Nasal Rosai-Dorfman disease with intracranial involvement: a case report.
Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a rare but distinctive entity. It was initially described as a nodal-based disease but later found to affect a variety of extranodal sites. The diagnosis of Rosai-Dorfman disease is based on the unique histologic changes that are characterized by proliferation of S-100 protein-positive histiocytes, the presence of emperipolesis, and a mixture of prominent lymphoplasmacytic infiltrates. The etiology of Rosai-Dorfman disease is unknown. It usually runs a benign clinic course and may resolve spontaneously, but cases running protracted courses have also been reported. We report an unusual case of recurrent extranodal Rosai-Dorfman disease in nasal and paranasal cavities with intracranial involvement surviving for more than 9 years.
Chang
Yu-Chien
YC
Department of Otolaryngology, China Medical College Hospital, Taichung, Taiwan.
Tsai
Ming-Hsui
MH
Chen
Chi-Long
CL
Tsai
Chon-Haw
CH
Lee
Alex Ying-Shyuan
AY
D001927:Brain Diseases / Q000473:pathology*; D015618:Histiocytosis, Sinus / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012008:Recurrence