Langerhans cell histiocytosis of the orbit.

PURPOSE: The management of Langerhans cell histiocytosis is controversial. This study evaluated our clinical experience and therapeutic results in orbital Langerhans cell histiocytosis.

METHODS

This is a retrospective, noncomparative interventional case series involving 17 consecutive patients with biopsy-proven orbital Langerhans cell histiocytosis. Response to surgery and chemotherapy and development of diabetes insipidus were the main outcome measures.

RESULTS

Thirteen (76.5%) of the patients were male and the mean age at diagnosis was 10.7 years (range 2-39 years). The most frequent presenting sign was proptosis (8 patients) and upper eyelid edema (4 patients). Pain was present in 5 cases and periocular redness in 6. No patient reported a history of trauma. The frontal bone was involved in 16 patients followed by the zygomatic in 9 cases. Five patients also had lesions in the calvarium, femur, facial, temporal, and parietal bones. No patients had systemic disease. Ten patients were managed with vinblastine (0.2 mg/kg, 6-12 months) chemotherapy because of major residual tumor burden (5 cases) and multi-bone involvement (5 cases). Four patients were observed following macroscopically complete tumor removal. Three patients with limited anterior orbital soft tissue tumors and single bone involvement received systemic corticosteroids (40 mg/m2/d, 6-10 weeks). No patients developed diabetes insipidus after a median follow-up of 46 months.

CONCLUSIONS

Macroscopically complete excision of the unifocal tumors may not necessitate any further treatment. Vinblastine chemotherapy following incomplete tumor removal and in patients with multifocal bone disease resulted in recurrence-free survival at 3 years in 90% of patients.