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This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http://cancer.gov or call 1-800-4-CANCER.
Unusual cancers of childhood are cancers rarely seen in children.
Cancer in children and adolescents is rare. Since 1975, the number of new cases of childhood cancer has slowly increased. Since 1975, the number of deaths from childhood cancer has decreased by more than half.
The unusual cancers discussed in this summary are so rare that most children's hospitals are likely to see less than a handful of some types in several years. Because the unusual cancers are so rare, there is not a lot of information about what treatment works best. A child's treatment is often based on what has been learned from treating other children. Sometimes, information is available only from reports of the diagnosis, treatment, and follow-up of one child or a small group of children who were given the same type of treatment.
Many different cancers are covered in this summary. They are grouped by where they are found in the body.
Tests are used to detect (find), diagnose, and stage unusual cancers of childhood.
Tests are done to detect, diagnose, and stage cancer. The tests used depend on the type of cancer. After cancer is diagnosed, tests are done to find out if cancer cells have spread from where the cancer began to other parts of the body. The process used to find out if cancer cells have spread to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan the best treatment.
The following tests and procedures may be used to detect, diagnose, and stage cancer:
There are three ways that cancer spreads in the body.
Cancer can spread through tissue, the lymph system, and the blood:
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.
The metastatic tumor is the same type of cancer as the primary tumor. For example, if thyroid cancer spreads to the lung, the cancer cells in the lung are actually thyroid cancer cells. The disease is metastatic thyroid cancer, not lung cancer.
There are different types of treatment for children with unusual cancers.
Different types of treatments are available for children with 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 unusual cancers should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
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 care providers who are experts in treating children with cancer and who specialize in certain areas of medicine. These may include the following specialists:
Nine types of standard treatment are used:
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 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:
Proton beam radiation therapy is a type of high-energy, external radiation therapy. A radiation therapy machine aims streams of protons (tiny, invisible, positively-charged particles) at the cancer cells to kill them. This type of treatment causes less damage to nearby healthy tissue.
The way the radiation therapy is given depends on the type of cancer being treated.
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.
High-dose chemotherapy with autologous stem cell rescue
High doses of chemotherapy are given to kill cancer cells. Healthy cells, including blood -forming cells, are also destroyed by the cancer treatment. Stem cell rescue is a treatment to replace the blood-forming cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.
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 corticosteroids may 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. Octreotide may also be used to treat thymoma that does not respond to other treatment. 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 radionuclide therapy.
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.
There are two types of immune checkpoint inhibitor therapy:
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 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.
Embolization is a treatment in which contrast dye and particles are injected into the hepatic artery through a catheter (thin tube). The particles block the artery, cutting off blood flow to the tumor. Sometimes a small amount of a radioactive substance is attached to the particles. Most of the radiation is trapped near the tumor to kill the cancer cells. This is called radioembolization.
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 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:
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).
See the PDQ summary on Childhood Nasopharyngeal Cancer Treatment for more information.
See the PDQ summary on Childhood Esthesioneuroblastoma Treatment for more information.
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 withNUTGene Changes (NUTMidline Carcinoma)
See the PDQ summary on Childhood Midline Tract Carcinoma with NUT Gene Changes Treatment for more information.
See the PDQ summary on Childhood Breast Cancer Treatment for more information.
See the following PDQ summaries for more information:
See the PDQ summary on Childhood Esophageal Cancer Treatment for more information.
Thymoma and Thymic Carcinoma
See the PDQ summary on Childhood Thymoma and Thymic Carcinoma Treatment for more information.
Cardiac (Heart) Tumors
See the PDQ summary on Childhood Cardiac (Heart) Tumors Treatment for more information.
See the PDQ summary on Childhood Mesothelioma Treatment for more information.
See the PDQ summary on Childhood Adrenocortical Carcinoma Treatment for more information.
Stomach (Gastric) Cancer
See the PDQ summary on Childhood Stomach (Gastric) Cancer Treatment for more information.
See the PDQ summary on Childhood Pancreatic Cancer Treatment for more information.
See the PDQ summary on Childhood Colorectal Cancer Treatment for more information.
Gastrointestinal Carcinoid Tumors
See the PDQ summary on Childhood Gastrointestinal Carcinoid Tumors Treatment for more information.
Gastrointestinal Stromal Tumors
See the PDQ summary on Childhood Gastrointestinal Stromal Tumors Treatment for more information.
See the PDQ summary on Childhood Bladder Cancer Treatment for more information.
See the PDQ summary on Childhood Testicular Cancer Treatment for more information.
See the PDQ summary on Childhood Ovarian Cancer Treatment for more information.
Cervical and Vaginal Cancer
See the PDQ summary on Childhood Cervical and Vaginal Cancer Treatment for more information.
Multiple Endocrine Neoplasia Syndromes
See the PDQ summary on Childhood Multiple Endocrine Neoplasia (MEN) Syndromes Treatment for more information.
Pheochromocytoma and Paraganglioma
See the PDQ summary on Childhood Pheochromocytoma and Paraganglioma Treatment for more information.
Skin Cancer (Melanoma, Basal Cell Carcinoma, and Squamous Cell Carcinoma of the Skin)
Intraocular (Uveal) Melanoma
See the PDQ summary on Childhood Intraocular (Uveal) Melanoma Treatment for more information.
See the PDQ summary on Childhood Chordoma Treatment for more information.
Carcinoma of Unknown Primary
See the PDQ summary on Childhood Carcinoma of Unknown Primary Treatment for more information.
For more information from the National Cancer Institute about unusual cancers of childhood, see the following:
For more childhood cancer information and other general cancer resources, see the following:
Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government's center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of unusual cancers of childhood. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
Permission to Use This Summary
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as "NCI's PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary]."
The best way to cite this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Unusual Cancers of Childhood Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/childhood-cancers/patient/unusual-cancers-childhood-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389276]
Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
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Last Revised: 2019-12-26
If you want to know more about cancer and how it is treated, or if you wish to know about clinical trials for your type of cancer, you can call the NCI's Cancer Information Service at 1-800-422-6237, toll free. A trained information specialist can talk with you and answer your questions.
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