NCCN Debuts First Pediatric Bone Sarcoma Guidelines Focused on Ewing Sarcoma

The NCCN has published the first-ever edition of its NCCN Clinical Practice Guidelines in Oncology for pediatric bone sarcoma, according to a news release from the organization.¹

The new guidelines center on Ewing sarcoma and other undifferentiated round cell sarcomas, with the goal of defining the best possible care for children and adolescents with these malignancies. Although extraordinarily rare overall, Ewing sarcoma is the second most common primary bone tumor affecting children and adolescents.

The disease typically affects teenagers between the ages of 15 and 19 years and can present as severe and persistent pain or swelling in the limbs or pelvis. Because those symptoms are also common to other bone issues in teens, the release notes that x-rays are a key first step in the diagnostic process.

Paula Head, a patient advocate on the NCCN Guidelines Panel for Pediatric Bone Sarcoma, said in the release “I know what these guidelines will mean to patients and their families. 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.”

NCCN’s evidence-based, expert consensus–driven recommendations for prevention, screening, treatment, and supportive care are relied on by care providers around the world. According to the release, the guidelines provide a roadmap for making optimal treatment decisions while putting guardrails in place against overtreatment or undertreatment.

Additionally, the NCCN categories of evidence and consensus for pediatric bone sarcoma are category 2A unless otherwise noted.²

• Principles of systemic therapy for Ewing sarcoma include:
  • First-line therapy for localized disease
    • Enrollment in a clinical trial
    • Interval compressed vincristine, doxorubicin, cyclophosphamide, and mesna (VDC) and alternating with ifosfamide, mesna, and etoposide (IE) for 14 cycles
  • First-line therapy for metastatic disease
    • Interval compressed VDC-IE
  • Second line therapy
    • Cyclophosphamide/mesna/topotecan
    • High-dose ifosfamide/mesna
    • Irinotecan/temozolomide
    • Irinotecan/temozolomide/vincristine
• Principles of systemic therapy for other undifferentiated round cell sarcomas:
  • First-line therapy for local disease
    • VDC-IE
  • First-line therapy for metastatic disease
    • VDC-IE
  • Second-line therapy
    • Enrollment in a clinical trial

Patrick Leavey, MD, of UT Southwestern Simmons Comprehensive Cancer Center, chairs the NCCN Guidelines Panel for Pediatric Bone Sarcoma, said “Teenagers are at a unique point in their lives. Intensive cancer treatment can drain their energy and disrupt their developing sense of autonomy. These guidelines can help ensure they are not alone; that they know they have a knowledgeable team ready to support them.”

Outcomes for patients with Ewing sarcoma remain closely tied to the extent of disease at diagnosis. The 5-year survival rate for Ewing sarcoma diagnosed at an early stage is currently 81%, though that figure drops to 41% for patients with metastatic disease.

The new guidelines join several other pediatric cancer guidelines that NCCN has added to its library during the last few years, including those for pediatric acute lymphoblastic leukemia, pediatric aggressive mature B-cell lymphomas, pediatric Hodgkin lymphoma, Wilms tumor, pediatric central nervous system cancers, and pediatric soft tissue sarcoma.

The NCCN Guidelines are updated continuously by more than 60 panels featuring more than 2000 interdisciplinary experts from NCCN’s 34 member institutions, along with patient advocates. The guidelines are intended to assist decision-making among individuals involved in cancer care, including physicians, nurses, pharmacists, payers, patients, and their families, with the goal of improving patient care and outcomes. According to the NCCN, independent studies have found that guideline-concordant care is associated with longer lives, better outcomes, and lower costs across a variety of cancer types.

Reference

1. A formidable type of childhood cancer gets added to highly regarded library of treatment recommendations. News release. National Comprehensive Cancer Network. October 1, 2026. Accessed October 12, 2026. https://tinyurl.com/3fp8yku6
2. NCCN clinical practice guidelines in oncology. Pediatric bone sarcoma. Versions 1.2027. Updated October 1, 2027. Accessed October 12, 2026. https://tinyurl.com/ye2473mv