Orbital langerhans cell histiocytosis in adults

Abstract

<h4>Purpose</h4>To report 3 patients with adult-onset orbital Langerhans cell histiocytosis (LCH).<h4>Design</h4>Retrospective interventional case reports.<h4>Participants</h4>Three adult patients with orbital LCH.<h4>Methods</h4>Case reports from 2 different clinics and review of the scientific literature.<h4>Main outcome measures</h4>Clinical course and radiologic and histologic findings.<h4>Results</h4>Three healthy patients, aged 20 to 36 (mean, 27) years, presented with biopsy-proven unilateral orbital LCH. The initial presenting complaints were mainly orbital pain and headache with or without orbital swelling and minimal effect on ocular movements. There was radiologic evidence of disease centered on the greater wing of sphenoid bone in all cases. Biopsy via lateral canthotomy was performed with LCH confirmed based on histologic and immunohistochemical findings. There was no evidence of disease elsewhere on systemic investigations. One patient had curettage and intralesional corticosteroids, 1 patient had a craniotomy for more complete resection of the lesion, and the third patient was referred for radiotherapy. All patients achieved complete resolution of symptoms and signs. None of the patients demonstrated any evidence of distant or recurrent disease at follow-up.<h4>Conclusions</h4>Orbital LCH in adults is a rare, usually unifocal, and relatively benign disease most commonly affecting the greater wing of sphenoid bone, where there is still active bone marrow in young adults. There is no consensus on cause or therapy, but our reported cases show that several treatment modalities are effective.