Langerhans cell histiocytosis of the temporal bone: A review of 29 cases at a single center

OBJECTIVES/HYPOTHESIS: Evaluate presentation, management, and clinical outcomes of patients with temporal bone Langerhans cell histiocytosis (LCH). STUDY DESIGN: Retrospective chart review. METHODS: Reviewed 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. RESULTS: Between 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. CONCLUSIONS: LCH 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 EVIDENCE: 4. Laryngoscope, 126:1899-1904, 2016.