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The National Comprehensive Cancer Network (NCCN) has released modern clinical practice guidelines aimed at improving the care of children and young adults with soft tissue sarcoma, with a specific focus on rhabdomyosarcoma (RMS). These guidelines, published February 17, 2026, represent the seventh dedicated to a pediatric cancer, expanding NCCN’s overall library to 91 topics in cancer care.
Rhabdomyosarcoma is the most common type of soft tissue sarcoma diagnosed in individuals under the age of 20, accounting for nearly 5% of all childhood cancers. The new guidelines address the complexities of RMS, a disease that can present with varying subtypes driven by different genetic changes, impacting treatment outcomes. The goal is to provide a roadmap for diagnosis, risk stratification, and treatment, balancing effective therapies with minimizing adverse effects.
Understanding Rhabdomyosarcoma and the Need for Specialized Guidelines
Treatment for RMS typically involves a combination of chemotherapy, surgery, and radiation therapy, tailored to the patient’s risk group, age, and developmental stage. Recognizing that childhood cancers differ fundamentally from those in adults, the NCCN convened a panel of experts to develop these specialized recommendations. As Stephen Skapek, MD, chair of the NCCN Guidelines Panel for Pediatric Soft Tissue Sarcoma and chief of the Division of Pediatric Hematology-Oncology at Duke Cancer Institute, explained, “RMS can be divided into specific subtypes that are driven by different genetic changes that can influence outcomes.”
The guidelines offer detailed considerations for various tumor locations, including masses in the head and neck, extremities, trunk, and genitourinary and gastrointestinal areas. For each location, treatment maps are provided based on patient characteristics. Risk stratification criteria – categorizing patients as very-low-risk, low-risk, intermediate-risk, or high-risk – are clearly outlined, along with corresponding treatment options.
Surveillance and Long-Term Management
Beyond initial treatment, the guidelines emphasize the importance of ongoing surveillance for patients achieving remission. Recommended surveillance protocols include physical examinations every 3 to 6 months for the first 2 to 3 years, then every 6 to 12 months for years 4 and 5, followed by annual check-ups. Imaging, utilizing CT scans or chest X-rays, is recommended at similar intervals, with the potential for whole-body fluorodeoxyglucose-PET imaging as clinically indicated.
The guidelines also address key aspects of diagnosis, including imaging principles, pathologic assessment, surgical approaches, lymph node evaluation, radiation therapy techniques, and systemic therapy regimens. Systemic therapy protocols frequently incorporate vincristine and dactinomycin, sometimes combined with cyclophosphamide and maintenance therapy, across all risk levels.
Addressing Treatment-Related Risks and Survivorship
Recognizing the potential long-term effects of cancer treatment, the guidelines dedicate a section to survivorship, outlining potential risks associated with chemotherapy, radiotherapy, and surgery. Chemotherapy can lead to vasospastic attacks, while radiotherapy carries risks of secondary cancers, pulmonary and cardiac toxicity, and other complications. Surgical interventions may result in complications such as amputation-related issues, bowel obstruction, or functional asplenia.
Douglas Hawkins, MD, vice-chair of the NCCN panel and professor of Hematology-Oncology at Seattle Children’s Hospital, underscored the unique challenges of treating young patients. “Children are most likely to be diagnosed with RMS at a very young age, toddlers or early elementary school. When treating someone so young, you are not looking to just prolong survival; the goal is a full cure with minimal [adverse] effects and zero recurrence.”
These updated NCCN guidelines represent a significant step forward in the standardized and evidence-based care of pediatric rhabdomyosarcoma, aiming to optimize outcomes and minimize long-term complications for young patients. Further research and ongoing refinement of these guidelines will be crucial as our understanding of this complex disease evolves.
As the field of pediatric oncology continues to advance, ongoing monitoring of treatment efficacy and long-term outcomes will be essential. The NCCN guidelines are slated for annual updates, reflecting the latest research and clinical insights.
Disclaimer: This information is intended for general knowledge and informational purposes only, and does not constitute medical advice. It is essential to consult with a qualified healthcare professional for any health concerns or before making any decisions related to your health or treatment.
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