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Langerhans cell sarcoma: case report and review of world literature
Ted Zwerdling1, Eric Won, Lisa Shane
1*Miller Children's Hospital, Long Beach †Department of Pediatrics, University of California, Irvine, CA ‡Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA.
Insights
Langerhans cell sarcoma is a rare pediatric cancer. This report details a second pediatric case with successful treatment and long-term remission, offering guidance for physicians.
Area of Science:
- Pediatric Oncology
- Hematology
- Oncogenesis
Background:
- Langerhans cell sarcoma (LCS) is an exceptionally rare malignant neoplasm.
- Pediatric cases of LCS are exceedingly uncommon, with only one previously reported instance.
Observation:
- We present the second reported case of pediatric LCS in a young patient.
- The child presented with symptomatic spinal cord compression, a critical manifestation.
Findings:
- The patient underwent a multidisciplinary treatment approach including surgical resection, chemotherapy, and radiation therapy.
- The child achieved a durable remission lasting 27 months post-treatment.
- A comprehensive review of 53 published LCS cases worldwide is included to aid clinical management.
Implications:
- This case highlights the possibility of successful outcomes in pediatric LCS with aggressive multimodal therapy.
- The findings underscore the importance of early diagnosis and prompt intervention for spinal cord compression in pediatric LCS.
- Discussion of the BRAF genetic mutation offers insights into potential targeted therapies and the molecular underpinnings of LCS.
Abstract:
Langerhans cell sarcoma is a rare malignancy with only 1 pediatric case (less than 15 y of age) reported. Here, we report the second case of Langerhans cell sarcoma in a child who presented with cord compression. This patient was treated with extensive surgical resection, postoperative chemotherapy, and involved-field radiation therapy. She completed therapy and remains in remission for 27 months. A review and analysis of all 53 cases published in the world literature is provided to help guide physicians treating this disease. Recently discovered genetic mutation involving BRAF is also discussed.
