New guidelines say risk classification ‘paramount’ in treating children with neuroblastoma
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Determining risk classifications of children with neuroblastoma is “paramount” to optimizing their care, the chair of the first National Comprehensive Cancer Network guidelines panel on the disease, told Healio.
Oncologists should focus on “do no harm” in children with low- and intermediate-risk neuroblastoma, and they should be “thoughtful and team-oriented” when treating patients who are high risk, Rochelle Bagatell, MD, solid tumor section chief at Children’s Hospital of Philadelphia, told Healio when describing the guidelines’ key recommendations.
“Neuroblastoma is a very clinically and biologically heterogeneous disease,” Bagatell said. “The care of these patients ranges from observation alone for the patients with the best prognosis to the most complicated, lengthy, multimodality therapy you could dream up,” she added. “Knowing how to use the right tools for the right patients is important, and having guidance from thought leaders will be very helpful to clinicians.”
More than 700 cases of neuroblastoma are diagnosed every year in the United States, making it the most common solid tumor cancer in children, according to background information provided the NCCN.
Children with low- and intermediate-risk diagnoses have an OS rate higher than 90%, while those categorized as high risk have a roughly 50% survival rate.
“There are a group of prognostic factors that have been studied very carefully over the years and have been shown to be important [in risk classification],” Bagatell said. “Those include clinical risk factors like age and [tumor] stage, but also aspects of tumor histology and tumor biology. There’s guidance in the document about evaluation of those risk factors, and then also how to put them together to come up with a risk group.”
A multidisciplinary panel spent more than 18 months creating the new guidelines.
“The committee was comprised of thought leaders from various disciplines at the centers that comprise the National Comprehensive Cancer Network,” Bagatell said. “That includes pediatric medical oncologists, radiation oncologists, surgical oncologists, pediatric pathologists, imagers — everybody who participates in the care of children with neuroblastoma helped us with these.”
The guidelines detail principles of pathology, imaging, risk classification, systemic therapy, surgery and radiation therapy, as well as follow-up and response.
“Understanding the risk for relapse is one of the most important things for people taking care of children with neuroblastoma,” Bagatell said.
“Once a risk group has been assigned, then the diagrams in the NCCN guidelines make a lot more sense because you can look at the low-risk and intermediate-risk paradigms, and then the high-risk paradigm, and understand much better where your patient fits,” she added. “The surveillance guidelines are important because too much surveillance can unnecessarily stoke anxiety, but we also want to monitor properly for disease recurrence so that we can take optimal care of the patients.”
Bagatell highlighted a primary takeaway for each subset of patients. For children with low- and intermediate-risk neuroblastoma, oncologists should be deliberate and not rush to treatment.
“The most important message in this guideline document is first do no harm,” Bagatell said. “That sounds old school — the Hippocratic Oath — but it’s real. If a child has an excellent prognosis and is going to live a long life, you don’t want to have overtreated them and caused harm.”
For children with high-risk neuroblastoma, multidisciplinary care is critical.
“Teamwork is really important in the care of children with high-risk neuroblastoma,” Bagatell said. “Their therapy includes multiagent chemotherapy, surgery to remove the primary tumor and adjacent lymph nodes, high-dose chemotherapy with autologous stem cell rescue, radiation therapy, immunotherapy directed at an important antigen on the surface of neuroblastoma cells, differentiating therapy and other agents that we put together in a very particular way. There are a lot of a people with expertise who are instrumental in the care of the patient.”
Collaboration has progressed over the years, but still needs development. “The emphasis is really very strong in these guidelines,” Bagatell said.
She hopes these guidelines can build on the improvement of care children with neuroblastomas have received.
“Since I began to practice in this field, the event-free survival rates for children with high-risk neuroblastoma have doubled, and it’s because of the multimodality care that we deliver,” Bagatell said. “But an approximately 50% survival rate is still unacceptable to patients, parents, providers — everybody. We must optimize what we’re doing and always be looking for ways to do it better so that we can get as close as we can to doubling it again.”
References:
- NCCN clinical practice guidelines in oncology — neuroblastoma. Available at: https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1520. Version 1.2024. Published Feb. 7, 2024. Accessed Feb. 12, 2024.
- New resource for selecting best treatment path for young children with cancerous tumors published by NCCN (press release). Available at: https://www.nccn.org/home/news/newsdetails?NewsId=4406. Published Feb. 7, 2024. Accessed Feb. 12, 2024.
For more information:
Rochelle Bagatell, MD, can be reached at bagatellr@chop.edu.
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