ArticleOrphanet journal of rare diseases2026
Clinical characteristics, genomic profiling, treatments, and outcomes of Langerhans cell sarcoma.
Article in Orphanet journal of rare diseases, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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Who cites it
1 citing paper in PubMed.
- Differential Expression of Apoptosis-Stimulating Proteins of p53 (ASPPs) Between Langerhans Cell Histiocytosis and Langerhans Cell Sarcoma.Diagnostics (Basel, Switzerland) · 2026Article
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Authors and funding
6 authors.
Funding
Abstract
backgroundLangerhans cell sarcoma (LCS), an exceptionally rare and aggressive neoplasm, remains poorly characterized due to its scarcity. To address this knowledge gap, we conducted a retrospective analysis of 13 LCS patients. This retrospective study included patients ≥ 18 years old with biopsy proven LCS from October 2015 to April 2025.
resultsThe median age at diagnosis was 59 years (range: 33–71). 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%). CBL 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: 2.6–93.1), the estimated 2-year overall survival rate was 92.3%, while the estimated 2-year progression-free survival (PFS) rate stood at 32.9%.
conclusionsIn 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. CLINICAL
trial registrationNot applicable.
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