PLYMOUTH MEETING, PA — The National Comprehensive Cancer Network has issued its first clinical guidelines specifically for pediatric bone sarcoma, establishing treatment recommendations for Ewing sarcoma and related rare cancers that primarily affect children and adolescents.
The NCCN Clinical Practice Guidelines in Oncology for Pediatric Bone Sarcoma focus on Ewing sarcoma and other undifferentiated round cell sarcomas, giving clinicians a pediatric-specific framework for diagnosis, treatment and supportive care.
Ewing sarcoma is rare but ranks as the second-most-common primary bone tumor among children and adolescents. It typically affects teenagers between 15 and 19 years old.
The cancer can cause severe and persistent pain or swelling in the limbs or pelvis. Because those symptoms can resemble other bone problems in teenagers, the guidelines identify X-rays as an important initial step in diagnosis.
Outcomes differ sharply depending on how far the cancer has spread when diagnosed. The five-year survival rate is 81% for Ewing sarcoma diagnosed at an early stage, compared with 41% for metastatic disease, according to data cited by NCCN.
Patrick Leavey, chair of NCCN’s Pediatric Bone Sarcoma Guidelines Panel and a physician at UT Southwestern Simmons Comprehensive Cancer Center, emphasized the particular challenges intensive cancer treatment creates for adolescents.
“Teenagers are at a unique point in their lives,” Leavey said. “Intensive cancer treatment can drain their energy and disrupt their developing sense of autonomy.”
The guidelines also emphasize clinical trials as a means of advancing treatment. Ewing sarcoma’s rarity can make therapeutic progress difficult, though risk stratification has improved in recent years.
NCCN develops its clinical recommendations through expert panels that review evidence and reach consensus on cancer prevention, screening, treatment and supportive care. More than 2,000 interdisciplinary experts and patient advocates from the organization’s 34 member institutions participate across more than 60 guideline panels.
The pediatric bone sarcoma recommendations expand a pediatric oncology portfolio that already includes guidelines covering acute lymphoblastic leukemia, aggressive mature B-cell lymphomas, Hodgkin lymphoma, Wilms tumor, central nervous system cancers and soft tissue sarcoma.
Paula Head, a patient advocate on the Pediatric Bone Sarcoma Guidelines Panel whose daughter experienced the disease, argued that separate pediatric recommendations are important because childhood cancers and their treatment can differ from adult disease.
“Families who are walking that journey need clear, accurate medical roadmaps that reduce anxiety, and bring confidence in the care their child is receiving,” Head said.
The Pediatric Bone Sarcoma Guidelines are available for noncommercial use at NCCN.org and through the Virtual Library of NCCN Guidelines App. Free registration is required to access the clinical content.
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