Korean J Pathol.  1999 Dec;33(12):1203-1206.

Rosai-Dorfman Disease of the Nose and Salivary Gland: A case report

• Roh MS
• Jeong JS
• Hong SH

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.

Keyword

Rosai-Dorfman disease; Nose; Salivary gland; Inflammatory pseudotumor

MeSH Terms

Cytoplasm
Female
Fibrosis
Granuloma, Plasma Cell
Histiocytosis
Histiocytosis, Sinus*
Humans
Lymph Nodes
Lymphocytes
Macrophages
Nasal Cavity
Nasal Obstruction
Neutrophils
Nose*
Phagocytosis
Plasma Cells
S100 Proteins
Salivary Glands*
Submandibular Gland
Young Adult
S100 Proteins