General Information About Childhood Cardiac (Heart) Tumors
Childhood cardiac tumors, which may be benign or malignant, form in the heart.
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 PDQ summary on Childhood Multiple Endocrine Neoplasia Syndromes 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 an unborn baby (fetus) or newborn.
Cardiac tumors are rare tumors that form in the tissues of the heart, including muscle tissue, connective tissue, and tissues that line the blood vessels, control heart rhythm, and cover the nerves of the heart. They may also form in the pericardium (the sac around the heart). Most cardiac tumors are benign (not cancer), but some may be malignant (cancer).
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.
- Undifferentiated pleomorphic sarcoma: A cancer that usually forms in the soft tissue, but it may also form in bone.
- Leiomyosarcoma: A cancer that forms in smooth muscle cells.
- Chondrosarcoma: A cancer that usually forms in bone cartilage, but very rarely can begin in the heart.
- Synovial sarcoma: A cancer that usually forms around joints, but may very rarely form in the heart or sac around the heart.
- Infantile fibrosarcoma: A cancer that forms in fiber-like tissue that holds bones, muscles, and other organs in place.
When cancer begins in another part of the body and spreads to the heart, it is called metastatic cancer. Some types of cancer, such as sarcoma, melanoma, and leukemia, start in other parts of the body and spread to the heart. This summary is about cancer that first forms in the heart, not metastatic cancer.
Signs and symptoms of a heart tumor include a change in the heart's normal rhythm and trouble breathing.
These and other signs and symptoms may be caused by heart tumors or by other conditions.
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 or tightness 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.
Tests that examine the heart are used to diagnose a heart tumor.
The following tests and procedures may be used:
- Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
- 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.
- MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
- 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.
- Cardiac catheterization: A procedure to look inside the blood vessels and heart for abnormal areas or cancer. A long, thin, catheter is inserted into an artery or vein in the groin, neck, or arm and threaded through the blood vessels to the heart. A sample of tissue may be removed using a special tool. A pathologist views the tissue under a microscope to look for cancer cells.
Stages of Heart Tumors
The process used to find out if malignant heart tumors (cancer) have spread from the heart to nearby areas or other parts of the body is called staging. There is no standard staging system for malignant childhood heart tumors. The results of tests and procedures done to diagnose malignant heart tumors are used to help make decisions about treatment.
Sometimes malignant childhood heart tumors recur (come back) after treatment.
Treatment Option Overview
There are different types of treatment for children with heart tumors.
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 heart tumors should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
Treatment of malignant heart tumors 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 heart surgeon.
- Pediatric cardiologist.
- Radiation oncologist.
- Pathologist.
- Pediatric nurse specialist.
- Social worker.
- Rehabilitation specialist.
- Psychologist.
- Child-life specialist.
Five types of treatment are used:
Watchful waiting
Watchful waiting is closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change. This treatment may be used for rhabdomyoma.
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).
Surgery
When possible, the cancer is removed by surgery. Types of surgery that may be done include the following:
- Surgery to remove the tumor and some healthy tissue around it.
- Heart transplant. If the patient is waiting for a donated heart, other treatment is given as needed.
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. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer.
Targeted therapy
Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.
- mTOR inhibitor therapy: These drugs stop cells from dividing and may prevent the growth of new blood vessels that tumors need to grow. Everolimus is used to treat children who have rhabdomyoma and tuberous sclerosis.
Targeted therapy is also being studied for the treatment of malignant childhood heart tumors that have recurred (come back).
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Treatment for childhood heart tumors 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:
- Physical problems.
- 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. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.
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 of Childhood Heart Tumors
For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of newly diagnosed childhood heart tumors may include the following:
- Watchful waiting, for rhabdomyoma, which sometimes shrinks and goes away on its own.
- Targeted therapy (everolimus) for patients who have rhabdomyoma and tuberous sclerosis.
- Chemotherapy followed by surgery (which may include removing some or all of the tumor or a heart transplant), for sarcomas.
- Surgery alone, for other tumor types.
- Radiation therapy for tumors that cannot be removed by surgery.
Treatment of Recurrent Childhood Heart Tumors
For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of malignant recurrent childhood tumors 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.
To Learn More About Childhood Heart Tumors
For more information from the National Cancer Institute about childhood heart tumors, see the following:
- Computed Tomography (CT) Scans and Cancer
- Targeted Cancer Therapies
For more childhood cancer information and other general cancer resources, see the following:
- About Cancer
- Childhood Cancers
- CureSearch for Children's Cancer
- Late Effects of Treatment for Childhood Cancer
- Adolescents and Young Adults with Cancer
- Children with Cancer: A Guide for Parents
- Cancer in Children and Adolescents
- Staging
- Coping with Cancer
- Questions to Ask Your Doctor about Cancer
- For Survivors and Caregivers
About This PDQ Summary
About PDQ
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.
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Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood cardiac (heart) tumors. 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.
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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).
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The best way to cite this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Cardiac (Heart) Tumors Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/cardiac/patient-child-cardiac-treatment-pdq. Accessed <MM/DD/YYYY>.
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Last Revised: 2021-05-20
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