Extranodal sinonasal Rosai-Dorfman disease: a clinical study of 10 cases

Abstract

Sinonasal Rosai-Dorfman disease (S-RDD) is a rare form of RDD limited to the sinonasal cavity. Multipatient studies of Chinese S-RDD and documentation of its clinical spectrum are rare. This study aimed to identify the clinical profiles of Chinese S-RDD. Medical records of and tissue sections from 10 patients diagnosed with S-RDD between 2007 and 2014 were reviewed. Data on clinical presentations, endoscopy signs, imageological change, treatment and outcome were analyzed. The mean age of five male and five female patients at the first visit was 40.3 years and the mean follow-up period was 58.6 months. Based on the lesion sites, five cases were divided into an anterior sinonasal group, accompanied by symptoms of epistaxis, nasal obstruction and nasal dorsal deformity. Five other cases were divided into a posterior sinonasal group, accompanied by symptoms of hyposmia, epistaxis and nasal obstruction. Endoscopy signs and imageological changes in the anterior group showed diffuse infiltration of the RDD lesion under the septum mucosa, but in the posterior group the RDD lesions often showed as formations on polyps. At the end of follow-up, only one case spontaneously resolved without surgery; two cases in the anterior sinonasal group and three cases in the posterior sinonasal group recurred after endoscopic surgery, but surgery can result in short-term symptomatic control and restoration of function in all cases. S-RDD of the anterior and posterior sinonasal cavity may have different clinical characteristics; endoscopic surgery is effective for short-term symptomatic control and restoration of function for S-RDD.