ArticleBJS open2024
Local recurrence in malignant peripheral nerve sheath tumours: multicentre cohort study.
Article in BJS open, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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Who cites it
9 citing papers in PubMed, 8 citations in OpenAlex.
- Pleomorphism in Soft Tissue Sarcomas: Molecular Characteristics and Clinical Features.Medical sciences (Basel, Switzerland) · 2026Review
- Article
- Malignant peripheral nerve sheath tumor of the ileocecal region: a case report.Frontiers in oncology · 2026Article
- Transformation of a gluteal type 1 plexiform neurofibroma into a high-grade malignant peripheral nerve sheath tumor: a rare case report.Frontiers in oncology · 2026Article
- Rectal malignant peripheral nerve sheath tumor with extremely high Ki-67 index (80%) and concomitant meningioma history: a case report.Frontiers in oncology · 2026Article
- The role of radiotherapy in MPNST and the impact of NF1 status on outcomes: Insights from a multicenter cohort study.Neuro-oncology · 2025Article
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundMalignant peripheral nerve sheath tumours (MPNSTs) have high local recurrence (LR) rates. Literature varies on LR risk factors and treatment. This study aimed to elucidate treatment options and risk factors for first and second LRs (LR1 and LR2) in a large multicentre cohort.
methodSurgically treated primary MPNSTs between 1988 and 2019 in the MONACO multicentre cohort were included. Cox regression analysed LR1 and LR2 risk factors and overall survival (OS) after LR1. Treatment of LR1 and LR2 was evaluated.
resultsAmong 507 patients, 28% developed LR1. Median follow-up was 66.9 months, and for survivors 111.1 months. Independent LR1 risk factors included high-grade tumours (HR 2.63; 95% c.i. 1.15 to 5.99), microscopically positive margins (HR 2.19; 95% c.i. 1.51 to 3.16) and large tumour size (HR 2.14; 95% c.i. 1.21 to 3.78). Perioperative radiotherapy (HR 0.62; 95% c.i. 0.43 to 0.89) reduced the risk. LR1 patients had poorer OS. Synchronous metastasis worsened OS (HR 1.79; 95% c.i. 1.02 to 3.14) post-LR1, while surgically treated LR was associated with better OS (HR 0.38; 95% c.i. 0.22 to 0.64) compared to non-surgical cases. Two-year survival after surgical treatment was 71% (95% c.i. 63 to 82%) versus 28% (95% c.i. 18 to 44%) for non-surgical LR1 patients. Most LR1 (75.4%) and LR2 (73.7%) patients received curative-intent treatment, often surgery alone (64.9% versus 47.4%). Radiotherapy combined with surgery was given to 11.3% of LR1 and 7.9% of LR2 patients.
conclusionLarge, high-grade MPNSTs with R1 resections are at higher LR1 risk, potentially reduced by radiotherapy. Surgically treated recurrences may provide improved survival in highly selected cases.
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