Orbital Involvement in Langerhans Cell Histiocytosis

Purpose: The authors studied a series of patients with Langerhans cell histiocytosis in an attempt to better define the incidence and significance of orbital involvement within the vast spectrum of the disease. Methods: Retrospective chart review of consecutive patients with a recorded diagnosis of Langerhans cell histiocytosis treated at St. Jude Children's Research Hospital between 1992 and 2007. Pertinent information included age, gender, clinical features, radiological features, treatment, disease progression, orbital involvement, and development of diabetes insipidus. Results: The authors found and evaluated 24 patients (16 male). The median age at diagnosis was 24 months (range, 4-179 months), and median follow-up was 75 months (range, 6-186 months). Nine (37.5%) patients had orbital involvement (6 on presentation, 3 on subsequent follow-up). The 2 patients with unifocal orbital lesions developed progressive disease. All patients with orbital lesions received systemic chemotherapy either at the time of diagnosis (n = 8) or on documented disease progression (n = 1). Six patients (25%), 2 of whom had orbital involvement, developed diabetes insipidus in the setting of either multibone (n = 1) or multisystem (n = 5) disease. Conclusions: In this study, orbital involvement occurred in one third of patients with Langerhans cell histiocytosis, usually in the context of multifiocal bone or multisystem disease. Thus, the authors believe a comprehensive workup and follow-up in the context of a multidisciplinary approach is necessary. The authors found that the response to local curretage or steroid injection for small lesions, and to systemic chemotherapy for extensive lesions, is usually excellent and that aggressive local control measures, such as surgical resection or radiation, are not indicated in most cases.