2026/02/25 by Mostafa Alwan, Ha My Nguyen, Eric Levi
Biochemistry, Genetics and Molecular Biology · Health Professions · Medicine · #Genetic and rare skin diseases. #Histiocytic Disorders and Treatments #Oral and Craniofacial Lesions
paper · doi:10.1017/s0022215126104514
crossref issued 2026/02/25 · crossref published 2026/02/25 · crossref published-online 2026/02/25 · openalex created_date 2026/02/25 · openalex publication_date 2026/02/25 · crossref created 2026/02/25 · crossref published-print 2026/05/01 · crossref deposited 2026/06/30 · openalex updated_date 2026/07/14 · crossref indexed 2026/07/30
BACKGROUND: Langerhans cell histiocytosis is a rare disorder in children that can involve the head and neck, may mimic common ENT conditions and delay diagnosis. While various case reports exist, ENT-focused reviews remain limited. METHODS: We conducted a literature review of 73 studies encompassing 244 paediatric patients with head and neck Langerhans cell histiocytosis, extracting data on presentation, treatment and outcomes. We also present a retrospective case series of 25 patients treated at a single tertiary institution. RESULTS: Otologic and craniofacial bone involvement were the most common presentations. Treatments ranged from surgical curettage and intra-lesional steroids to systemic chemotherapy. Recurrence was more common in multifocal and multisystem disease. Our institutional series supported many of these findings, with detailed follow-up data and treatment responses. CONCLUSION: This is the most extensive ENT-focused review of paediatric Langerhans cell histiocytosis to date. It highlights key clinical patterns and management approaches to aid early recognition and tailored treatment.