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Background <p>Langerhans 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.</p> Results <p>The 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%). <i>CBL</i> 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%.</p> Conclusions <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.</p> Clinical trial registration <p>Not applicable.</p>

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Clinical characteristics, genomic profiling, treatments, and outcomes of Langerhans cell sarcoma

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年1月20日

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  • Min Lang,
  • Xiao-juan Zheng,
  • Long Chang,
  • Dao-bin Zhou,
  • Wei Zhang,
  • Xin-xin Cao

摘要

Background

Langerhans 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.

Results

The 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%.

Conclusions

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.

Clinical trial registration

Not applicable.