Malignant peripheral nerve sheath tumor
ICD-10 C47 · ICD-11 2B5E

Treatment of MPNST of the Extremity When Resection Would Require Amputation or Cause Significant Morbidity

Clinical Scenario

Malignant peripheral nerve sheath tumour (MPNST) located in the extremity, where standard surgical resection is not feasible without resulting in significant morbidity or necessitating amputation. This presentation requires a limb-preservation strategy rather than immediate surgery.

Treatment Approach

A neo-adjuvant radiotherapy-based strategy — used alone or in combination with a regional treatment approach — followed by resection is applied with the aim of enabling limb-preserving surgery. The complete treatment algorithm, eligibility criteria, and sequencing are defined in the full structured protocol.

Full regimen details are available in the protocol below.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.3390/cancers16193266

For extremity tumours not amendable to resection without significant morbidity or amputation, neo-adjuvant RT combined with/without isolated limb perfusion (ILP) with melphalan and tumour necrosis factor alpha, followed by resection, can contribute to successful limb preservation.

ILP, with or without adjuvant RT, was shown to have similar oncological outcomes to only adjuvant RT, but limb salvage rates (>80%) were reported to be superior with the addition of ILP.

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