Arndt, Carola S

Objectives/HypothesisEvaluate presentation, management, and clinical outcomes of patients with temporal bone Langerhans cell histiocytosis (LCH).Study DesignRetrospective chart review.MethodsReviewed all patients with temporal bone LCH at a tertiary academic referral center between 1978 and 2014. Presentation, disease course, intervention, and clinical outcomes were analyzed.ResultsBetween 1978 and 2014, 29 temporal bones in 20 patients (12 males; median age 32 years, range 1.3–88 years) were diagnosed with temporal bone LCH, representing 4% of all patients diagnosed with LCH at our institution during that time. Twelve (60%) patients presented purely with head and neck disease, nine (45%) with bilateral temporal bone involvement, seven (35%) with intracranial spread, and eight (40%) with multisystem disease. The most common presenting symptoms were otorrhea (n = 11; 55%) and subjective hearing loss (n = 10; 50%). Treatment included primary chemotherapy or radiation alone(n = 7; 35%), local resection alone (n = 2; 10%), and chemotherapy or radiation with surgery(n = 11; 55%). Ten patients suffered local or systemic relapse (50%; median 12 months). Median follow‐up for patients was 31 months. One patient died secondary to pulmonary complications of multisystem LCH.ConclusionsLCH is an uncommon histiocytic disorder with a range of clinical manifestations and disease severity. Otologic involvement is rare, and frequently manifests with symptoms similar to otomastoiditis. Evaluation and close follow‐up of both ears is important. Bilateral simultaneous or sequential LCH can be seen in up to 45% of cases. Patients with temporal bone LCH should be managed by a multidisciplinary team with treatment tailored to the patient. Relapse is more common in patients with multisystem involvement. Even in subjects with isolated otologic involvement, long‐term follow‐up is critical, with relapses occurring years beyond treatment.Level of Evidence4. Laryngoscope, 126:1899–1904, 2016