Understanding Cancer Predisposition Syndromes

Genetics plays a key role in understanding some childhood cancers. Research shows that at least 10% of cancers in children are linked to gene changes present from birth. In this post, we’ll explain what cancer predisposition syndromes are, how they work, who might be affected, and what families and doctors can do when one is identified.

What is cancer predisposition syndrome?

When a person is born with a gene change that increases their risk of getting cancer, this is called a cancer predisposition syndrome. It means that cancer may develop earlier in life. In fact, some people with this type of syndrome may have multiple types of cancer over their lifetime.

What are genes?

Genes are the instructions that tell our bodies how to grow and develop. Genes usually come in pairs, and we receive one copy of a gene from our mother and one copy from our father. We have thousands of genes. Some genes are called tumor suppressors. Their job is to protect us from developing tumors and cancer.

What causes a cancer predisposition syndrome?

Some people are born with a gene change in one of the genes that usually protects us from getting cancer. Typically, these gene changes make it harder for our bodies to stop tumors and cancers from growing. This leads to an increased risk of tumors and cancer.

Sometimes, a cancer predisposition syndrome is passed down from a parent to their child. Other times, it happens for the first time in a child.

Once a gene change is identified in a family, genetic testing may be recommended for other family members to see if they also have an increased risk of getting cancer.

How does Lurie Children’s help patients with cancer predisposition syndromes?

Children and families with a suspected or known cancer predisposition syndrome are followed in the Cancer Predisposition Program. This program provides comprehensive genetic counseling and testing services, as well as guidance for managing the syndrome. This is all based on the results of personalized cancer risk assessments.  The program also offers support services to help families navigate treatment.  We connect families with resources and support organizations. The team also helps ensure that the patient and their family have the tools needed to succeed in daycare, school, work, and other important parts of life.  

A typical visit includes meeting with a cancer predisposition physician (a cancer doctor, also called an oncologist), genetic counselor, and social worker. After the visit, a nurse helps coordinate any screening and follow-up that was recommended at the visit.

There are many reasons a child may be referred to the Cancer Predisposition Clinic. Sometimes, a child has a tumor or other health concern which has been associated with a specific cancer predisposition syndrome. Some children are referred because they have family members with a cancer predisposition syndrome or have close family members who have had cancer. Some children are referred because they had genetic testing for other reasons and were found to have a genetic change that increases their risk of cancer.

No matter what brings a child to the Cancer Predisposition Clinic, our dedicated multidisciplinary team provides a personalized approach to meet the unique needs of each family.

Learn more about our Cancer Predisposition Program