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

• Published in: The Laryngoscope 2016-08, Vol.126 (8), p.1899-1904
• Main Authors: Modest, Mara C., Garcia, Joaquin J., Arndt, Carola S., Carlson, Matthew L.
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Bone Diseases - diagnosis; Bone Diseases - therapy; Child; Child, Preschool; ear; eosinophilic granuloma; Female; head and neck; Histiocytosis, Langerhans-Cell - diagnosis; Histiocytosis, Langerhans-Cell - therapy; Humans; Infant; Langerhans cell histiocytosis; Male; Middle Aged; Recurrence; Retrospective Studies; skull base; Temporal Bone; Young Adult
• ISSN: 0023-852X; 1531-4995
• DOI: 10.1002/lary.25773

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