{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T03:08:23Z","timestamp":1777604903707,"version":"3.51.4"},"reference-count":119,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2023,10,27]],"date-time":"2023-10-27T00:00:00Z","timestamp":1698364800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pigment Cell Melanoma Res"],"published-print":{"date-parts":[[2024,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Primary central nervous system (CNS) melanoma is an extremely rare condition, with an incidence rate of 0.01 per 100,000 individuals per year. Despite its rarity, the etiology and pathogenesis of this disease are not yet fully understood. Primary CNS melanoma exhibits highly aggressive biological behavior and presents clinically in a distinct manner from other types of melanomas. It can develop at any age, predominantly affecting the meninges as the primary site, with clinical symptoms varying depending on the neoplasm\u2019s location. Due to the lack of specificity in its presentation and the challenging nature of imaging diagnosis, distinguishing primary CNS melanoma from other CNS diseases. The combination of challenges in early detection, heightened tumor aggressiveness, and the obscured location of its origin contribute to an unfavorable prognostic outcome. Furthermore, there has been currently no consensus on a standardized treatment approach for primary CNS melanoma. Despite recent advancements in targeted therapy and immunotherapy for CNS melanoma, patients with primary CNS melanoma have limited treatment options due to their inadequate response to these therapies. Here, we provided a comprehensive summary of the epidemiology, clinical features, molecular pathological manifestations, and available diagnostic and therapeutic approaches of primary CNS melanoma. Additionally, we proposed potential therapeutic strategies for it.<\/jats:p>","DOI":"10.1111\/pcmr.13140","type":"journal-article","created":{"date-parts":[[2023,10,27]],"date-time":"2023-10-27T08:20:13Z","timestamp":1698394813000},"page":"265-275","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Clinicopathological features, current status, and progress of primary central nervous system melanoma diagnosis and treatment"],"prefix":"10.1111","volume":"37","author":[{"given":"Pengna","family":"Guo","sequence":"first","affiliation":[{"name":"Cancer Center The First Hospital Of Jilin University  Changchun China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaoting","family":"Wei","sequence":"additional","affiliation":[{"name":"Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education\/Beijing), Department of Melanoma and Sarcoma Peking University Cancer Hospital &amp; Institute  Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhen","family":"Guo","sequence":"additional","affiliation":[{"name":"Cancer Center The First Hospital Of Jilin University  Changchun China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1870-798X","authenticated-orcid":false,"given":"Di","family":"Wu","sequence":"additional","affiliation":[{"name":"Cancer Center The First Hospital Of Jilin University  Changchun China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,10,27]]},"reference":[{"key":"e_1_2_16_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000012121"},{"key":"e_1_2_16_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097\u20100142(19821101)50:9<1751::aid\u2010cncr2820500918>3.0.co;2\u2010q"},{"key":"e_1_2_16_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40618\u2010019\u201001158\u20108"},{"key":"e_1_2_16_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/bjc.2014.541"},{"key":"e_1_2_16_6_1","doi-asserted-by":"publisher","DOI":"10.1227\/00006123\u2010199305000\u201000004"},{"key":"e_1_2_16_7_1","doi-asserted-by":"publisher","DOI":"10.1158\/2326\u20106066.CIR\u201021\u20100870"},{"key":"e_1_2_16_8_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12885\u2010016\u20102556\u2010y"},{"key":"e_1_2_16_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clineuro.2010.08.003"},{"key":"e_1_2_16_10_1","doi-asserted-by":"publisher","DOI":"10.1177\/1971400917708581"},{"key":"e_1_2_16_11_1","doi-asserted-by":"publisher","DOI":"10.1155\/2015\/293802"},{"key":"e_1_2_16_12_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078\u20100432.CCR\u201008\u20100575"},{"key":"e_1_2_16_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/bf01406550"},{"key":"e_1_2_16_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00701\u2010005\u20100705\u20101"},{"key":"e_1_2_16_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2022.06.007"},{"key":"e_1_2_16_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neurol.2010.03.011"},{"issue":"4","key":"e_1_2_16_17_1","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1111\/j.1699-0463.1977.tb03875.x","article-title":"Primary cerebral melanomas. Report of six cases and a review of the literature","volume":"85","author":"Bojsen\u2010M\u00f8ller M.","year":"1977","journal-title":"Acta Pathologica et Microbiologica Scandinavica. Section A"},{"key":"e_1_2_16_18_1","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2021.691017"},{"key":"e_1_2_16_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clineuro.2017.11.012"},{"key":"e_1_2_16_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2015.05.044"},{"key":"e_1_2_16_21_1","doi-asserted-by":"publisher","DOI":"10.1080\/02688697.2020.1834511"},{"key":"e_1_2_16_22_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1993.00540030027009"},{"key":"e_1_2_16_23_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.Mp.0000072747.08404.38"},{"key":"e_1_2_16_24_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2011.41.2452"},{"key":"e_1_2_16_25_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1981.00510080090015"},{"key":"e_1_2_16_26_1","doi-asserted-by":"publisher","DOI":"10.3171\/jns.2000.92.3.0461"},{"key":"e_1_2_16_27_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401\u2010015\u20101423\u20102"},{"key":"e_1_2_16_28_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365\u20102559.2004.01860.x"},{"key":"e_1_2_16_29_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaophthalmol.2016.0903"},{"key":"e_1_2_16_30_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2011.37.3175"},{"key":"e_1_2_16_31_1","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.152.1.137"},{"key":"e_1_2_16_32_1","doi-asserted-by":"publisher","DOI":"10.1038\/bjc.2013.804"},{"key":"e_1_2_16_33_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11060\u2010018\u20102848\u2010y"},{"key":"e_1_2_16_34_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiographics.21.3.g01ma19625"},{"issue":"6","key":"e_1_2_16_35_1","first-page":"1087","article-title":"Melanocytoma of the cavernous sinus: CT and MR findings","volume":"17","author":"Faro S. H.","year":"1996","journal-title":"AJNR. American Journal of Neuroradiology"},{"issue":"10","key":"e_1_2_16_36_1","first-page":"1864","article-title":"MR findings of a primary intramedullary malignant melanoma: Case report and literature review","volume":"22","author":"Farrokh D.","year":"2001","journal-title":"AJNR. American Journal of Neuroradiology"},{"key":"e_1_2_16_37_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0142(19960415)77:8<1544::AID-CNCR18>3.0.CO;2-#"},{"key":"e_1_2_16_38_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2006.06.0442"},{"key":"e_1_2_16_39_1","doi-asserted-by":"publisher","DOI":"10.1080\/02688697.2020.1864291"},{"key":"e_1_2_16_40_1","doi-asserted-by":"publisher","DOI":"10.1111\/pcmr.12839"},{"key":"e_1_2_16_41_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10143\u2010017\u20100914\u20100"},{"key":"e_1_2_16_42_1","doi-asserted-by":"publisher","DOI":"10.1038\/cgt.2016.56"},{"key":"e_1_2_16_43_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365\u20102990.2012.01288.x"},{"key":"e_1_2_16_44_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.1700740220"},{"key":"e_1_2_16_45_1","doi-asserted-by":"publisher","DOI":"10.1136\/esmoopen\u20102017\u2010000283"},{"key":"e_1_2_16_46_1","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.12363"},{"key":"e_1_2_16_47_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0090\u20103019(01)00348\u20102"},{"key":"e_1_2_16_48_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jocn.2019.03.029"},{"key":"e_1_2_16_49_1","doi-asserted-by":"publisher","DOI":"10.4103\/0028\u20103886.173644"},{"key":"e_1_2_16_50_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.29468"},{"key":"e_1_2_16_51_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13760\u2010018\u20100931\u2010y"},{"key":"e_1_2_16_52_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.39.6.526"},{"key":"e_1_2_16_53_1","doi-asserted-by":"publisher","DOI":"10.3109\/02841868909111189"},{"key":"e_1_2_16_54_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2013.50.0264"},{"key":"e_1_2_16_55_1","doi-asserted-by":"publisher","DOI":"10.4103\/0028\u20103886.82758"},{"key":"e_1_2_16_56_1","doi-asserted-by":"publisher","DOI":"10.4184\/asj.2010.4.1.48"},{"key":"e_1_2_16_57_1","doi-asserted-by":"publisher","DOI":"10.3171\/jns.2006.104.3.444"},{"key":"e_1_2_16_58_1","doi-asserted-by":"publisher","DOI":"10.3171\/jns.1961.18.5.0616"},{"key":"e_1_2_16_59_1","doi-asserted-by":"publisher","DOI":"10.1038\/bjc.2017.49"},{"key":"e_1_2_16_60_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2007.11.1807"},{"key":"e_1_2_16_61_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.3361"},{"key":"e_1_2_16_62_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40478\u2010016\u20100272\u20100"},{"key":"e_1_2_16_63_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12253\u2010014\u20109841\u20103"},{"key":"e_1_2_16_64_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yexmp.2016.12.006"},{"key":"e_1_2_16_65_1","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.12241"},{"key":"e_1_2_16_66_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ydbio.2018.07.019"},{"key":"e_1_2_16_67_1","doi-asserted-by":"publisher","DOI":"10.3348\/kjr.2013.14.2.343"},{"key":"e_1_2_16_68_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00586\u2010010\u20101285\u20101"},{"key":"e_1_2_16_69_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cllc.2022.11.011"},{"key":"e_1_2_16_70_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcb.27863"},{"key":"e_1_2_16_71_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jocn.2010.01.017"},{"key":"e_1_2_16_72_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0887\u20108994(01)00261\u20102"},{"issue":"5","key":"e_1_2_16_73_1","first-page":"387","article-title":"Same genes, different brains: Neuroanatomical differences between monozygotic twins discordant for musical training","volume":"28","author":"Martin\u2010Blondel G.","year":"2009","journal-title":"Clinical Neuropathology"},{"issue":"5","key":"e_1_2_16_74_1","first-page":"387","article-title":"Primary pineal melanoma with leptomeningeal spreading: Case report and review of the literature","volume":"28","author":"Martin\u2010Blondel G.","year":"2009","journal-title":"Clinical Neuropathology"},{"key":"e_1_2_16_75_1","doi-asserted-by":"publisher","DOI":"10.1002\/mpo.2950190108"},{"key":"e_1_2_16_76_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0967\u20105868(98)90078\u20109"},{"key":"e_1_2_16_77_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0028\u20103770(04)98304\u2010x"},{"key":"e_1_2_16_78_1","doi-asserted-by":"publisher","DOI":"10.1227\/00006123\u2010198112000\u201000017"},{"key":"e_1_2_16_79_1","doi-asserted-by":"publisher","DOI":"10.1016\/0887\u20108994(94)00155\u2010u"},{"issue":"6","key":"e_1_2_16_80_1","first-page":"542","article-title":"Meningeal melanocytoma. An uncommon diagnostic pitfall in surgical neuropathology","volume":"119","author":"O'Brien T. F.","year":"1995","journal-title":"Archives of Pathology & Laboratory Medicine"},{"key":"e_1_2_16_81_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600\u20100560.2007.00891.x"},{"key":"e_1_2_16_82_1","doi-asserted-by":"publisher","DOI":"10.1227\/00006123\u2010198407000\u201000020"},{"issue":"7","key":"e_1_2_16_83_1","first-page":"1349","article-title":"Intracranial and intraspinal meningeal melanocytosis","volume":"21","author":"Painter T. J.","year":"2000","journal-title":"AJNR. American Journal of Neuroradiology"},{"key":"e_1_2_16_84_1","doi-asserted-by":"publisher","DOI":"10.1186\/1477\u20107819\u201012\u2010265"},{"key":"e_1_2_16_85_1","doi-asserted-by":"publisher","DOI":"10.1227\/01.Neu.0000372919.96651.34"},{"key":"e_1_2_16_86_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10544\u2010014\u20109908\u20107"},{"key":"e_1_2_16_87_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.wneu.2019.07.095"},{"key":"e_1_2_16_88_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00066\u2010002\u20100930\u2010y"},{"key":"e_1_2_16_89_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10227\u2010001\u20100004\u20102"},{"key":"e_1_2_16_90_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1904059"},{"key":"e_1_2_16_91_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2003.10.014"},{"key":"e_1_2_16_92_1","doi-asserted-by":"publisher","DOI":"10.3171\/jns.2004.101.3.0528"},{"key":"e_1_2_16_93_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jocn.2018.01.052"},{"issue":"8","key":"e_1_2_16_94_1","first-page":"1404","article-title":"Increased uptake of technetium\u201099m\u2010hexamethylpropyleneamine oxime related to primary leptomeningeal melanoma","volume":"23","author":"Sagiuchi T.","year":"2002","journal-title":"AJNR. American Journal of Neuroradiology"},{"key":"e_1_2_16_95_1","doi-asserted-by":"publisher","DOI":"10.1136\/pgmj.65.764.387"},{"key":"e_1_2_16_96_1","doi-asserted-by":"publisher","DOI":"10.3171\/jns.1998.89.4.0659"},{"key":"e_1_2_16_97_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2012.46.5773"},{"key":"e_1_2_16_98_1","doi-asserted-by":"publisher","DOI":"10.3171\/jns.1987.67.6.0923"},{"key":"e_1_2_16_99_1","doi-asserted-by":"publisher","DOI":"10.1055\/s\u20102008\u20101046822"},{"key":"e_1_2_16_100_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078\u20100432.CCR\u201021\u20101694"},{"key":"e_1_2_16_101_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2012.41.6115"},{"key":"e_1_2_16_102_1","doi-asserted-by":"publisher","DOI":"10.1016\/0149\u2010936x(85)90029\u20103"},{"key":"e_1_2_16_103_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.wneu.2016.06.039"},{"key":"e_1_2_16_104_1","doi-asserted-by":"publisher","DOI":"10.1055\/s\u20102007\u2010993162"},{"key":"e_1_2_16_105_1","doi-asserted-by":"publisher","DOI":"10.3171\/jns.2001.94.3.0528"},{"issue":"3","key":"e_1_2_16_106_1","first-page":"287","article-title":"Spinal meningeal melanocytoma. Report of two cases and review of the literature","volume":"100","author":"Turhan T.","year":"2004","journal-title":"Journal of Neurosurgery"},{"key":"e_1_2_16_107_1","doi-asserted-by":"publisher","DOI":"10.1007\/bf02478943"},{"key":"e_1_2_16_108_1","doi-asserted-by":"publisher","DOI":"10.1111\/pcmr.12843"},{"key":"e_1_2_16_109_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jid.2016.03.031"},{"key":"e_1_2_16_110_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jid.2017.04.022"},{"key":"e_1_2_16_111_1","doi-asserted-by":"publisher","DOI":"10.1590\/0004\u2010282x\u2010anp\u20102021\u20100082"},{"key":"e_1_2_16_112_1","doi-asserted-by":"publisher","DOI":"10.4103\/0028\u20103886.72209"},{"key":"e_1_2_16_113_1","doi-asserted-by":"publisher","DOI":"10.3171\/spi.2007.6.5.451"},{"key":"e_1_2_16_114_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440\u20101789.2010.01160.x"},{"key":"e_1_2_16_115_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejso.2013.11.024"},{"issue":"1","key":"e_1_2_16_116_1","first-page":"214","article-title":"MR characteristics of a primary melanoma of the quadrigeminal plate","volume":"9","author":"Weindling S. M.","year":"1988","journal-title":"AJNR. American Journal of Neuroradiology"},{"key":"e_1_2_16_117_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.1710990211"},{"key":"e_1_2_16_118_1","doi-asserted-by":"publisher","DOI":"10.14791\/btrt.2022.0022"},{"key":"e_1_2_16_119_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiology.165.1.3628773"},{"key":"e_1_2_16_120_1","doi-asserted-by":"publisher","DOI":"10.3109\/00313021003631205"}],"container-title":["Pigment Cell &amp; Melanoma Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pcmr.13140","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,2,16]],"date-time":"2024-02-16T07:06:06Z","timestamp":1708067166000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pcmr.13140"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,10,27]]},"references-count":119,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2024,3]]}},"alternative-id":["10.1111\/pcmr.13140"],"URL":"https:\/\/doi.org\/10.1111\/pcmr.13140","archive":["Portico"],"relation":{},"ISSN":["1755-1471","1755-148X"],"issn-type":[{"value":"1755-1471","type":"print"},{"value":"1755-148X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,10,27]]},"assertion":[{"value":"2023-04-15","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-09-23","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-10-27","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}