ArticleJournal of the National Cancer Institute2026
Malignant peripheral nerve sheath tumors: a report from Children's Oncology Group study ARST0332.
Article in Journal of the National Cancer Institute, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT00346164 (Risk-Based Treatment for Non-Rhabdomyosarcoma Soft Tissue Sarcomas), which is not on this map. Cited by 1 paper.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Risk-Based Treatment for Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS) in Patients Under 30 Years of Age
Who cites it
1 citing paper in PubMed.
- Surgical management of plexiform neurofibromas: An imaging-based perioperative framework.JPRAS open · 2026Review
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12 authors.
Funding
Abstract
backgroundThe cornerstone of the treatment of malignant peripheral nerve sheath tumors 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).
methodsWe present data for 58 patients with newly diagnosed malignant peripheral nerve sheath tumors 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.
resultsMost patients had primary tumors that were greater than 5 cm (86%), deep (95%), and invasive (74%), and 10% had distant metastases. Of the patients, 32 (55%) had germline NF-1, and 26 (45%) did not. Among patients, 31 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 was 87%, 52%, and 0% for the low- (n = 8), intermediate-, (n = 44), and high-risk (n = 6) patients, respectively. In univariate analysis, event-free survival and overall survival differed by sex, presence or absence of metastatic disease, risk group, and achievement of upfront or delayed R0 and/or R1. There was no difference in event-free survival or overall survival based on germline NF-1 status.
conclusionThe treatment strategy in ARST0332 achieved excellent outcomes for low-risk malignant peripheral nerve sheath tumors. Patients with high-risk (metastatic) malignant peripheral nerve sheath tumors have poor outcomes, and novel treatments are needed (NCT00346164).
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