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Lehman Department of Pathology and Laboratory Medicine Brown Cancer Center Department of Biochemistry and Molecular Genetics University of Louisville 505 South Hancock Street Louisville, KY 40202 USA [email protected] Submitted: 03 April 2022 Accepted: 12 August 2022 Copyedited by: Jeffrey Nirschl Published: 26 August 2022 Keywords: Malignant melanotic nerve sheath tumor, MMNST, Psammomatous melanotic schwannoma, Nerve sheath tumor, Pigmented epithelioid melanocytoma, PRKAR1A, KMT2C, GNAQ Abstract Malignant melanotic nerve sheath tumor (MMNST) is a rare and potentially aggressive lesion defined in the 2021 WHO Classification of Tumors of the Central Nervous System

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