ISOLATED EXTRANODAL INTRACRANIAL SINUS HISTIOCYTOSIS IN A 5-YEAR-OLD BOY - CASE-REPORT

Sinus histiocytosis with massive lymphadenopathy was first described i n 1969 by Rosai and Dorfman. The typical clinical characteristics of t his disease include painless cervical lymphadenopathy, fever, and weig ht loss. The condition can present with an extranodal mass in about 25 % of patients, and isolated masses without lymph node involvement occu r rarely. The authors describe a 5-year-old boy with cavernous sinus s yndrome due to an isolated extranodal form of sinus histiocytosis with massive lymphadenopathy in the temporal fossa. Several cases of this disease involving the central nervous system are reviewed. The histopa thological and magnetic resonance imaging characteristics are discusse d.