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
Pathology Quiz Case 2: Diagnosis. JAMA Otolaryngol Head Neck Surg. 2013;139(5):530–531. doi:10.1001/jamaoto.2013.2864b
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