Extranodal Rosai-Dorfman Disease of the Nasal Septum: A Case Report

Abstract

Rosai-Dorfman Disease (RDD) is a rare, benign histiocytic proliferative disorder mostly involving the lymph nodes. Extranodal disease can occur in the skin and soft tissue including the mucosal surface, central nervous system, gastrointestinal tract, and breast. Here, a case of a 17-year-old Indo-asian girl is presented, who came to the Ear Nose Throat (ENT) Outpatient Department (OPD) with a mass in her left nostril for the past two years. A sessile globular mass from the left nasal septum was seen during diagnostic visual endoscopy. A Computerised Tomography (CT) scan of the paranasal sinus revealed a rather large right maxillary sinus with a soft tissue density lesion filling it and obliterating the osteomeatal complex, while blood tests revealed an increased eosinophilic count. The nasal tumour was removed, and its histological analysis revealed characteristics of RDD. This is typically sporadic histiocytosis with emperipolesis in the cells. Clinically, the condition is benign, and all that is required is conservative therapy and periodic monitoring of its progression. Surgical excision is rarely advised until the disease has grown to an unmanageable size and is causing respiratory impairment or a cosmetic deformity. Currently, therapy revolves around surgical and immunosuppressive treatments, but the optimal diagnostic and therapeutic management of RDD remains to be defined.