Partial Removal of Orbital Tumor in Rosai-Dorfman Disease

Background: Rosai-Dorfman disease is a rare idiopathic histiocytic proliferation disorder that typically presents with painless cervical lymphadenopathy. We report our experience with the management of a case of Rosai-Dorfman disease with compressive optic neuropathy. Case: Rosai-Dorfman disease involving the bilateral orbital and paranasal sinuses was diagnosed in a 14-year-old boy. Diagnosis was based on the characteristic histopathologic features of sinus histiocytosis, composed of large, round S-100 protein-positive histiocytes with striking emperipolesis. The boy received chemotherapy to resolve the bilateral proptosis and compressive optic neuropathy in the right eye, but this treatment failed. Orbital debulking surgery using the Lynch approach was performed. Observations: Corneal exposure was resolved and visual acuity recovered from 14/20 to 20/20 after partial removal of the tumor mass. There were no complications after surgery. During the 22 months of follow-up, orbital tumor masses redeveloped to cause lagophthalmos again, but did not cause visual impairment. Conclusions: Rosai-Dorfman disease is a rare disorder, especially in Asia. The disease is usually chronic with spontaneous remission and is refractory to treatment. Partial removal of tumor masses is a workable way to improve visual acuity and correct corneal exposure. Before carrying out this procedure, we discussed with the parents of the patient the potential complications that might follow surgery and secured their permission before proceeding further.