{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,29]],"date-time":"2026-01-29T22:33:25Z","timestamp":1769726005792,"version":"3.49.0"},"reference-count":72,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"20","license":[{"start":{"date-parts":[[2025,11,17]],"date-time":"2025-11-17T00:00:00Z","timestamp":1763337600000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Neurosurg Case Lessons"],"published-print":{"date-parts":[[2025,11,17]]},"abstract":"<jats:sec>\n                    <jats:title>BACKGROUND<\/jats:title>\n                    <jats:p>\n                      Malignant melanotic nerve sheath tumors (MMNSTs) are rare neoplasms of Schwann cell origin that often arise along spinal nerves. They exhibit an estimated prevalence of 1% among primary peripheral nerve sheath tumors and carry an estimated mortality rate of 26%. This disease most commonly presents with localized pain and may also present with symptoms of cord compression. After radiographic imaging to identify suspected lesions, a definitive diagnosis is usually made through identification of\n                      <jats:italic>PRKAR1A<\/jats:italic>\n                      gene mutations. Typical management consists of tumor resection with adjuvant radiotherapy or chemotherapy, depending on case specifics. However, misdiagnosis due to tumor mimicking may slow correct diagnoses and subsequent treatment.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>OBSERVATIONS<\/jats:title>\n                    <jats:p>The authors present a unique case of MMNST mimicking a benign nerve sheath lesion that resulted in neuropathy. Initial pathology reports identified the lesion to be of metastatic melanoma, but reexamination resulted in an ultimate diagnosis of MMNST. Rapid progression of the disease was observed. A subsequent review of the literature identified diagnostic considerations, treatment strategies, and avenues of research that may aid in diagnosis and treatment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>LESSONS<\/jats:title>\n                    <jats:p>MMNST should be considered when encountering suspicious peripheral nerve sheath lesions. Promptly performing appropriate biopsies and molecular testing is critical due to this condition\u2019s poor prognosis.<\/jats:p>\n                    <jats:p>\n                      <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"https:\/\/thejns.org\/doi\/abs\/10.3171\/CASE25314\">https:\/\/thejns.org\/doi\/abs\/10.3171\/CASE25314<\/jats:ext-link>\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/case25314","type":"journal-article","created":{"date-parts":[[2026,1,29]],"date-time":"2026-01-29T11:09:33Z","timestamp":1769684973000},"source":"Crossref","is-referenced-by-count":0,"title":["Malignant melanotic nerve sheath tumor mimicking a benign nerve sheath lesion: illustrative case"],"prefix":"10.3171","volume":"10","author":[{"given":"Michael B.","family":"Lemonick","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, New York;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Siddharth","family":"Venigalla","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Virginia Commonwealth University School of Medicine, Richmond, Virginia;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kevin V.","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, New York;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9729-9122","authenticated-orcid":true,"given":"Bahie","family":"Ezzat","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, New York;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthew T.","family":"Carr","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, New York;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexander J.","family":"Schupper","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, New York;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cheyanne","family":"Slocum","sequence":"additional","affiliation":[{"name":"Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marc K.","family":"Rosenblum","sequence":"additional","affiliation":[{"name":"Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nadejda M.","family":"Tsankova","sequence":"additional","affiliation":[{"name":"Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, New York; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tanvir","family":"Choudhri","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, New York;"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"issue":"12","key":"bib1","series-title":"AJNR Am J Neuroradiol","first-page":"1696","article-title":"Malignant melanotic nerve sheath tumor","volume":"43","author":"Benson","year":"2022","unstructured":"Benson JCMarais MDFlanigan PMMalignant melanotic nerve sheath tumor. AJNR Am J Neuroradiol. 2022;43(12):1696-1699."},{"issue":"1","key":"bib2","series-title":"J Neurosurg Spine","first-page":"28","article-title":"Surgical management of malignant melanotic nerve sheath tumors: an institutional experience and systematic review of the literature","volume":"40","author":"Ghaith","year":"2024","unstructured":"Ghaith AKJohnson SEEl-Hajj VGSurgical management of malignant melanotic nerve sheath tumors: an institutional experience and systematic review of the literature. J Neurosurg Spine. 2024;40(1):28-37."},{"issue":"3","key":"bib3","series-title":"J Microsc Ultrastruct","first-page":"125","article-title":"Malignant melanotic nerve sheath tumors: a review of clinicopathologic and molecular characteristics","volume":"11","author":"Hammad","year":"2023","unstructured":"Hammad RM.Malignant melanotic nerve sheath tumors: a review of clinicopathologic and molecular characteristics. J Microsc Ultrastruct. 2023;11(3):125-129."},{"issue":"24","key":"bib4","series-title":"World J Clin Cases","first-page":"8735","article-title":"Malignant melanotic nerve sheath tumors in the spinal canal of psammomatous and non-psammomatous type: two case reports","volume":"10","author":"Yeom","year":"2022","unstructured":"Yeom JASong YSLee ISHan IHChoi KU.Malignant melanotic nerve sheath tumors in the spinal canal of psammomatous and non-psammomatous type: two case reports. World J Clin Cases. 2022;10(24):8735-8741."},{"issue":"1","key":"bib5","series-title":"Neurosurgery","first-page":"47","article-title":"Melanotic schwannoma","volume":"2","author":"McGavran","year":"1978","unstructured":"McGavran WLSypert GWBallinger WE.Melanotic schwannoma. Neurosurgery. 1978;2(1):47-51."},{"issue":"7","key":"bib6","series-title":"AJNR Am J Neuroradiol","first-page":"928","article-title":"The 2021 World Health Organization classification of tumors of the central nervous system: what neuroradiologists need to know","volume":"43","author":"Osborn","year":"2022","unstructured":"Osborn AGLouis DNPoussaint TYLinscott LLSalzman KL.The 2021 World Health Organization classification of tumors of the central nervous system: what neuroradiologists need to know. AJNR Am J Neuroradiol. 2022;43(7):928-937."},{"key":"bib7","series-title":"Front Oncol","first-page":"1100532","article-title":"Sporadic spinal psammomatous malignant melanotic nerve sheath tumor: a case report and literature review","volume":"13","author":"Bonomo","year":"2023","unstructured":"Bonomo GGans AMazzapicchi ESporadic spinal psammomatous malignant melanotic nerve sheath tumor: a case report and literature review. Front Oncol. 2023;13:1100532."},{"issue":"1","key":"bib8","series-title":"Am J Surg Pathol","first-page":"94","article-title":"Malignant melanotic schwannian tumor: a clinicopathologic, immunohistochemical, and gene expression profiling study of 40 cases, with a proposal for the reclassification of \u201cmelanotic schwannoma.\u201d","volume":"38","author":"Torres-Mora","year":"2014","unstructured":"Torres-Mora JDry SLi XBinder SAmin MFolpe AL.Malignant melanotic schwannian tumor: a clinicopathologic, immunohistochemical, and gene expression profiling study of 40 cases, with a proposal for the reclassification of \u201cmelanotic schwannoma.\u201d Am J Surg Pathol. 2014;38(1):94-105."},{"issue":"12","key":"bib9","series-title":"Arch Pathol Lab Med","first-page":"1517","article-title":"Pathology of melanotic schwannoma","volume":"142","author":"Alexiev","year":"2018","unstructured":"Alexiev BAChou PMJennings LJ.Pathology of melanotic schwannoma. Arch Pathol Lab Med. 2018;142(12):1517-1523."},{"issue":"8","key":"bib10","series-title":"Genes Chromosomes Cancer","first-page":"463","article-title":"Consistent copy number changes and recurrent PRKAR1A mutations distinguish melanotic schwannomas from Melanomas: SNP-array and next generation sequencing analysis","volume":"54","author":"Wang","year":"2015","unstructured":"Wang LZehir ASadowska JConsistent copy number changes and recurrent PRKAR1A mutations distinguish melanotic schwannomas from Melanomas: SNP-array and next generation sequencing analysis. Genes Chromosomes Cancer. 2015;54(8):463-471."},{"issue":"3","key":"bib11","series-title":"Am J Surg Pathol","first-page":"206","article-title":"Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome","volume":"14","author":"Carney","year":"1990","unstructured":"Carney JA.Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. Am J Surg Pathol. 1990;14(3):206-222."},{"key":"bib12","series-title":"MDText.com","article-title":"Carney complex","volume":"2023","author":"Kaltsas","unstructured":"Kaltsas GKanakis GChrousos G.Carney complex. In: Feingold KR, Ahmed SF, Anawalt B, et al., eds. Endotext. MDText.com; 2023. Accessed April 27, 2025. http:\/\/www.ncbi.nlm.nih.gov\/books\/NBK279117\/"},{"key":"bib13","series-title":"GeneReviews\u00ae","article-title":"Carney complex","author":"Stratakis","year":"2003","unstructured":"Stratakis CA.Carney complex. In: Adam MP, Feldman J, Mirzaa GM, et al., eds. GeneReviews\u00ae. University of Washington\u2013Seattle; 2003. Accessed April 27, 2025. http:\/\/www.ncbi.nlm.nih.gov\/books\/NBK1286\/"},{"issue":"1","key":"bib14","series-title":"BMC Neurol","first-page":"181","article-title":"Malignant melanotic schwannoma of the cervical spinal cord: a case report","volume":"24","author":"Chen","year":"2024","unstructured":"Chen SWei Y.Malignant melanotic schwannoma of the cervical spinal cord: a case report. BMC Neurol. 2024;24(1):181."},{"issue":"3-4 Pt 1","key":"bib15","series-title":"Neurochirurgie","first-page":"183","article-title":"Melanocytic schwannomas. Report of three cases","volume":"51","author":"Peltier","year":"2005","unstructured":"Peltier JPage CToussaint PBruniau ADesenclos CLe Gars D.Melanocytic schwannomas. Report of three cases. Neurochirurgie. 2005;51(3-4 Pt 1):183-189."},{"issue":"9","key":"bib16","series-title":"J Clin Endocrinol Metab","first-page":"4324","article-title":"Mutations of the PRKAR1A gene in Cushing\u2019s syndrome due to sporadic primary pigmented nodular adrenocortical disease","volume":"87","author":"Groussin","year":"2002","unstructured":"Groussin LJullian EPerlemoine KMutations of the PRKAR1A gene in Cushing\u2019s syndrome due to sporadic primary pigmented nodular adrenocortical disease. J Clin Endocrinol Metab. 2002;87(9):4324-4329."},{"issue":"4","key":"bib17","series-title":"Hum Mutat","first-page":"369","article-title":"Mutations and polymorphisms in the gene encoding regulatory subunit type 1-alpha of protein kinase A (PRKAR1A): an update","volume":"31","author":"Horvath","year":"2010","unstructured":"Horvath ABertherat JGroussin LMutations and polymorphisms in the gene encoding regulatory subunit type 1-alpha of protein kinase A (PRKAR1A): an update. Hum Mutat. 2010;31(4):369-379."},{"key":"bib18","series-title":"Hum Pathol","first-page":"90","article-title":"Prevalence and detection of actionable BRAF V600 and NRAS Q61 mutations in malignant peripheral nerve sheath tumor by droplet digital PCR","volume":"129","author":"Kao","year":"2022","unstructured":"Kao EYWakeman KMWu YPrevalence and detection of actionable BRAF V600 and NRAS Q61 mutations in malignant peripheral nerve sheath tumor by droplet digital PCR. Hum Pathol. 2022;129:90-97."},{"issue":"4","key":"bib19","series-title":"Br J Dermatol","first-page":"785","article-title":"CDKN2A mutations with p14 loss predisposing to multiple nerve sheath tumours, melanoma, dysplastic naevi and internal malignancies: a case series and review of the literature","volume":"175","author":"Sargen","year":"2016","unstructured":"Sargen MRMerrill SLChu EYNathanson KL.CDKN2A mutations with p14 loss predisposing to multiple nerve sheath tumours, melanoma, dysplastic naevi and internal malignancies: a case series and review of the literature. Br J Dermatol. 2016;175(4):785-789."},{"issue":"2","key":"bib20","series-title":"Semin Dermatol","first-page":"90","article-title":"Carney complex: the complex of myxomas, spotty pigmentation, endocrine overactivity, and schwannomas","volume":"14","author":"Carney","year":"1995","unstructured":"Carney JA.Carney complex: the complex of myxomas, spotty pigmentation, endocrine overactivity, and schwannomas. Semin Dermatol. 1995;14(2):90-98."},{"issue":"3","key":"bib21","series-title":"Mayo Clin Proc","first-page":"165","article-title":"Dominant inheritance of the complex of myxomas, spotty pigmentation, and endocrine overactivity","volume":"61","author":"Carney","year":"1986","unstructured":"Carney JAHruska LSBeauchamp GDGordon H.Dominant inheritance of the complex of myxomas, spotty pigmentation, and endocrine overactivity. Mayo Clin Proc. 1986;61(3):165-172."},{"issue":"2","key":"bib22","series-title":"Am J Med Genet","first-page":"183","article-title":"Carney complex: diagnosis and management of the complex of spotty skin pigmentation, myxomas, endocrine overactivity, and schwannomas","volume":"80","author":"Stratakis","year":"1998","unstructured":"Stratakis CAKirschner LSCarney JA.Carney complex: diagnosis and management of the complex of spotty skin pigmentation, myxomas, endocrine overactivity, and schwannomas. Am J Med Genet. 1998;80(2):183-185."},{"key":"bib23","series-title":"Soft Tissue Tumors: A Practical and Comprehensive Guide to Sarcomas and Benign Neoplasms","first-page":"543","article-title":"Melanotic schwannoma","author":"Mocellin","year":"2021","unstructured":"Mocellin S.Melanotic schwannoma. In: Mocellin S, ed. Soft Tissue Tumors: A Practical and Comprehensive Guide to Sarcomas and Benign Neoplasms. Springer International Publishing; 2021:543-545."},{"issue":"8","key":"bib24","series-title":"Medicine (Baltimore)","first-page":"e24803","article-title":"18F-FDG PET\/CT imaging for aggressive melanotic schwannoma of the L3 spinal root: a case report","volume":"100","author":"Shen","year":"2021","unstructured":"Shen XZWang WLuo ZY.18F-FDG PET\/CT imaging for aggressive melanotic schwannoma of the L3 spinal root: a case report. Medicine (Baltimore). 2021;100(8):e24803."},{"key":"bib25","series-title":"Surg Neurol Int","article-title":"Spinal malignant melanotic nerve sheath tumor with atypical magnetic resonance imaging findings: a case report","volume":"15","author":"Kageyama","year":"2024","unstructured":"Kageyama H.Spinal malignant melanotic nerve sheath tumor with atypical magnetic resonance imaging findings: a case report. Surg Neurol Int. 2024;15:250."},{"issue":"4","key":"bib26","series-title":"Int J Surg Pathol","first-page":"1068","article-title":"The \u201cpigmented side\u201d of nerve sheaths: malignant melanotic nerve sheath tumor","volume":"33","author":"Franca","year":"2025","unstructured":"Franca RADi Crescenzo RMUgga LDella Monica RD\u2019Avella E.The \u201cpigmented side\u201d of nerve sheaths: malignant melanotic nerve sheath tumor. 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Dev Biol. 2003;259(1):19-33."},{"issue":"5","key":"bib29","series-title":"Am J Pathol","first-page":"1717","article-title":"Anti-human Olig2 antibody as a useful immunohistochemical marker of normal oligodendrocytes and gliomas","volume":"164","author":"Yokoo","year":"2004","unstructured":"Yokoo HNobusawa STakebayashi HAnti-human Olig2 antibody as a useful immunohistochemical marker of normal oligodendrocytes and gliomas. Am J Pathol. 2004;164(5):1717-1725."},{"issue":"4","key":"bib30","series-title":"J Neurosci Rural Pract","first-page":"499","article-title":"Immunohistochemical expression of glial fibrillary acidic protein and CAM5.2 in glial tumors and their role in differentiating glial tumors from metastatic tumors of central nervous system","volume":"6","author":"Goyal","year":"2015","unstructured":"Goyal RMathur SKGupta SImmunohistochemical expression of glial fibrillary acidic protein and CAM5.2 in glial tumors and their role in differentiating glial tumors from metastatic tumors of central nervous system. J Neurosci Rural Pract. 2015;6(4):499-503."},{"issue":"12","key":"bib31","series-title":"Histopathology","first-page":"1315","article-title":"An analysis of the sensitivity and specificity of the cytokeratin marker CAM 5.2 for epithelial tumours. 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AJNR Am J Neuroradiol. 2022;43(12):1696-1699."},{"issue":"1","key":"bib2","series-title":"J Neurosurg Spine","first-page":"28","article-title":"Surgical management of malignant melanotic nerve sheath tumors: an institutional experience and systematic review of the literature","volume":"40","author":"Ghaith","year":"2024","unstructured":"Ghaith AKJohnson SEEl-Hajj VGSurgical management of malignant melanotic nerve sheath tumors: an institutional experience and systematic review of the literature. J Neurosurg Spine. 2024;40(1):28-37."},{"issue":"3","key":"bib3","series-title":"J Microsc Ultrastruct","first-page":"125","article-title":"Malignant melanotic nerve sheath tumors: a review of clinicopathologic and molecular characteristics","volume":"11","author":"Hammad","year":"2023","unstructured":"Hammad RM.Malignant melanotic nerve sheath tumors: a review of clinicopathologic and molecular characteristics. J Microsc Ultrastruct. 2023;11(3):125-129."},{"issue":"24","key":"bib4","series-title":"World J Clin Cases","first-page":"8735","article-title":"Malignant melanotic nerve sheath tumors in the spinal canal of psammomatous and non-psammomatous type: two case reports","volume":"10","author":"Yeom","year":"2022","unstructured":"Yeom JASong YSLee ISHan IHChoi KU.Malignant melanotic nerve sheath tumors in the spinal canal of psammomatous and non-psammomatous type: two case reports. World J Clin Cases. 2022;10(24):8735-8741."},{"issue":"1","key":"bib5","series-title":"Neurosurgery","first-page":"47","article-title":"Melanotic schwannoma","volume":"2","author":"McGavran","year":"1978","unstructured":"McGavran WLSypert GWBallinger WE.Melanotic schwannoma. Neurosurgery. 1978;2(1):47-51."},{"issue":"7","key":"bib6","series-title":"AJNR Am J Neuroradiol","first-page":"928","article-title":"The 2021 World Health Organization classification of tumors of the central nervous system: what neuroradiologists need to know","volume":"43","author":"Osborn","year":"2022","unstructured":"Osborn AGLouis DNPoussaint TYLinscott LLSalzman KL.The 2021 World Health Organization classification of tumors of the central nervous system: what neuroradiologists need to know. AJNR Am J Neuroradiol. 2022;43(7):928-937."},{"key":"bib7","series-title":"Front Oncol","first-page":"1100532","article-title":"Sporadic spinal psammomatous malignant melanotic nerve sheath tumor: a case report and literature review","volume":"13","author":"Bonomo","year":"2023","unstructured":"Bonomo GGans AMazzapicchi ESporadic spinal psammomatous malignant melanotic nerve sheath tumor: a case report and literature review. Front Oncol. 2023;13:1100532."},{"issue":"1","key":"bib8","series-title":"Am J Surg Pathol","first-page":"94","article-title":"Malignant melanotic schwannian tumor: a clinicopathologic, immunohistochemical, and gene expression profiling study of 40 cases, with a proposal for the reclassification of \u201cmelanotic schwannoma.\u201d","volume":"38","author":"Torres-Mora","year":"2014","unstructured":"Torres-Mora JDry SLi XBinder SAmin MFolpe AL.Malignant melanotic schwannian tumor: a clinicopathologic, immunohistochemical, and gene expression profiling study of 40 cases, with a proposal for the reclassification of \u201cmelanotic schwannoma.\u201d Am J Surg Pathol. 2014;38(1):94-105."},{"issue":"12","key":"bib9","series-title":"Arch Pathol Lab Med","first-page":"1517","article-title":"Pathology of melanotic schwannoma","volume":"142","author":"Alexiev","year":"2018","unstructured":"Alexiev BAChou PMJennings LJ.Pathology of melanotic schwannoma. 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