# Malignant peripheral nerve sheath tumors: a report from Children’s Oncology Group study ARST0332 — GCMD Library
Jacquelyn N Crane, Wei Xue, Amira Qumseya, Donald A Barkauskas, Khang Chau, Serena Y Tan, Susan Hiniker, Roshni Dasgupta, Rajkumar Venkatramani, Sheri L Spunt, Aaron R Weiss, Theodore W Laetsch
Background: The cornerstone of the treatment of malignant peripheral nerve sheath tumors (MPNST) is surgical resection. Radiation and chemotherapy are variably employed. The optimal treatment remains uncertain, particularly for unresectable or metastatic disease and patients with neurofibromatosis type-1 (NF-1).
Methods: We present data for fifty-eight patients with newly diagnosed MPNST enrolled on the Children's Oncology Group study ARST0332. Patients were treated with risk-adapted therapy including surgery with or without radiotherapy and ifosfamide and doxorubicin chemotherapy.
Results: Most patients had primary tumors that were greater than 5 cm (86%), deep (95%), and invasive (74%), and 10% had distant metastases. Thirty-two (55%) patients had germline NF-1 and 26 (45%) did not. Thirty-one patients received neoadjuvant therapy and 22 were evaluable for response with 5 (23%) attaining an objective response, 10 (45%) stable disease, and 7 (32%) progressive disease. Estimated 5-year event-free survival (EFS) was 87%, 52% and 0% for the low- (n = 8), intermediate- (n = 44) and high-risk (n = 6) patients, respectively. In univariate analysis, EFS and overall survival (OS) differed by sex, presence or absence of metastatic disease, risk group, and achievement of upfront or delayed R0/R1. There was no difference in EFS or OS based on germline NF-1 status.
Conclusion: The treatment strategy in ARST0332 achieved excellent outcomes for low-risk MPNST. Patients with high risk (metastatic) MPNST have poor outcomes and novel treatments are needed. (NCT00346164).
Type: video · 1 min · posted 2026-03-24
Canonical: https://library.globalcastmd.com/watch/malignant-peripheral-nerve-sheath-tumors-a-report-from-children-s-oncology-group-study-arst0332-11726
## Chapters
- [0:00](https://library.globalcastmd.com/watch/malignant-peripheral-nerve-sheath-tumors-a-report-from-children-s-oncology-group-study-arst0332-11726?t=0) Introduction and study context
- [0:22](https://library.globalcastmd.com/watch/malignant-peripheral-nerve-sheath-tumors-a-report-from-children-s-oncology-group-study-arst0332-11726?t=22) Study design and treatment arms
- [0:34](https://library.globalcastmd.com/watch/malignant-peripheral-nerve-sheath-tumors-a-report-from-children-s-oncology-group-study-arst0332-11726?t=34) Outcomes and clinical implications
## Statements
- "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." — Jill Knepprath (clinical) [0:11](https://library.globalcastmd.com/watch/malignant-peripheral-nerve-sheath-tumors-a-report-from-children-s-oncology-group-study-arst0332-11726?t=11)
- "Patients were assigned to four treatment groups: observation, adjuvant radiation, adjuvant chemoradiation, and neoadjuvant chemoradiation." — Jill Knepprath (clinical) [0:24](https://library.globalcastmd.com/watch/malignant-peripheral-nerve-sheath-tumors-a-report-from-children-s-oncology-group-study-arst0332-11726?t=24)
- "Resection has a major influence on survival for patients with malignant peripheral nerve sheath tumors." — Jill Knepprath (clinical) [0:35](https://library.globalcastmd.com/watch/malignant-peripheral-nerve-sheath-tumors-a-report-from-children-s-oncology-group-study-arst0332-11726?t=35)
- "Female patients with malignant peripheral nerve sheath tumors had improved outcomes." — Jill Knepprath (epidemiological) [0:40](https://library.globalcastmd.com/watch/malignant-peripheral-nerve-sheath-tumors-a-report-from-children-s-oncology-group-study-arst0332-11726?t=40)
- "Patients without metastatic disease had improved outcomes." — Jill Knepprath (clinical) [0:40](https://library.globalcastmd.com/watch/malignant-peripheral-nerve-sheath-tumors-a-report-from-children-s-oncology-group-study-arst0332-11726?t=40)
- "Low-risk patients had improved outcomes." — Jill Knepprath (clinical) [0:40](https://library.globalcastmd.com/watch/malignant-peripheral-nerve-sheath-tumors-a-report-from-children-s-oncology-group-study-arst0332-11726?t=40)
- "In patients receiving neoadjuvant therapy, 23% showed a response." — Jill Knepprath (clinical) [0:46](https://library.globalcastmd.com/watch/malignant-peripheral-nerve-sheath-tumors-a-report-from-children-s-oncology-group-study-arst0332-11726?t=46)
- "In patients receiving neoadjuvant therapy, 45% were stable." — Jill Knepprath (clinical) [0:46](https://library.globalcastmd.com/watch/malignant-peripheral-nerve-sheath-tumors-a-report-from-children-s-oncology-group-study-arst0332-11726?t=46)
- "In patients receiving neoadjuvant therapy, 32% had progressive disease." — Jill Knepprath (clinical) [0:46](https://library.globalcastmd.com/watch/malignant-peripheral-nerve-sheath-tumors-a-report-from-children-s-oncology-group-study-arst0332-11726?t=46)
- "There was no difference in survival based on the response to neoadjuvant therapy among patients who went on to have resection." — Jill Knepprath (clinical) [0:59](https://library.globalcastmd.com/watch/malignant-peripheral-nerve-sheath-tumors-a-report-from-children-s-oncology-group-study-arst0332-11726?t=59)
- "Resection should be done for patients with malignant peripheral nerve sheath tumors whenever possible, even if they show a good response to chemoradiation." — Jill Knepprath (guideline) [1:03](https://library.globalcastmd.com/watch/malignant-peripheral-nerve-sheath-tumors-a-report-from-children-s-oncology-group-study-arst0332-11726?t=63)
## Transcript
Is there a role for chemo radiation in peripheral nerve sheath tumors, or is excision all that is needed? I'm Jill Knerath with Stay Current MD, and this is an article you should know. In 2025, the Children's Oncology Group published a prospective trial in the Journal of the National Cancer Institute. They explored treatments and outcomes for patients with these rare tumors. So what did they do for these patients? Patients were assigned to 4 groups observation, adjuvant radiation. Adjuvant chemo radiation and neoadjuvant chemo radiation. What were the outcomes? Overall results confirmed that resection has a major influence on survival for these patients. Female patients, patients without metastatic disease, and low-risk patients also had improved outcomes. In patients receiving neoadjuvant therapy, 23% showed a response, 45% were stable, and 32% had progressive disease. Of these patients that went on to have their resection. There was no difference in survival based on the response to neoadjuvant therapy. Overall, this study confirms that resection should be done for these patients whenever possible, even if they show a good response to chemo radiation.
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Not medical advice · citation policy: https://library.globalcastmd.com/ai