From
StayCurrentMD
Malignant peripheral nerve sheath tumors: a report from Children’s Oncology Group study ARST0332
hosted by Dr. Jill Knepprath
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
The Children's Oncology Group published a prospective trial on malignant peripheral nerve sheath tumors in the Journal of the National Cancer Institute in 2025.
Patients were assigned to four treatment groups: observation, adjuvant radiation, adjuvant chemoradiation, and neoadjuvant chemoradiation.
Resection has a major influence on survival for patients with malignant peripheral nerve sheath tumors.
Female patients with malignant peripheral nerve sheath tumors had improved outcomes.
Patients without metastatic disease had improved outcomes.
Low-risk patients had improved outcomes.
In patients receiving neoadjuvant therapy, 23% showed a response.
In patients receiving neoadjuvant therapy, 45% were stable.
In patients receiving neoadjuvant therapy, 32% had progressive disease.
There was no difference in survival based on the response to neoadjuvant therapy among patients who went on to have resection.
Resection should be done for patients with malignant peripheral nerve sheath tumors whenever possible, even if they show a good response to chemoradiation.