{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T10:39:47Z","timestamp":1777891187325,"version":"3.51.4"},"reference-count":26,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Childs Nerv Syst"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Purpose<\/jats:title>\n                    <jats:p>Pediatric primary diffuse leptomeningeal melanomatosis (PDLM) is a rare, aggressive malignancy with no established standard of care. We performed an integrated survival analysis to characterize disease course and identify treatment factors associated with overall survival (OS).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A systematic literature search of PubMed, Embase, and Scopus (2000\u20132025) was conducted in accordance with PRISMA guidelines. Eligible studies included patients\u2009&lt;\u200918 years with histopathologically confirmed PDLM per 2021 World Health Organization and Expert Care for Rare Adult Solid Cancers criteria and reported OS and treatment data. Survival was assessed using Kaplan\u2013Meier analyses, with univariable and multivariable Cox proportional hazards regression.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Thirty-three pediatric PDLM cases were identified (57.6% male; median age 10 [IQR 3\u201314] years). Diagnosis was established via tissue sampling in 72.7%, cerebrospinal fluid cytology in 21.2%, and both in 6.1%. Among 13 patients with molecular testing, 69.2% harbored NRAS mutations. Twenty-four patients (72.7%) received treatment: chemotherapy (70.7%), radiation therapy (41.7%), and immunotherapy (62.5%). Median OS was 5.0 [3.5\u20137.0] months. On Kaplan\u2013Meier analysis, immunotherapy was associated with improved OS (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001), including when combined with radiation (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.038), chemotherapy (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.006), or both (p\u2009=\u20090.048). Univariable Cox regression showed immunotherapy to be significantly associated with reduced hazard of death (HR 0.23 [0.10\u20130.51],\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001). On multivariable analysis, immunotherapy (HR 0.22 [0.10\u20130.49],\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001) and chemotherapy (HR 0.44 [0.21\u20130.94],\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.035) were independently associated with improved OS.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Pediatric PDLM carries a uniformly poor prognosis. Immunotherapy and chemotherapy were the only treatments independently associated with improved survival, highlighting their central role in management of this rare disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00381-026-07264-2","type":"journal-article","created":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T05:56:31Z","timestamp":1777614991000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Impact of chemotherapy and immunotherapy on survival in pediatric primary diffuse leptomeningeal melanomatosis: a systematic review and pooled survival analysis"],"prefix":"10.1007","volume":"42","author":[{"given":"Khushi H.","family":"Shah","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mauricio J.","family":"Guerrero-Ocampo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Italo G.","family":"Flecha","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Caleigh S.","family":"Roach","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sima","family":"Vazquez","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Diego","family":"Servi\u00e1n","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Victor M.","family":"Lu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2026,5,1]]},"reference":[{"key":"7264_CR1","doi-asserted-by":"publisher","first-page":"e1091","DOI":"10.1016\/j.wneu.2019.07.095","volume":"130","author":"C Puyana","year":"2019","unstructured":"Puyana C, Denyer S, Burch T, Bhimani AD, McGuire LS, Patel AS et al (2019) Primary malignant melanoma of the brain: a population-based study. 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