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Head and Neck Langerhans Cell Histiocytosis in Children
Joyce Xu1, Julie R Gilbert2, Kathryn S Sutton3
1US/CA OMS resident; Resident in Training, Oral and Maxillofacial Surgery, Department of Surgery, Emory University School of Medicine, Atlanta, Georgia.
Insights
Management of Langerhan's Cell Histiocytosis (LCH) in children varies by presentation. Single-system LCH without CNS risk may be treated with surgery, while single-system with CNS risk and multisystem LCH require chemotherapy.
Area of Science:
- Pediatric Oncology
- Head and Neck Surgery
- Histiocytosis Research
Background:
- Langerhan's Cell Histiocytosis (LCH) management presents challenges in head and neck surgical specialties.
- Evaluating diagnosis, treatment, and outcomes in pediatric head and neck LCH is crucial.
Purpose of the Study:
- To evaluate the diagnosis, management, and treatment outcomes of pediatric head and neck Langerhan's Cell Histiocytosis.
- To analyze different presentations and their impact on LCH treatment strategies.
Main Methods:
- Retrospective cohort study of pediatric patients with head and neck LCH (2009-2021).
- Analysis of demographic data, lesion location, clinical presentation, imaging, diagnostics, treatment, and follow-up.
- Patients grouped by LCH presentation: single-system without CNS risk (SS-), single-system with CNS risk (SS+), and multisystem involvement.
Main Results:
- Three LCH presentations identified: SS- (n=24, avg. age 10, calvaria/mandible, treated with debridement, 2 reactivations); SS+ (n=30, avg. age 6, temporal bone/orbit, treated with chemotherapy, 1 reactivation); Multisystem (n=13, avg. age 2, skin/lymphatic, treated with chemotherapy, 40% reactivation).
- SS- patients primarily underwent surgical debridement with low reactivation rates.
- SS+ and multisystem LCH patients, often younger, required chemotherapy and showed higher reactivation rates.
Conclusions:
- LCH treatment in children is presentation-dependent.
- Surgical debridement is effective for SS- head and neck LCH.
- Early diagnosis and chemotherapy are vital for SS+ and multisystem LCH to improve outcomes and reduce reactivation.
Purpose:
Controversy exists among head and neck surgical specialties regarding management of Langerhan's Cell Histiocytosis (LCH). The purpose of this study was to evaluate diagnosis, management, and treatment outcomes in children with LCH of the head and neck.
Methods:
This is a retrospective cohort study of children with LCH of the head and neck who presented to Children's Healthcare of Atlanta hospital from 2009 to 2021. The independent variables were demographic information, lesion locations, clinical presentation, radiographic findings, diagnostic workup, treatment, and length of follow-up. The patients were grouped based on these variables. The outcome variable was disease reactivation. Descriptive statistics were calculated.
Results:
There were 3 presentations of LCH of the head and neck. Group 1 presented as a lesion in 1 system without CNS risk (SS-). There were 24 patients with an average age of 10 years. Lesions were located in calvaria and/or mandible. Majority of the patients were treated with only debridement. Two of the patients experienced reactivation. Group 2 presented as a lesion in 1 system with CNS risk (SS+). There were 30 patients with an average age of 6 years. Common locations were temporal bone and/or orbit. These patients present with recurrent ear infections and ptosis. Majority of the patients were treated with chemotherapy (n = 28). One patient had disease reactivation. Group 3 presented with multisystem involvement. There were 13 patients with an average age of 2 years. LCH was found in skin and the lymphatic system. Imaging demonstrated extracranial organ involvement. All of them were treated with chemotherapy. There was 40% reactivation of LCH.
Conclusions:
Treatment of LCH depends on presentation. SS- subgroup can be adequately treated via surgical debridement. SS+ and multisystem groups benefit from an early disease diagnosis and require chemotherapy.
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