Earlier today, the Texas Children’sCancer Center announced a new, first of its kind, Childhood Cancer Prevention and Screening Clinic which will screen and follow children who are at an elevated risk for developing childhood cancer.

The new clinic will reassure families of children with genetic conditions such as Li-Fraumeni syndrome and Beckwith-Wiedemann syndrome which predispose individuals to childhood cancer, as well as children with other conditions strongly associated with pediatric cancer, such as hemihypertrophy, where one side or part of one side of the body is enlarged.

Centralized cancer screening
“This new clinic will allow us to centralize the cancer screening services for children who have been diagnosed with a predisposition to cancer through a medical evaluation from a geneticist or another physician experienced with these conditions,” said Surya Rednam, MD pediatric oncologist at Texas Children’s Cancer Center and director of the new clinic. “Using cancer screening methods based on the latest medical evidence and tailored to the needs of each child, we can identify tumors sooner in these children, allowing for treatments to be implemented earlier, ultimately leading to improved survival rates,” said Rednam, who is also an instructor in pediatric hematology/oncology at Baylor College of Medicine (BCM).

Genetic disorder
Li-Fraumeni syndrome is a rare disorder that greatly increases the risk of developing several types of cancer, particularly in children and young adults. The syndrome is most often associated breast cancer, a form of bone cancer called osteosarcoma, and cancers of soft tissues called soft tissue sarcomas. Other cancers commonly seen in this syndrome include brain tumors, cancers of blood-forming tissues (leukemias), and a cancer called adrenocortical carcinoma that affects the outer layer of the adrenal glands. Several other types of cancer also occur more frequently in people with Li-Fraumeni syndrome.

A very similar condition called Li-Fraumeni-like syndrome shares many of the features of classic Li-Fraumeni syndrome. Both conditions significantly increase the chances of developing multiple cancers beginning in childhood; however, the pattern of specific cancers seen in affected family members is different.

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The exact prevalence of Li-Fraumeni is unknown. More than half of all patients with Li-Fraumeni syndrome have inherited mutations in the TP53 gene. TP53, a tumor suppressor gene, normally helps control the growth and division of cells. However, mutations in this gene may lead to uncontrolled cell growth. Noteworthy is that a number of patients with cancers characteristic of Li-Fraumeni syndrome and Li-Fraumeni-like syndrome do not have TP53 mutations, but mutations in the CHEK2 gene. Just like the TP53 gene, CHEK2 is a tumor suppressor gene.

Early detection
Detecting tumors earlier may also allow for less intensive treatments. For example, with earlier detection, a less aggressive surgery may be performed or the dose of radiation may be decreased or even avoided entirely, which would lessen treatment-related chronic health issues.

The new clinic is part of Texas Children’s Cancer Center’s Genetics Program. Most patients will first be seen by the Cancer Genetics Clinic team to be evaluated for genetic risk factors. Those children found to be at an increased risk of developing childhood cancer will then be followed long-term in the new clinic, using screening guidelines developed for their specific cancer risks. Since opening, this clinic has seen 30 patients who were identified as having an increased risk for childhood cancer.

High risk tumors and cancer
Among children who may be at a high risk for tumors and cancers are current pediatric cancer patients with a genetic condition, siblings of pediatric cancer patients and children with other family histories of cancer. Families who fall into one of these categories who are interested in having their child seen by the Cancer Genetics Clinic and the Childhood Cancer Prevention and Screening Clinic are encouraged to visit their pediatrician for a referral.

“Over time, the data collected by centralizing the care of these patients will allow us to better assess the effectiveness of our current screening methods in detecting cancer and improving patient outcomes,” said Rednam. “Additionally, as new tests and technologies are developed, this clinic will enable us to compare the currently recommended screenings with newer methods so we are constantly striving to ensure that our patients receive the most up-to-date and effective cancer screening services available.”

Texas Children’s Cancer Center is a joint program of Texas Children’s Hospital and BCM, and is the pediatric program of BCM’s NCI-designated Dan L. Duncan Cancer Center.

Photo: Surya RednamMD, Department of Pediatrics, Section of Hematology-Oncology Baylor College of Medicine. Photo courtesy: Texas Children’s Cancer Center.

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