UPMC Video Rounds - Inherited Cancer Predisposition Syndrome

December 2, 2019

Julia Meade, MD, assistant professor, Division of Pediatric Hematology/Oncology:

Inherited cancer predisposition syndromes are moving into an area where we're discovering more and more of these syndromes as the cause for a child developing cancer. And so, 20 years ago, we didn't know why some children developed cancer. And now, we're able to provide families with that understanding.

The pediatric cancer predisposition program at Children's is a new service that we're offering to children and families. Some children are already diagnosed with a cancer. Others, there may be a concern based upon the family history, that there could be an inherited cancer predisposition syndrome in the family. And, other patients are survivors of childhood cancer. And so, all together, we have a staff physician, a genetic counselor, and a psychologist on our team, who work together to support families as they make these decisions, whether or not genetic counseling is right for their family and their child.

We know that picking up cancers at an earlier stage is one of the best ways that we can ensure a better outcome for children. So, the screening guidelines that we use are based upon national recommendations, and they involve often MRI tests, abdominal ultrasounds, various types of blood work on a routine interval, usually every, quarterly to yearly, depending upon the type of condition. And so, families can receive a lot of reassurance when they come for their screening, that we're actively monitoring their child for the development of cancer, so that we can intervene at the earliest possible stage.

We have a molecular tumor board that we offer as a service to anybody across the country who has questions regarding their patients, whether or not genetic testing would benefit the family, or even if they have a specific question regarding a patient's tumor, or some other mutation that was discussed on the family history. 

One of the greatest stressors that we hear families talk about is the emotional weight of making the decision to test or knowing that their child is positive for an inherited cancer predisposition syndrome. This is why our clinic appointments are longer than even a regular oncology appointment, to allow families to have time to process that narrative and also ask questions and verbalize their distress, either to our team or, sometimes, in our sessions with our clinical psychologist, who is really dedicated to helping support families long term with the distress that they sometimes face, knowing that there is an inherited cancer predisposition syndrome in the family.

Learn more about the Division of Pediatric Hematology/Oncology at UPMC Children's Hospital of Pittsburgh.