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To address this knowledge gap, we conducted a retrospective analysis of 13 LCS patients. This retrospective study included patients\u2009\u2265\u200918 years old with biopsy proven LCS from October 2015 to April 2025.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The median age at diagnosis was 59 years (range: 33\u201371). The most commonly affected organs were the subcutaneous soft tissue (61.5%), followed by lymph nodes (53.8%), skin (30.8%), and bone (23.1%).\n                      <jats:italic>CBL<\/jats:italic>\n                      was the most common mutation, detected in four patients (33.3%). Notably, first-line treatment options included surgery and chemotherapy, with an overall response rate of 53.8%. Among all the relapsed or refractory patients, three eventually received targeted therapies (two trametinib and one niraparib), demonstrating promising efficacy with all patients achieved partial remission. With a median follow-up of 18.2 months (range: 2.6\u201393.1), the estimated 2-year overall survival rate was 92.3%, while the estimated 2-year progression-free survival (PFS) rate stood at 32.9%.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>In our cohort of LCS, we found that the PFS of LCS was poor. Genetic sequencing and the use of targeted therapies may offer a survival advantage for patients with LCS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Clinical trial registration<\/jats:title>\n                    <jats:p>Not applicable.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s13023-026-04199-4","type":"journal-article","created":{"date-parts":[[2026,1,20]],"date-time":"2026-01-20T11:36:19Z","timestamp":1768908979000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Clinical characteristics, genomic profiling, treatments, and outcomes of Langerhans cell sarcoma"],"prefix":"10.1186","volume":"21","author":[{"given":"Min","family":"Lang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiao-juan","family":"Zheng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Long","family":"Chang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dao-bin","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7884-3073","authenticated-orcid":false,"given":"Xin-xin","family":"Cao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,1,20]]},"reference":[{"key":"4199_CR1","doi-asserted-by":"publisher","first-page":"2672","DOI":"10.1182\/blood-2016-01-690636","volume":"127","author":"J-F Emile","year":"2016","unstructured":"Emile J-F, et al. 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