Treating Malignant Peripheral Nerve Sheath Tumors
Name of the Trial
Why This Trial Is Important
Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that form in the outer layers of peripheral nerves (nerves outside the brain and spinal cord). About half of MPNSTs are found in individuals with a hereditary condition called neurofibromatosis type 1 (NF1). Surgery is the only curative treatment for MPNSTs. The prognosis for patients with unresectable tumors (tumors that cannot be surgically removed) is poor.
In this trial, patients with unresectable MPNSTs will be treated with neoadjuvant chemotherapy followed by surgery and/or radiation therapy and more chemotherapy. Neoadjuvant chemotherapy is given to reduce the size of the MPNSTs prior to the administration of definitive local therapy (surgery and/or radiation therapy). The choice of treatment following neoadjuvant chemotherapy will be based on tumor location and tumor response to the chemotherapy.
"We hope MPNSTs shrink in response to neoadjuvant chemotherapy with doxorubicin, ifosfamide, and etoposide, which are standard agents with proven activity in other pediatric and adult sarcomas," said Dr. Widemann.
"Furthermore, because outcomes for NF1-associated MPNSTs have been reported to be worse compared with sporadic tumors," Dr. Widemann said, "we will evaluate outcomes in the two groups treated with identical therapy to determine if patients with NF1-associated MPNSTs face a worse prognosis."
Who Can Join This Trial
Study Sites and Contact Information
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