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They can be benign or malignant and occur in adults as well as in children, the latter often in the context of neurocutaneous melanosis. Until recently, the genetic alterations in these tumors were largely unknown. This is in contrast with cutaneous and uveal melanomas, which are known to harbor distinct oncogenic mutations that can be used as targets for treatment with small\u2010molecule inhibitors in the advanced setting. Recently, novel insights in the molecular alterations underlying primary melanocytic tumors of the\n                    <jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content>\n                    were obtained, including different oncogenic mutations in tumors in adult patients (especially\n                    <jats:styled-content style=\"fixed-case\">\n                      <jats:italic>GNAQ<\/jats:italic>\n                    <\/jats:styled-content>\n                    ,\n                    <jats:styled-content style=\"fixed-case\">\n                      <jats:italic>GNA11<\/jats:italic>\n                    <\/jats:styled-content>\n                    ) vs. children (especially\n                    <jats:styled-content style=\"fixed-case\">\n                      <jats:italic>NRAS<\/jats:italic>\n                    <\/jats:styled-content>\n                    ). In this review, the focus is on molecular characteristics of primary melanocytic tumors of the\n                    <jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content>\n                    . We summarize what is known about their genetic alterations and discuss implications for pathogenesis and differential diagnosis with other pigmented tumors in or around the\n                    <jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content>\n                    . Finally, new therapeutic options with targeted therapy are discussed.\n                  <\/jats:p>","DOI":"10.1111\/bpa.12241","type":"journal-article","created":{"date-parts":[[2014,12,23]],"date-time":"2014-12-23T05:18:48Z","timestamp":1419311928000},"page":"209-226","source":"Crossref","is-referenced-by-count":122,"title":["Primary Melanocytic Tumors of the Central Nervous System: a Review with Focus on Molecular Aspects"],"prefix":"10.1111","volume":"25","author":[{"given":"Heidi V.N.","family":"K\u00fcsters\u2010Vandevelde","sequence":"first","affiliation":[{"name":"Department of Pathology Canisius Wilhelmina Hospital  P.O. Box\u20099015 6500 GS Nijmegen"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benno","family":"K\u00fcsters","sequence":"additional","affiliation":[{"name":"Department of Pathology Radboud University Medical Center  P.O. Box\u20099101 6500 HB Nijmegen"},{"name":"Department of Pathology Maastricht University Medical Center  P.O. 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