Heart Tumors in Children

Reviewed on 12/9/2022
Heart Tumors in Children
Most cardiac tumors in children do not cause specific symptoms, thus making it difficult to identify their presence.

Children with primary heart tumors, also known as cardiac tumors, are extremely rare, with an incidence of 0.017 to 0.28 percent in autopsies.

A primary heart/cardiac tumor occurs when an abnormal tissue grows in the heart. In rare situations, cancer spreads to the heart, and this is referred to as a secondary or metastatic tumor. Heart tumors are uncommon in children; they can be benign (noncancerous) or malignant (cancerous), and both can impair heart function.

Because these tumors are uncommon, it is difficult to acquire useful information about their natural history, pathology, and therapy.

Although heart tumors in children are uncommon, they can be aggressively treated with favorable results.

  • Benign tumors have an excellent survival rate, with most problems being due to the tumor site.
  • Malignant tumors have a high death rate; however, surgery and adjuvant treatment can help patients live longer.

What Are the Types of Heart Tumors in Children?

Heart tumors can be benign or malignant.

Benign heart tumors

  • Rhabdomyoma
    • The most common cardiac tumor in children, with 40 to 60 percent incidence. 
    • It was developed in the long fibers of the muscle.
  • Teratoma
    • A germ cell tumor often develops in the pericardium of the heart. Some teratomas may be malignant. 
    • Incidence of 15 to19 percent in children
  • Hemangioma 
    • Tumors develop in the blood vessels.
    • Incidence of five percent in children
  • Myxoma
    • A rare cardiac tumor with two to four percent incidence. 
    • Although its etiology is unknown, it is believed that an inherited syndrome, the Carney complex, can cause myxoma. 

Malignant heart tumors

  • Angiosarcoma: A malignancy developed in the lining of the blood vessels and lymphatic vessels 
  • Rhabdomyosarcoma: Malignancy developed in the long fibers of the muscle.

Staging determines if malignant cardiac tumors (cancer) have moved from the heart to surrounding regions or other sections of the body. There is no uniform staging for malignant pediatric cardiac tumors. The findings of tests and procedures used to detect malignant cardiac tumors are used to decide on appropriate treatment. Malignant pediatric cardiac tumors can relapse (reappear) following therapy.

What Are the Diagnostic Modalities for Heart Tumors in Children?

Some cardiac tumors are detected prenatally (before the baby is born) by a doctor during fetal echocardiography

The following diagnostic modalities are used to detect heart tumors in a newborn and children:

  • Echocardiogram
    • Sound waves are used to get a heart picture to detect structural abnormalities.
    • It forms an image on the monitor and provides real-time data on the heart's functionality and structures.
  • Electrocardiogram
    • This records the heart's electrical activity, and any abnormality or limitation in the heart activity can be identified.
  • X-ray of the chest
    • The child is subjected to an X-ray to produce a picture of the heart and surrounding structures. 
    • An X-ray of the chest can reveal any structural heart abnormality.
  • Cardiac MRI
    • A three-dimensional scan of the heart that reveals any abnormalities.
  • Cardiac catheterization
    • This may be necessary in rare cases.
    • A small tube is inserted into the heart through a vein or artery in the leg or the umbilicus.

What are the treatment options for heart tumors in children?

There are not enough patients with heart cancer to do clinical trials (human research) to determine the optimum treatment; therefore, there are no conventional therapies. Instead, treatment is individualized for everyone with heart cancer.

Most pediatric tumors are benign, do not produce symptoms, and may not require treatment. In other cases, therapy will differ based on cancer (its location, whether benign or malignant, confined or disseminated, primary or secondary).

  • Surgery
    • Pediatric primary cardiac tumors should be treated separately in the clinic. 
    • Most pediatric primary cardiac tumors are benign, and spontaneous remission, particularly in rhabdomyomas, is possible. 
    • The primary goal of surgical therapy is to restore normal hemodynamics while protecting vital structures and heart tissue.
    • However, this may not be possible if the tumor is vast or on the left side of the heart. 
    • Larger tumors may require partial removal to alleviate symptoms that affect the heart's function.
    • The child may require a heart pacemaker to be installed to correct any heart arrhythmia.
  • Other therapies
    • Chemotherapy and radiation therapy may extend a patient’s life. 
    • The doctor may recommend targeted therapy, often used for sarcomas detected in locations other than the heart.
  • Transplants
    • In rare cases of heart malignancy, doctors have explored heart transplants and heart–lung transplants. 
    • Transplants have helped some people live longer lives, but they do not generally function well. 
    • These procedures are ineffective for treating cardiac cancer that has progressed to other body regions.
Reviewed on 12/9/2022
Image Source: iStock image

Childhood Cardiac (Heart) Tumors Treatment (PDQ®)–Patient Version: https://www.cancer.gov/types/cardiac/patient-child-cardiac-treatment-pdq#:~:text=a%20heart%20tumor.-,Childhood%20cardiac%20tumors%2C%20which%20may%20be%20benign%20or%20malignant%2C%20form,made%20up%20of%20long%20fibers.

Cardiac Tumours In Children: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3225855/