Sinonasal Rosai-Dorfman disease

Abstract

Aim: Rosai-dorfman disease (RDD) is a rare histiocytic proliferative disorder characterized by painless massive lymphadenopathy. Extranodal manifestations may occur in the head and neck area. This is a case report of a patient with sinonasal RDD and its management. Materials and methods: This is a case report of a 36-yearold patient with sinonasal RDD. The clinical features, imaging and histological features are described. The patient underwent endoscopic surgery. Recurrence (8 months later) was managed by laserization of the nasal mass with KTP/532 laser followed by oral corticosteroid therapy. Results: A 36-year-old lady presented with complaints of left sided nasal obstruction, nasal discharge and left facial discomfort of 6 months duration. There was history of occasional blood stained nasal discharge. Diagnostic nasal endoscopy revealed a polypoidal mass in the left nasal cavity. CT scans revealed soft tissue opacity in the left maxillary antrum, left ostiomeatal complex and extending into the left orbit. There was erosion of the posterolateral wall of the left maxilla. The patient underwent endoscopic surgery and had an uneventful postoperative recovery. Biopsy was reported as Rosai-Dorfman disease. Subsequently, 8 months later, the patient reported with a complaint of nasal obstruction and discharge. A recurrent lesion was noted in the left nasal cavity and maxillary antrum. The mass was managed by laserization followed by oral corticosteroid therapy. One year later, the patient is free from disease and has been advised regarding the necessity of periodic follow-ups. Conclusion: RDD is a rare disease encountered by the otolaryngologist. The manifestation of extranodal Rosai- Dorfman disease is most commonly seen in the nasal cavity and paranasal sinuses. The clinical findings and imaging characteristics are variable and the diagnosis can be easily missed. Otolaryngologists and pathologists must be aware of the clinical presentation, imaging characteristics and histologic features of Rosai-Dorfman disease.