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Primary head and neck Langerhans cell histiocytosis in children
Luke Buchmann1, Abbas Emami, Julie L Wei
1Department of Otolaryngology-Head and Neck Surgery, University of Kansas School of Medicine, Medical Center, 3901 Rainbow Boulevard, Kansas City, KS 66160, USA.
Insights
Langerhans' cell histiocytosis (LCH) frequently impacts the head and neck, often requiring chemotherapy. While outcomes are generally good, recurrence is common, necessitating vigilance in diagnosis and treatment.
Area of Science:
- Pediatric Oncology
- Otolaryngology
- Histiocytosis
Background:
- Langerhans' cell histiocytosis (LCH) is a rare disorder characterized by the abnormal proliferation of Langerhans' cells.
- Head and neck involvement is common in pediatric LCH, presenting complex diagnostic and therapeutic challenges.
Purpose of the Study:
- To review the experience of a tertiary children's hospital in managing primary head and neck Langerhans' cell histiocytosis.
- To analyze the diagnosis, treatment, and long-term outcomes of affected patients.
Main Methods:
- Retrospective study of patients diagnosed with LCH affecting head and neck sites.
- Data collected from January 1, 1986, to December 31, 2004, at Children's Mercy Hospital.
Main Results:
- Fourteen patients had primary head and neck LCH, representing 64% of head and neck cases.
- Head and neck lesions involved complex structures like the calvarium and skull base.
- Ten of 14 patients achieved disease-free status; however, 50% experienced recurrence.
Conclusions:
- LCH frequently affects the head and neck, demanding thorough evaluation and management.
- Primary chemotherapy is the preferred treatment for complex head and neck LCH lesions.
- Otolaryngologists must be knowledgeable about LCH presentation, work-up, and treatment due to its prevalence in the head and neck region.
Objective:
To evaluate the experience of a tertiary care children's hospital in the diagnosis, treatment, and long-term outcomes of patients with primary head and neck Langerhans' cell histiocytosis.
Study Design And Setting:
A retrospective study of patients who presented with Langerhans' cell histiocytosis that primarily affected head and neck sites between January 1, 1986, and December 31, 2004, at Children's Mercy Hospital.
Results:
Twenty-two patients were diagnosed and treated for LCH at our hospital during this time period. Seventeen (77%) patients had head and neck involvement; 14 (64%) of these patients had primary head and neck LCH. Lesions of the head and neck are complicated and involve multiple structures including the calvarium and skull base. Overall outcomes were good with 10 of 14 patients without disease at time of last follow-up. Recurrence is common and involved 50% of our patients.
Conclusions:
Langerhans' cell histiocytosis commonly affects the head and neck. These lesions are complex and require prudent evaluation and treatment. Due to the complex nature of head and neck lesions, primary chemotherapy is the treatment of choice.
Significance:
Because of the frequent head and neck involvement of this disease, otolaryngologists need to be familiar with its presentation, work-up, and treatment.
Ebm Rating:
C-4.