Malignant peripheral nerve sheath tumor (MPNST) presents a significant therapeutic challenge when surgical resection is not feasible or when the disease has spread. In these situations, the treatment strategy shifts toward systemic therapy.
This protocol applies to patients with inoperable MPNST — where curative surgery cannot be achieved — or with metastatic MPNST, in which disease has disseminated beyond the primary site. In both settings, the prognosis is generally poor, and the focus is on systemic disease control.
The standard of care in this setting is chemotherapy. An anthracycline-based regimen is the preferred first-line option — the complete details of the regimen, including the specific agents, their combinations, and the selection criteria between options, are set out in the full protocol.
DOI: 10.3390/cancers16193266
For inoperable or metastatic MPNST, the outcome is usually poor.
The standard of care in this setting remains chemotherapy.
An anthracycline-based chemotherapy (doxorubicin) single or in combination is the preferred first-line option, with response rates varying from 20 to 60%, depending on different studies.
Although the highest response rates are seen in regimens containing ifosfamide, there is no survival superiority to adding this drug (based on ECOG group—median OS 8.8 months in single agent doxorubicin versus 11.5 months in doxorubicin + ifosfamide).
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