Heart Tumors in Children

How Do Heart Tumors Affect Children?

A heart tumor is an abnormal growth that can occur in the heart muscle or one of its chambers. Parents should know that heart tumors in children are rare, affecting around 1% of newborns, and most are not cancerous. In fact, fewer than 1% of all heart tumors are cancerous.

But tumors of the heart can still be concerning because of their potential for affecting the heart’s functions. A tumor can block the valves in the heart and put pressure on the arteries and veins around the heart. It can also interfere with the heart’s electrical system and more.

Heart Tumor Symptoms in Children

A tumor of the heart may not always have visible signs and symptoms. The heart tumor symptoms that most commonly occur in children include:

• A heart murmur, which is an abnormal sound made by blood moving through the heart’s chambers or valves when heard through a stethoscope
• Arrhythmia, an abnormal heart rhythm
• Trouble breathing, especially while lying down
• Chest pain or tightness that eases when the child sits up
• Unexplained weight loss, fatigue (tiredness), joint or muscle pain, and frequent fevers — especially in older children

If the tumor has caused heart failure, related heart tumor symptoms include:

• Difficulty breathing and eating
• Excessive sweating
• Poor growth

Types of Heart Tumors in Kids

Most heart tumors in children are benign (noncancerous). The most common benign heart tumors found in children include:

Rhabdomyoma

Cardiac rhabdomyomas are the most common benign heart tumors in children, accounting for about 60% of all pediatric cardiac tumors. They occur most frequently in infants less than a year old. They are generally discovered in the lower chambers of the heart and form in muscle composed of long fibers. Rhabdomyomas sometimes shrink or go away on their own with no treatment needed.

Myxoma

Myxoma tumors are most often located in the heart’s upper chamber. They are more typical in teenagers and adults than in children. Because they can substantially interfere with heart function and potentially cause arrhythmias, surgery is typically needed to remove them.

Fibroma

Fibromas are tumors that develop most frequently on the inner wall of the right or left lower chambers of the heart or in the wall between them known as the septum. Typically detected soon after a baby is born, fibromas are removed via surgery quickly thereafter because of their potential for causing arrhythmia, an irregular heart rhythm.

Pericardial Teratoma

This type of tumor is typically found at the base of the aorta and pulmonary artery where they connect to the heart. Teratomas can put pressure on the arteries, cut off blood flow and cause other problems as well.

Malignant (cancerous) heart tumors are even rarer than benign heart tumors in children. The most commonly diagnosed malignant heart tumors are:

Rhabdomyosarcoma

While very rare, rhabdomyosarcoma is the most common type of malignant soft tissue tumor in children and is typically diagnosed in kids under age 10. Rhabdomyosarcoma is caused by genetic changes in tumor cells and can form anywhere in the body — not just in the heart.

Fibrosarcoma

Forming in connective tissue throughout the body (including the heart), fibrosarcoma is typically seen in newborns and young children but can also be detected before birth via ultrasound. Fibrosarcoma tumors are frequently large and grow quickly but usually don’t spread throughout the body.

Risk Factors and Causes of Heart Tumors in Children

While causes of heart tumors vary, abnormal heart tissue growth is thought to be the cause for most. Rhabdomyomas — the most common tumor type — are often caused by tuberous sclerosis, an inherited condition that can cause tumors to form in the heart as well as the kidneys, brain, eyes, lungs and skin.

Carney complex is also a known risk factor in the development of myxoma tumors. More frequently found in teenagers than young children, Carney complex is a rare disorder that typically causes unusual skin pigmentation as well as an increased risk for multiple types of tumors beyond the heart, including tumors in the endocrine glands.

Diagnosing Heart Tumors

A variety of tests and tools can be used to diagnose tumors of the heart in children both before and after birth. Tumors can be diagnosed prenatally (before birth) via a fetal echocardiogram. This test uses sound waves to create an image of the heart. A regular echocardiogram may be used to diagnose a heart tumor after birth.

Other diagnostic tools may include:

• A health history and physical exam to check for disease indications or any unusual signs, like lumps
• An X-ray of the chest
• Electrocardiogram (EKG), which records the heart’s electrical activity
• Cardiac MRI, which can provide 3D images that highlight any abnormalities
• Cardiac catheterization, where a thin tube is inserted into the heart through a vein or artery in the leg or through the belly button. This procedure is rare for heart tumors.

Treatment for Heart Tumors in Children

When it comes to heart tumors in children, the good news is that most are benign with no symptoms and don’t require treatment. For benign heart tumors that do need treatment, a wide range of options is available. They vary based on multiple factors, including the tumor’s type and size and how much it’s affecting heart function.

For example, “watchful waiting” is a common first course of treatment for rhabdomyomas, the most common heart tumors, as they often shrink and may disappear over time on their own. A physician may monitor the tumor to see if it’s shrinking. It will likely not require surgical removal unless it begins to interfere with heart function.

Surgery may be required in cases where a tumor is blocking the heart’s normal functioning. A biopsy may be needed to remove and examine the tumor under a microscope to determine if it’s malignant or benign. During surgery to remove the tumor, the surgeon will also make repairs to the heart as needed to ensure proper functioning. If a tumor has caused extensive damage to the heart, a transplant may be necessary. For malignant tumors, treatment may include watchful waiting, chemotherapy, surgery, radiation therapy and targeted drug therapies.

So, can heart tumors be “cured”? Benign tumors like rhabdomyomas can be managed and typically don’t cause issues later in life while others like myxomas can be completely removed via surgery.

Long-Term Outlook for Heart Tumors in Children

Children who have a heart tumor (or who’ve had one removed) will need lifelong follow-up care. This care helps ensure that tumors don’t recur and that the heart stays in working order. If the tumor has caused an issue such as arrhythmia, it will need to be managed through lifelong care and may require medications or placement of a pacemaker (a device that helps the heart beat faster). Checkups with a pediatric cardiologist will be needed at least once a year.

10 Questions About Heart Tumors to Ask Your Doctor

If your child has been diagnosed with a heart tumor, it can be difficult to know what questions to ask to ensure they get the care they need (and you get some peace of mind). Here are a few essential questions to ask your doctor.

1. How many tumors are there? One or multiple?
2. What type of tumor is it?
3. Where is the tumor located — inside or around the heart?
4. Is the tumor causing issues with blood flow to or within the heart?
5. Could the tumor be malignant? What are the chances?
6. Because tumors can cause other problems like arrhythmia, does my child need to be tested for them?
7. Could the tumor be due to genetics? Does treatment require a genetics specialist?
8. What is the recommended treatment for the type of tumor my child has?
9. Can the tumor be biopsied or removed?
10. What is the long-term outlook for my child’s health?

Heart Tumor Care at Rady Children’s

Rady Children’s Heart Institute provides top-ranked, personalized heart care for infants, children and adults with congenital heart defects or acquired heart disease. We offer a full range of heart surgery and care. We are a major referral center for Imperial, Riverside and Orange counties as well as the Pacific Rim. Our cardiologists and heart surgeons are highly experienced and skilled, and will be with you every step of the way on your child’s journey.

Rady Children’s is a member of the exclusive U.S. News & World Report Best Children’s Hospitals Honor Roll, and our heart program is ranked among the top 10 in the nation for pediatric cardiology and heart surgery.

To learn more about heart tumor diagnosis, treatment and surgery at Rady Children’s, call us at 858-966-5855.