Langerhans cell histiocytosis of the orbit: A study of eight cases

BACKGROUND: Langerhans cell histiocytosis (LCH) of the orbit is a rare clinical entity with a diagnostic and therapeutic dilemma. MATERIALS AND METHODS: This was a retrospective study of eight patients with orbital LCH. RESULTS: All eight patients in our series were male, and the mean age at presentation was 8 years (median 6 years; range, 7 months–23 years). All of them had unilateral disease, and the most common presenting complaint was upper eyelid swelling (n = 6). The mean duration of symptoms was 6 weeks (median, 3 weeks; range, 2–20 weeks). Visual acuity was unaffected in seven cases. Clinical diagnosis included rhabdomyosarcoma (n = 4), malignant lacrimal gland tumor (n = 2), orbital cysticercosis (n = 1), and orbital tuberculosis (n = 1). The diagnosis of orbital LCH was confirmed by incisional biopsy (n = 7) or fine-needle aspiration cytology (n = 1). Four cases underwent careful limited curettage and received intralesional steroid, and four cases were treated with intralesional steroid alone after incisional biopsy. Complete tumor resolution was achieved in seven cases after receiving a mean of one intralesional steroid injection (median, 1; range, 1–2), while one patient was advised systemic chemotherapy for residual tumor. No tumor recurrence was noted in any case at a mean follow-up duration of 30 months (median, 23 months; range, 7–96 months). None of the cases developed diabetes insipidus or multisystem disease during the follow-up period. CONCLUSION: Minimal local intervention with intralesional steroids with/without careful curettage achieves complete tumor resolution in unifocal orbital LCH.