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.


METHODS
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.


METHODS
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.
Wu
Shu-Ya
SY
Department of Ophthalmology, Chang Gung Memorial Hospital, Taiwan.
Ma
Lih
L
Tsai
Yueh-Ju
YJ
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003131:Combined Modality Therapy; D004724:Endoscopy; D015618:Histiocytosis, Sinus / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D009916:Orbital Diseases / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D010254:Paranasal Sinus Diseases / Q000378:metabolism / Q000473:pathology / Q000601:surgery*; D012008:Recurrence; D009418:S100 Proteins / Q000378:metabolism; D014057:Tomography, X-Ray Computed
D009418:S100 Proteins