The National Comprehensive Cancer Network (NCCN) has released its first-ever NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for pediatric bone sarcomas.
These new guidelines offer a comprehensive set of treatment recommendations for a challenging pediatric cancer, and offer evidence-based, expert consensus-driven recommendations for the prevention, screening, treatment of, and supportive care for pediatric patients with bone sarcomas.
About Pediatric Bone Sarcomas
The new guidelines are focused on pediatric Ewing sarcoma and other undifferentiated round cell sarcomas. Ewing sarcoma is the second most common primary pediatric bone tumor. The rare disease mostly impacts teenagers between the ages of 15 and 19, and typically presents as severe or persistent pain or swelling of the limbs or pelvis.
“Teenagers are at a unique point in their lives. Intensive cancer treatment can drain their energy and disrupt their developing sense of autonomy,” explained Patrick Leavey, MD, of the UT Southwestern Simmons Comprehensive Cancer Center, and Chair of the NCCN Guidelines Panel for Pediatric Bone Sarcoma. “These guidelines can help ensure they are not alone; that they know they have a knowledgeable team ready to support them.”
According to the American Cancer Society, the 5-year survival rate for early-stage Ewing sarcoma is 81%, but when diagnosed at a late stage, the 5-year survival rate is 41%.
“I know what these guidelines will mean to patients and their families,” said Paula Head, a patient advocate on the NCCN Guidelines Panel for Pediatric Bone Sarcoma. “Families who are walking that journey, need clear, accurate medical roadmaps that reduce anxiety, and bring confidence in the care their child is receiving. As a parent who experienced this with my daughter, I wanted there to be guidelines that are specifically for pediatrics, because these cancers and their treatments are different than for adults.”
About the New Guidelines
NCCN's new guidelines will provide physicians with a roadmap for making treatment decisions for pediatric patients with bone sarcomas to improve patient care and outcomes for these patients.
The guidelines recommend evaluating pediatric patients by a multidisciplinary team, at least including an orthopedic oncologist; a pediatric, bone, or sarcoma pathologist, a pediatric oncologist, a radiation oncologist, and a musculoskeletal radiologist. This expert multidisciplinary group should treat the patient with a team-based approach.
Symptomatic bone lesions are typically assessed with an x-ray followed by magnetic resonance imaging and biopsy to make a diagnosis.
Typical treatments for Ewing sarcoma and other undifferentiated round cell sarcomas include chemotherapy, radiation therapy, and surgery. Clinical trials are also recommended to advance new treatment options.
