Malignant peripheral nerve sheath tumors(MPNST) of the bladder is a very rare malignant tumor, usually secondary to neurofibromatosis type 1 (NF-1), with a few sporadic cases. This paper reports a case of 70-year-old man with bladder MPNST who underwent transurethral resection. Adjuvant intravesical instillation with gemcitabine was given after surgery, and there was no local recurrence and distant metastasis after 12-month follow-up. This paper also made a corresponding literature review.
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