The National Comprehensive Cancer Network® (NCCN®) today published new NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Pediatric Soft Tissue Sarcomas. This is the 7th NCCN Guidelines® for a pediatric cancer, expanding the overall library to cover 91 topics in cancer care. The guidelines present the latest evidence-based, expert consensus-driven recommendations for nearly every type of cancer plus prevention, screening, survivorship, and supportive care. NCCN Guidelines are updated at least once a year and help cancer care providers worldwide to attain the best possible outcomes.
The new NCCN Guidelines® for Pediatric Soft Tissue Sarcomas is now available for free at NCCN.org. They focus on a subtype called rhabdomyosarcoma (RMS) which is the most common type of soft tissue sarcoma in people under 20. RMS represents nearly 5% of all childhood cancers.
"Most cancers that occur in children are fundamentally different from cancers occurring in adults," explained Stephen Skapek, MD, Duke Cancer Institute, Chair of the NCCN Guidelines Panel for Pediatric Soft Tissue Sarcoma. "RMS can be divided into specific subtypes that are driven by different genetic changes that can influence outcomes. Those changes and other clinical and pathology features are all incorporated into the multi-faceted treatments. That's why it was essential to form a group of leading, multi-disciplinary experts from across the country to put together a roadmap for diagnosing, risk stratifying, and treating these 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 side effects and zero recurrence."
Douglas Hawkins, MD, Panel Vice-Chair, Seattle Children's
RMS is tricky to diagnose; it can be located almost anywhere on the body. When it presents in the head, neck, or limb, it can show up as a lump with or without pain. Other times it occurs on an internal organ, which might cause problems for breathing or urination. There are three major risk groups with varying survival rates. The intensity of the treatment, which typically includes chemotherapy, surgery, and radiation therapy, must be tailored to the risk group as well as the age and the patient's developmental stage, which might be influenced by the treatment. The new NCCN Guidelines explain how to provide treatment in a way that balances the goal of cure with the need to minimize acute side effects, such as severe infections, and long-term effects that could bring additional problems at some point in the future.
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