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Clinical Problem Solving: Pathology
May 2013

Pathology Quiz Case 2: Diagnosis

JAMA Otolaryngol Head Neck Surg. 2013;139(5):530-531. doi:10.1001/jamaoto.2013.2864b

Diagnosis: Extranodal Rosai-Dorfman disease (RDD) of the maxillary sinus

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare clinicopathologic entity characterized by an accumulation of distinctive proliferating histiocytes within lymph node sinuses and lymphatics.1 This idiopathic and benign histiocytic disorder was first described by Rosai and Dorfman in 1969.2 It most commonly presents as bilateral, painless, massive cervical lymphadenopathy in children and adolescents, although any age group may be affected.3 The lymphadenopathy may be accompanied by a viral-like prodrome with fever, malaise, night sweats, elevated erythrocyte sedimentation rate, polyclonal hypergammaglobulinemia, and leukocytosis.4