Extranodal Rosai Dorfman Disease of the Orbit

Abstract

 

Purpose:

 

Rosai Dorfman Disease (RDD) is a rare idiopathic clinical entity. Classic presentation is with cervical lymphadenopathy. Extranodal disease is uncommon, with only 8% of cases having orbital disease. We report the presentation, radiography, histology, & treatment of 7 patients with orbital RDD.

 

Methods:

 

Records of 7 patients with orbital RDD were reviewed.

 

Results:

 

4 males & 3 females had median age of 10 years (range 2-78). Presenting symptoms included pain, orbital mass, proptosis, decreased vision & decreased eye movements. 3 patients had bilateral orbital disease, while 4 had unilateral disease. 5 cases were extraconal, 1 was intraconal, & 1 was both intra & extraconal. 3 cases had lymphadenopathy, while 4 had extranodal disease, 3 of which had localized orbital disease. Diagnosis was confirmed by exam, orbital & systemic CT, & biopsy in all cases. Treatment included excision (3 cases); debulking (2 cases); IV & oral steroids (1 case); & chemoradiation, oral steroids, & debulking (1 case). At last follow up 3 patients were disease free, 3 had asymptomatic disease & 1 had residual ptosis.

 

Conclusions:

 

RDD can often be mistaken for a malignant process. Purely extranodal disease is rare (17%), with isolated orbital disease being exceedingly rare. This study is unique in that over half of the patients had isolated extranodal disease (3 of these 4 cases had localized disease only of the orbits), & 3 of the cases had bilateral orbital disease. Most cases had disease in the extraconal space as compared to prior reports stating the intraconal space to be the more common site of disease. Although there is no consensus on treatment, surgical excision should be attempted in symptomatic patients especially if the orbit represents a localized site of disease.  

 

Keywords: orbit • imaging/image analysis: clinical • pathology: human