The lymph nodes and vessels where cancer has spread are also removed.
Chemotherapy.
Radiation therapy.
Targeted therapy (crizotinib).

Treatment of recurrent tracheobronchial 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 Neuroendocrine Tumors (Carcinoid Tumors) section of this summary for more information.

Pleuropulmonary Blastoma

Pleuropulmonary blastomas (PPBs) form in the tissue of the lung and pleura (tissue that covers the lungs and lines the inside of the chest). PPBs can also form in the organsbetween the lungs including the heart, aorta, and pulmonary artery, or in the diaphragm(the main breathing muscle below the lungs).

There are three types of PPB:

Type I tumors are cyst -like tumors in the lung. They are most common in children aged 2 years and younger and can usually be cured. Type Ir tumors are Type I tumors that have gotten smaller or have not grown or spread.
Type II tumors are cyst-like with some solid parts. These tumors sometimes spread to the brain.
Type III tumors are solid tumors. These tumors often spread to the brain.

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

The risk of PPB is increased by the following:

Having pleuropulmonary blastoma familial cancer syndrome.
Having a certain change in the DICER1 gene.

PPB may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

A cough that doesn’t go away.
Trouble breathing.
Fever.
Lung infections, such as pneumonia.
Wheezing.
Pain in the chest or abdomen.
Loss of appetite.
Weight loss for no known reason.
Feeling very tired.

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

Tests to diagnose and stage PPB may include the following:

Physical exam and history.
X-ray of the chest.
CT scan.
PET scan.

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

Other tests used to diagnose PPB include the following:

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. In some cases, this procedure is used to remove part of the esophagus or lung. If the thoracoscope cannot reach certain tissues, organs, or lymph nodes, a thoracotomy may be done. In this procedure, a larger incision is made between the ribs and the chest is opened.

PPBs may spread or recur (come back) even after being removed by surgery.

Prognosis

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

The type of pleuropulmonary blastoma.
Whether the tumor has spread to other parts of the body at the time of diagnosis.
Whether the tumor was completely removed by surgery.

Treatment

For information about the treatments listed below, see the Treatment Option Overviewsection.

Treatment of pleuropulmonary blastoma in children includes the following:

Surgery to remove the whole lobe of the lung the tumor is in, with or without chemotherapy.

Treatment of recurrent pleuropulmonary blastoma in children may include the following:

A clinical trial of targeted therapy using a monoclonal antibody.
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.

Esophageal Tumors

Esophageal tumors may be benign (not cancer) or malignant (cancer). Esophageal cancer is a disease in which malignant 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. Most esophageal tumors in children begin in the thin, flat cells that line the esophagus.

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.

Signs and Symptoms

Esophageal cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

Trouble swallowing.
Streaks of blood in sputum (mucus coughed up from the lungs).
Weight loss.
Hoarseness and cough.
Indigestion and heartburn.
Vomiting with streaks of blood.

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

Diagnostic and Staging Tests

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

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

Other tests used to diagnose esophageal cancer include the following:

Esophagoscopy : A procedure to look inside the esophagus to check for abnormalareas. 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 lungfor 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.
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.

Prognosis

Esophageal cancer is hard to cure because it usually is not possible to remove the whole tumor by surgery.

Treatment

For information about the treatments listed below, see the Treatment Option Overviewsection.

Treatment of 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 all or part of the tumor.

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.

See the PDQ summary on adult Esophageal Cancer for more information.

Thymoma

Thymoma is a rare tumor of the cells that cover the outside surface of the thymus. The thymus is a small organ in the upper chest under the breastbone. It is part of the lymph system and makes white blood cells, called lymphocytes, that help fight infection. Thymoma usually forms in the front part of the chest and is often found during a chest x-ray that is done for another reason.

Anatomy of the thymus gland; drawing shows the thymus gland in the upper chest under the breastbone. Also shown are the ribs, lungs, and heart. — Anatomy of the thymus gland. The thymus gland is a small organ that lies in the upper chest under the breastbone. It makes white blood cells, called lymphocytes, which protect the body against infections.

Thymoma is a slow-growing tumor that usually does not spread to the lymph nodes or to other parts of the body. Most children are diagnosed with thymoma at an early stage.

Other types of tumors, such as lymphoma or germ cell tumors, may form in the thymus but they are not considered to be thymoma.

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

People who develop thymoma often have one of the following immune system diseases or hormone disorders:

Thymoma may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

Coughing.
Trouble swallowing.
Hoarseness.
Pain or a tight feeling in the chest.
Trouble breathing.
Fever.
Weight loss.

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

Tests to diagnose and stage thymoma may include the following:

Physical exam and history.
X-ray of the chest.
CT scan.
PET scan.
MRI.
Biopsy.

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

Prognosis

The prognosis (chance of recovery) is better when the tumor has not spread. Childhood thymoma is usually diagnosed before the tumor has spread.

Treatment

For information about the treatments listed below, see the Treatment Option Overviewsection.

Treatment of thymoma in children may include the following:

Surgery to remove as much of the tumor as possible.
Radiation therapy.
Chemotherapy.

Treatment of recurrent thymoma 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 Thymoma and Thymic Carcinoma Treatment for more information.

Thymic Carcinoma

Thymic carcinoma is a rare cancer of the cells that cover the outside surface of the thymus. The thymus is a small organ in the upper chest under the breastbone. It is part of the lymph system and makes white blood cells, called lymphocytes, that help fight infection. Thymic carcinoma usually forms in the front part of the chest and is often found during a chest x-ray that is done for another reason.

Thymic carcinoma is more likely than thymoma to spread to the lymph nodes or to other parts of the body by the time it is diagnosed.

Other types of tumors, such as lymphoma or germ cell tumors, may form in the thymus but they are not considered to be thymic carcinoma or thymoma. (See the Thymomasection above for more information).

Treatment

For information about the treatments listed below, see the Treatment Option Overviewsection.

Thymic carcinoma can rarely be completely removed by surgery and is likely to recur (come back) after treatment.

Treatment of thymic carcinoma in children may include the following:

Surgery to remove as much of the tumor as possible.
Radiation therapy.
Chemotherapy.
Targeted therapy (sunitinib).

Treatment of recurrent thymic 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 Thymoma and Thymic Carcinoma Treatment for more information.

Heart Tumors

Most tumors that form in the heart are benign (not cancer). Benign heart tumors that may appear in children include the following:

Rhabdomyoma: A tumor that forms in muscle made up of long fibers.
Myxoma: A tumor that may be part of an inherited syndrome called Carney complex. (See the Multiple Endocrine Neoplasia Syndromes section for more information.)
Teratomas: A type of germ cell tumor. In the heart, these tumors form most often in the pericardium (the sac that covers the heart). Some teratomas are malignant(cancer).
Fibroma: A tumor that forms in fiber -like tissue that holds bones, muscles, and other organs in place.
Histiocytoid cardiomyopathy tumor: A tumor that forms in the heart cells that control heart rhythm.
Hemangiomas: A tumor that forms in the cells that line blood vessels.
Neurofibroma: A tumor that forms in the cells and tissues that cover nerves.

Before birth and in newborns, the most common benign heart tumors are teratomas. An inherited condition called tuberous sclerosis can cause heart tumors to form in a fetus or newborn.

Malignant tumors that begin in the heart are even more rare than benign heart tumors in children. Malignant heart tumors include:

Malignant teratoma.
Lymphoma.
Rhabdomyosarcoma: A cancer that forms in muscle made up of long fibers.
Angiosarcoma: A cancer that forms in cells that line blood vessels or lymph vessels.
Chondrosarcoma: A type of cancer that usually forms in bone cartilage but very rarely can begin in the heart.
Infantile fibrosarcoma.
Synovial sarcoma: A cancer that usually forms around joints but may very rarely form in the heart or sac around the heart.

Signs and Symptoms

Heart tumors may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

Change in the heart's normal rhythm.
Trouble breathing, especially when the child is lying down.
Pain in the middle of the chest that feels better when the child is sitting up.
Coughing.
Fainting.
Feeling dizzy, tired, or weak.
Fast heart rate.
Swelling in the legs, ankles, or abdomen.
Feeling anxious.
Signs of a stroke.
- Sudden numbness or weakness of the face, arm, or leg (especially on one side of the body).
- Sudden confusion or trouble speaking or understanding.
- Sudden trouble seeing with one or both eyes.
- Sudden trouble walking or feeling dizzy.
- Sudden loss of balance or coordination.
- Sudden severe headache for no known reason.

Sometimes heart tumors do not cause any signs or symptoms.

Other conditions that are not heart tumors may cause these same signs and symptoms.

Diagnostic and Staging Tests

Tests to diagnose and stage heart tumors may include the following:

Physical exam and history.
X-ray of the chest.
CT scan.
MRI.

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

Other tests used to diagnose or stage heart tumors include the following:

Echocardiogram : A procedure in which high-energy sound waves (ultrasound) are bounced off the heart and nearby tissues or organs and make echoes. A moving picture is made of the heart and heart valves as blood is pumped through the heart.
Electrocardiogram (EKG): A recording of the heart's electrical activity to check its rate and rhythm. A number of small pads (electrodes) are placed on the patient’s chest, arms, and legs, and are connected by wires to the EKG machine. Heart activity is then recorded as a line graph on paper. Electrical activity that is faster or slower than normal may be a sign of heart disease or damage.

Treatment

For information about the treatments listed below, see the Treatment Option Overviewsection.

Treatment of heart tumors in children may include the following:

Watchful waiting for benign tumors of heart muscle (rhabdomyomas), which sometimes shrink and go away on their own.
Surgery (which may include removing some or all of the tumor or a heart transplant) and chemotherapy.
Targeted therapy for patients who also have tuberous sclerosis.

Treatment of recurrent heart 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.

Mesothelioma

Malignant mesothelioma is a disease in which malignant (cancer) cells are found in the pleura (the thin layer of tissue that lines the chest cavity and covers the lungs) or the peritoneum (the thin layer of tissue that lines the abdomen and covers most of the organsin the abdomen). The tumors often spread over the surface of organs without spreading into the organ. They may spread to lymph nodes nearby or in other parts of the body. Malignant mesothelioma may also form in the heart or testicles, but this is rare.

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

Mesothelioma is sometimes a late effect of treatment for an earlier cancer, especially after treatment with radiation therapy. In adults, mesothelioma has been linked to being exposed to asbestos, which was once used as building insulation. There is no information about the risk of mesothelioma in children exposed to asbestos.

Mesothelioma may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

Trouble breathing.
Pain under the rib cage.
Weight loss for no known reason.

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

Tests to diagnose and stage mesothelioma may include the following:

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

Other tests used to diagnose mesothelioma include the following:

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. In some cases, this procedure is used to remove part of the esophagus or lung.
Thoracotomy : An incision (cut) is made between two ribs to check inside the chest for signs of disease.
Cytologic exam: An exam of cells under a microscope (by a pathologist) to check for anything abnormal. For mesothelioma, fluid is taken from around the lungs or from the abdomen. A pathologist checks the cells in the fluid.

Prognosis

The prognosis (chance of recovery) is better when the tumor has not spread.

Treatment

For information about the treatments listed below, see the Treatment Option Overviewsection.

Treatment of mesothelioma in children may include the following:

Surgery to remove the part of the chest lining with cancer and some of the healthy tissue around it.
Chemotherapy.
Radiation therapy, as palliative therapy, to relieve pain and improve quality of life.

Treatment of recurrent mesothelioma 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 Malignant Mesothelioma Treatment for more information.