Malignant peripheral nerve sheath tumor medical therapy: Difference between revisions

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==Medical Therapy==
==Medical Therapy==
'''Radiation'''
'''Radiation'''
* [[Radiation]] will be used in conjunction with surgery, especially if the limb was not amputated. Radiation is rarely used as a sole treatment.<ref>Malignant peripheral nerve sheath tumor. Wikipedia 2015. https://en.wikipedia.org/wiki/Malignant_peripheral_nerve_sheath_tumor#cite_note-Valeyrie-Allanore_2005_79-4</ref>
* [[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.  
*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.
*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'''
'''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 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.


==References==
==References==

Revision as of 13:40, 27 August 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]

Overview

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.

Medical Therapy

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.

References

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