Malignant peripheral nerve sheath tumor medical therapy

In the setting of localized disease, as is the case with all soft tissue sarcomas, complete surgical extirpation with clear margins is the treatment of choice.The predominant therapy for malignant peripheral nerve sheath tumor is surgical resection.In the setting of advanced or metastatic MPNST, outcomes are generally poor. Doxorubicin and ifosfamide are the most active agents in unselected soft tissue sarcomas, with a Response Evaluation Criteria in Solid Tumors (RECIST) response rate of approximately 25% for the combination. Adjunctive chemotherapy and radiation therapy may be required.Multiple retrospective datasets have shown the negative prognostic impact of involved margins and local recurrence. As in the case with most large (>5 cm) high-grade limb sarcomas, adjuvant radiation is advocated to reduce local recurrence. The risk-benefit profile of adjuvant radiation in patients with NF1 must be carefully discussed with all patients in view of the heightened risk of radiation-induced sarcomas.

Radiation

• Radiation will be used in conjunction with surgery, especially if the limb was not amputated. Radiation is rarely used as a sole treatment.
• As in the case with most large (>5 cm) high-grade limb sarcomas, adjuvant radiation is advocated to reduce local recurrence.
• The risk-benefit profile of adjuvant radiation in patients with NF1 must be carefully discussed with all patients in view of the heightened risk of radiation-induced sarcomas.

Chemotherapy

• In some instances, the oncologist may choose chemotherapy drugs when treating a patient with neurofibrosarcoma, usually in conjunction with surgery. Patients undergoing chemotherapy must be prepared for the side effects that come with any other chemotherapy treatment, such as hair loss, lethargy, weakness, etc.
• In the setting of advanced or metastatic MPNST, outcomes are generally poor.
• Doxorubicin and ifosfamide are the most active agents in unselected soft tissue sarcomas, with a Response Evaluation Criteria in Solid Tumors (RECIST) response rate of approximately 25% for the combination.
• There exist very limited data suggesting the possible greater activity of etoposide than doxorubicin in MPNST.
• A case series demonstrated significant responses to the combination of carboplatin and etoposide in two MPNST patients who were refractory to doxorubicin and ifosfamide
• Several other promising targets for therapy have emerged from recent preclinical studies.
• Histone deacetylase inhibitors (HDACi) have been found to induce cell death selectively in cells demonstrating enhanced ras signaling

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