Topics:
Sarcoma • Medical Oncology
What is your approach to systemic treatment of a rapidly progressing, symptomatic, unresectable MPNST arising from Plexiform Neurofibroma in a patient with NF1?
Would you consider using recently approved agent for symptomatic / unresectable PN, Selumetinib prior to Systemic Chemotherapy in this setting?
Answer from: Medical Oncologist at Academic Institution
The efficacy of standard sarcoma chemo, like adriamycin or ifosfamide in NF1 mutant MPNST specifically, is pretty dismal, and in my opinion we should be upfront with patients that success rates are probably on the order of 10% or less. For non-NF1 mutant MPNST, chemo is probably a little more effect...
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