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Clinical Problem Solving: Pathology
April 2006

Pathology Quiz Case: Diagnosis

Author Affiliations


Arch Otolaryngol Head Neck Surg. 2006;132(4):456. doi:10.1001/archotol.132.4.456

Rosai-Dorfman disease is a rare benign disorder characterized by massive lymphadenopathy and associated with fever and leukocytosis.1 It was originally described in the late 1960s by Rosai and Dorfman,2 and since its discovery more than 650 cases have been reported.3 Lymphadenopathy, typically cervical, is the most common presentation of this entity and is seen in more than 95% of patients; however, extranodal involvement occurs in approximately one third of patients.4 Examples of nonlymphoid sites of involvement include the head and neck, paranasal sinuses, eyes, central nervous system, kidneys, upper respiratory tract, gastrointestinal tract, testicles, temporal bone, and salivary glands.

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