(887) Temporal Bone Histiocytosis in Children: Patterns of Presentation and Management

Introduction: Langerhans histiocytosis (LH) of the temporal bone is a rare clonal histiocytic disorder that often presents with otologic symptoms that mimic more common pediatric ear pathology. This scoping review aims to better understand presentation patterns, diagnostic features, and management approaches to guide otolaryngologic care for these patients.

Methods: PubMed and MEDLINE were search following PRISMA guidelines for English language studies published between 2003 and 2025 describing temporal bone LH in children. Two independent reviewers screened 255 articles identified through database and manual reference searches, yielding 12 studies for qualitative synthesis. Extracted data included patient demographics, presenting symptoms, temporal bone subsite involvement, imaging findings, treatment modality, and reported outcomes. Results are presented using descriptive statistics.

Results: Twelve studies comprising 199 pediatric patients met inclusion criteria. Patients had a mean age of 5.3 years and were 56% male. The most common presenting features were postauricular mass (67%), otorrhea (24%), and otalgia (9%), with less frequent symptoms including hearing loss, cervical lymphadenopathy, bleeding from the external auditory canal, aural fullness, and balance disturbances. Imaging most often demonstrated lytic or destructive temporal bone lesions, most commonly involving the mastoid. Management varied across cohorts, with chemotherapy most frequently employed, while surgical resection, radiotherapy, or multimodal therapy were used selectively. Reported recurrence rates ranged from approximately 10% to 40%, though heterogeneity in study design and follow-up precluded pooled analysis.

Conclusions: Pediatric temporal bone LH is a rare but clinically significant disorder with heterogeneous presentation and management, often mimicking more common otologic pathology. Early recognition of its characteristic clinical and radiographic features is essential, as treatment strategies, including the role of surgery, remain unclear.