Abstract

Rosai-Dorfman disease (RDD) is a rare type of benign histiocytosis characterized histologically by intracellular engulfment of lymphocytes. Extranodal RDD may occur as a part of generalized process involving lymph nodes or may involve extranodal sites independent of the lymph node status. We have experienced a case of extranodal Rosai-Dorfman disease of the nose as an initial lesion prior to nodal involvement. The patient was a 20-year-old woman who complained of nasal obstruction for 4 years, remotely, and left submandibular mass for 3 months, recently. Histologically, the lesion taken from nasal cavity, submandibular gland and left upper jugular lymph node all showed an heavy infiltrate consisted of plasma cells, lymphocytes and sheets of macrophages with abundant pale cytoplasm, which replaced organ architecture. The associated focal fibrosis made it difficult to differentiate from inflammatory pseudotumor. Some macrophages demonstrated phagocytosis of lymphocytes, plasma cells and occasionally neutrophils. The macrophages were strongly positive for S-100 protein.

• Keywords: Rosai-Dorfman disease;Nose;Salivary gland;Inflammatory pseudotumor