lunes, 8 de abril de 2019

Unusual Cancers of Childhood Treatment (PDQ®) 2/5 —Patient Version - National Cancer Institute

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

National Cancer Institute



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

Seven types of standard treatment are used:

Surgery

Surgery is a procedure used to find out whether cancer is present, to remove cancer from the body, or to repair a body part. Palliative surgery is done to relieve symptoms caused by cancer. Surgery is also called an operation.
After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.

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. There are different types of radiation therapy:
The way the radiation therapy is given depends on the type of cancer being treated.

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 affect cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, a body cavity such as the abdomen, or an organ, the drugs mainly affect cancer cells in those areas. Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Hormone therapy

Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances that are made by glands in the body and flow through the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working. Hormone therapy with drugs called corticosteroidsmay be used to treat thymoma or thymic carcinoma.
Hormone therapy with a somatostatin analogue (octreotide or lanreotide) may be used to treat neuroendocrine tumors that have spread or cannot be removed by surgery. This treatment stops extra hormones from being made by the neuroendocrine tumor. Octreotide or lanreotide are somatostatin analogues which are injected under the skin or into the muscle. Sometimes a small amount of a radioactive substance is attached to the drug and the radiation also kills cancer cells. This is called peptide receptor radionuclidetherapy.

Immunotherapy

Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or biologic therapy.
  • Interferon: Interferon affects the division of cancer cells and can slow tumor growth. It is used to treat nasopharyngeal cancer and papillomatosis.
  • Epstein-Barr virus (EBV)-specific cytotoxic T-lymphocytes: White blood cells (T-lymphocytes) are treated in the laboratory with Epstein-Barr virus and then given to the patient to stimulate the immune system and fight cancer. EBV-specific cytotoxic T-lymphocytes are being studied for the treatment of nasopharyngeal cancer.
  • Vaccine therapy: Vaccine therapy uses a substance to stimulate the immune system to destroy a tumor. Vaccine therapy is used to treat papillomatosis.
  • Immune checkpoint inhibitor therapy: Some types of immune cells, such as T cells, and some cancer cells have certain proteins, called checkpoint proteins, on their surface that keep immune responses in check. When cancer cells have large amounts of these proteins, they will not be attacked and killed by T cells. Immune checkpoint inhibitors block these proteins and the ability of T cells to kill cancer cells is increased. CTLA-4 is a type of immune checkpoint inhibitor.
    CTLA-4 is a protein on the surface of T cells that helps keep the body’s immune responses in check. When CTLA-4 attaches to another protein called B7 on a cancer cell, it stops the T cell from killing the cancer cell. CTLA-4 inhibitors attach to CTLA-4 and allow the T cells to kill cancer cells. Ipilimumab is a type of CTLA-4 inhibitor. Although its use has not been studied in children, ipilimumab may be considered for the treatment of high-risk melanoma that has been completely removed during surgery.

Watchful waiting

Watchful waiting is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. Watchful waiting may be used when the tumor is slow-growing or when it is possible the tumor may disappear without treatment.

Targeted therapy

Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Types of targeted therapies used to treat unusual childhood cancers include the following:
Targeted therapies are being studied in the treatment of other unusual cancers of childhood.

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.

Gene therapy

Gene therapy is a treatment in which foreign genetic material (DNA or RNA) is inserted into a person's cells to prevent or fight disease. Gene therapy is being studied in the treatment of papillomatosis.

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.
Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order 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 for unusual cancers of childhood may cause side effects.

For information about side effects that begin during treatment for cancer, see our Side Effects page.
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 the following:
  • Physical problems.
  • Changes in mood, feelings, thinking, learning, or memory.
  • Second cancers (new types of cancer).
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 cancers and cancer treatments. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information).

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:
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.
ENLARGERespiratory 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:
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:
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:
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:
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.
ENLARGEGastrointestinal (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.
ENLARGEAnatomy 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:
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:
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.
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