Clinical Scenario

This protocol applies to patients with a localized malignant peripheral nerve sheath tumour (MPNST) where there is no evidence of distant metastasis — a setting where curative-intent management is the primary goal.

Specific Situation

Complete surgical excision with clear margins is a pivotal element of potentially curative management in this localized, non-metastatic setting. The anatomical position of these tumours can influence the achievability of an adequate resection margin.

Treatment Approach (Partial Overview)

The approach centres on surgical resection aiming for clear margins. The specific extent of surgery, margin targets, and whether any additional locoregional treatment is warranted depend on tumour grade and individual factors.

Full regimen details, decision criteria, and evidence summary are in the complete protocol below.

References

For localized MPNST, complete surgical excision with clear margins is a pivotal element of potentially curative management.
Generally, a R0 resection is recommended with at least 2 cm margin in all directions, but in MPNSTs this is not always achieved due to the nature of the anatomical position of these tumours.
Adjuvant/neo-adjuvant RT is still debatable for localized MPNSTs but should be really considered in a high-grade MPNST, whereas for the majority of low-grade localized MPNSTs, surgery alone is recommended.

DOI: 10.3390/cancers16193266

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Treatment of Localized Malignant Peripheral Nerve Sheath Tumor Without Distant Metastasis

References