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When compared with other reports in the literature, this case stands out on IHC, presenting an atypical set of findings, previously outside the patterns reported in MPNST related to NF1.10 Negative for S100 and SOX10, weakly positive CD34, and positive H3K27me3 are extremely rare and, as previously mentioned, indicative of a worse prognosis, which may explain the extremely aggressive behavior of the reported MPNST, which recurred with metastatic lesions 68 days after surgery

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