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.

Rosai–Dorfman‐type histiocytes occur in eosinophilic chronic rhinosinusitis and potentially play a role in disease initiation and persistence

“…Isolated sinonasal RDD poses a diagnostic challenge in the absence of accompanying lymphadenopathy. At presentation, symptoms of sinonasal RDD include nasal obstruction, epistaxis, hyposmia or anosmia, nasal and facial deformity . The average age of presentation is 40 years, with a slight male predominance .…”

“…At presentation, symptoms of sinonasal RDD include nasal obstruction, epistaxis, hyposmia or anosmia, nasal and facial deformity . The average age of presentation is 40 years, with a slight male predominance . Polypoid lesions of SN‐RDD are similar to nasal polyps of CRS, but while the latter polyps are often located in the middle nasal meatus or olfactory cleft, polypoid lesions in SN‐RDD are more often bilateral sinonasal.…”

“…Polypoid lesions of SN‐RDD are similar to nasal polyps of CRS, but while the latter polyps are often located in the middle nasal meatus or olfactory cleft, polypoid lesions in SN‐RDD are more often bilateral sinonasal. Although diagnosis is based on histology, clinical presentation may aid in diagnosis even in the absence of accompanying cervical lymphadenopathy . Histologically, cases are characterized by RDH and background lymphocytes and plasma cells, with emperipolesis of these and of red cells.…”

“…RDD‐type histiocytes (RDH) are characterized by S100 protein expression and they lack CD1a . Sinonasal RDD displays RDH within an inflammatory background, which typically includes lymphocytes and plasma cells . Chronic rhinosinusitis (CRS) is common, with an incidence of between 2 and 14%, and may present with and without nasal and/or sinus polyposis.…”

Hematolymphoid lesions of the sinonasal tract

“…48,49 Most patients have evidence of RDD in other sites (Figure 7; A,B), although disease limited to the sinonasal cavity has also been described (Figure 7; C,D). 48-50 Patients with RDD may be treated with endoscopic surgery for symptomatic control, and in many cases the disease is benign and self-limiting, particularly if the lesion can be excised in its entirety. 49 Histologically, this disorder is characterized by S100-positive macrophages that demonstrate emperipolesis, the engulfment of intact lymphocytes.…”