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However, given the shift in diagnostic pathology toward the use of integrated histopathologic and genomic approaches, the incorporation of additional molecular strata in the classification of Neurofibromatosis Type 1 (NF1)-associated peripheral nerve sheath tumors should be formalized to aid in accurate diagnosis and early identification of malignant transformation and enable appropriate intervention for affected patients. To this end, we assembled a multi-institutional expert pathology working group as part of a \u201cSymposium on Atypical Neurofibroma: State of the Science.\u201d Herein, we provide a suggested framework for adequate interventional radiology and surgical sampling and recommend molecular profiling for clinically or radiologically worrisome noncutaneous lesions in patients with NF1 to identify diagnostically-relevant molecular features, including CDKN2A\/B inactivation for ANNUBP, as well as SUZ12, EED, or TP53 inactivating mutations, or significant aneuploidy for MPNST. We also propose renaming \u201clow-grade MPNST\u201d to \u201cANNUBP with increased proliferation\u201d to avoid the use of the \u201cmalignant\u201d term in this group of tumors with persistent unknown biologic potential. This refined integrated diagnostic approach for NF1-associated peripheral nerve sheath tumors should continue to evolve in concert with our understanding of these neoplasms.<\/jats:p>","DOI":"10.1093\/neuonc\/noae235","type":"journal-article","created":{"date-parts":[[2024,11,5]],"date-time":"2024-11-05T15:22:37Z","timestamp":1730820157000},"page":"616-624","source":"Crossref","is-referenced-by-count":49,"title":["Consensus recommendations for an integrated diagnostic approach to peripheral nerve sheath tumors arising in the setting of Neurofibromatosis Type 1"],"prefix":"10.1093","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8347-9592","authenticated-orcid":false,"given":"Calixto-Hope G","family":"Lucas","sequence":"first","affiliation":[{"name":"Department of Oncology, Johns Hopkins University School of Medicine , Baltimore, Maryland ,","place":["USA"]},{"name":"Department of Neurosurgery, Johns Hopkins University School of Medicine , Baltimore, Maryland 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