Pediatric Langerhans cell histiocytosis of the lateral skull base

Objective Describe the presentation, imaging characteristics, management, and outcomes of pediatric patients with Langerhans cell histiocytosis (LCH) of the temporal bone. Methods A retrospective chart review was performed between 2000 and 2014 at a single tertiary care children's hospital. Fourteen patients were identified with a diagnosis of LCH and involvement of the temporal bone. Results Ten patients were female and ten were Caucasian. Mean age at diagnosis was 3 years (range 0.3–9.6 years). The most common presenting symptoms were scalp lesions, postauricular lesions, otalgia, and persistent ear infections. Three patients had documented hearing loss. Four cases had otic capsule invasion. Computed tomography demonstrated a lytic temporal bone lesion within the following subsites: mastoid, squamous temporal bone, external auditory canal, middle ear, and petrous apex. Four patients had intracranial disease. Extent and location of disease prompted all patients to be initially managed with chemotherapy. Surgical excision was limited to one case of localized recurrence. After a mean follow-up of 85.2 months (SD 42.4 months) there were no deaths but eight patients had a recurrence. Two patients developed long-term otologic sequelae requiring surgery. One patient developed labyrinthitis ossificans. Conclusion LCH has a varied presentation, age distribution, and treatment algorithm. This series of 14 pediatric patients with temporal bone involvement supports a limited role for surgical resection. Long-term follow-up is critical for detection of local and disseminated disease, and for monitoring of otologic complications.