Isolated intracranial Rosai-Dorfman disease with orbital extension.

摘要

Sinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease (RDD) is a rare non-neoplastic histiocytic disorder that was first described in 1969 by Rosai and Dorfman. This lymphoproliferative disorder of uncertain etiology that affects mainly children and young adults is characterized clinically by massive, painless cervical lymphadenopathy, fever, leukocytosis, elevated erythrocyte sedimentation rate and polyclonal hypergammaglobulinemia.2 Histopathologically, the disease is characterized by lymph node sinus dilation with emperipolesis, a distinctive feature whereby histiocytes phagocytize intact lymphocytes.3 Most patients with RDD present with nodal involvement, while a minority of patients (40%) have extranodal involvement.4 Interestingly, the disease can affect any system with a propensity for tissues of the head and neck, with nasal and oral cavities and paranasal cavities being affected in 10% to 20% of cases.