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

Treatment of Localized Malignant Peripheral Nerve Sheath Tumor with No Distant Metastasis

This protocol addresses patients with a localized malignant peripheral nerve sheath tumour (MPNST) in whom there is no evidence of distant metastasis. In this setting, complete surgical excision with clear margins is a pivotal element of potentially curative management.

The systemic treatment strategy involves peri-operative chemotherapy combining an alkylating agent with an anthracycline, considered on an individual case-by-case basis. The full regimen — including agent selection, sequencing, and all decision criteria — is detailed in the structured protocol.

Complete regimen, dosing criteria, and clinical algorithm available below →
References
DOI: 10.3390/cancers16193266
  • For localized MPNST, complete surgical excision with clear margins is a pivotal element of potentially curative management.
  • The majority of studies have used as preferred systemic chemotherapy ifosfamide in association with an anthracycline—usually doxorubicin.
  • At present, the use of peri-operative chemotherapy should be considered on a case-by-case basis.
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