ArticleJournal of neurosurgery. Case lessons2026
Diagnosing metastatic angiosarcoma arising from MPNST using H3K27me3 staining in NF1: illustrative case.
Article in Journal of neurosurgery. Case lessons, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundMalignant peripheral nerve sheath tumors (MPNSTs) are malignant tumors that rarely demonstrate heterologous differentiation from angiosarcomatous components. These lesions can be hard to accurately diagnose. OBSERVATIONS: A 25-year-old man with neurofibromatosis type 1 and a history of a left lumbar subcutaneous MPNST developed a lung metastasis of only the angiosarcoma component of the MPNST. Prior to H3K27me3 staining of the lung mass, there was no association of the angiosarcoma with the MPNST. Reinterpretation of the lumbar subcutaneous MPNST led to the identification of a previously unrecognized angiosarcomatous portion in that tumor as well. LESSONS: This case highlights the importance of using H327me3 staining for accurate diagnosis of biopsies only capturing the heterologous components of MPNST. https://thejns.org/doi/10.3171/CASE251012.
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