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Recurrent MPNST
Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 21, 2025 · Captured 2026-09-09.
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Context: Treatment of Peripheral Nerve Sheath Tumors / Malignant Peripheral Nerve Sheath Tumor (MPNST) NOS
Of 120 patients enrolled in Italian pediatric protocols from 1979 to 2004, an analysis identified 73 patients younger than 21 years with relapsed MPNST. Treatment options included surgery, radiation therapy, and chemotherapy.[23]
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The time to relapse from initial diagnosis ranged from 1 month to 204 months, with a median time to relapse of 7 months.
Median OS from first relapse was 11 months, with an OS rate of 39% at 1 year and 16% at 5 years.
The factors associated with a higher probability of survival after relapse were lower tumor invasiveness at initial presentation, longer time to relapse, and complete surgical resection of the tumor at relapse.
A retrospective study evaluated nine patients with unresectable or metastatic MPNST (seven patients were previously treated) who were treated with selinexor with or without doxorubicin. Three patients experienced a partial response that lasted for 3 months to longer than 8 months, and four patients had stable disease.[24]
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Preserved source evidence · Not medical advice