RT Journal Article SR Electronic T1 Malignant Melanotic Nerve Sheath Tumor JF American Journal of Neuroradiology JO Am. J. Neuroradiol. FD American Society of Neuroradiology DO 10.3174/ajnr.A7691 A1 Benson, J.C. A1 Marais, M.D. A1 Flanigan, P.M. A1 Bydon, M. A1 Giannini, C. A1 Spinner, R.J. A1 Folpe, A.L. YR 2022 UL http://www.ajnr.org/content/early/2022/10/27/ajnr.A7691.abstract AB SUMMARY: Malignant melanotic nerve sheath tumors are uncommon pigmented tumors of Schwann cell origin, most often found along the spinal nerves. Although well-described in the literature, the tumors are quite rare, making up <1% of nerve sheath tumors. Physicians are, therefore, often unfamiliar with both the appearance and the optimal treatment of such tumors. Morphologically, many imaging features overlap with schwannomas and neurofibromas. Nevertheless, the malignant melanotic nerve sheath tumors are crucial to identify. They can be extremely aggressive, and the management of these tumors is considerably different from their benign counterparts. In this radiology-pathology review, we will highlight the imaging appearance, histologic features, surgical resection, and subsequent therapeutic strategies in a patient with a lumbar malignant melanotic nerve sheath tumor.MMNSTmalignant melanotic nerve sheath tumor