Intracranial Rosai-Dorfman Disease Involving the Cavernous Sinus: A Case Report and Review of the Literature

Abstract

Background: Rosai-Dorfman disease (RDD), also called sinus histiocytosis with massive lymphadenopathy, is an idiopathic, non-neoplastic, lymphoproliferative disorder. Histologically, the disease is characterized by emperipolesis and S-100 immunoreactivity. RDD usually presents with massive painless cervical lymphadenopathy, with only 5% of cases affecting the central nervous system.

Case description: We report the case of a 46-year-old woman with isolated RDD of the left cavernous sinus with left facial numbness and weakness of the left masticatory muscle. The patient was successfully treated by total resection without recurrence found at the 65-month follow-up examination.

Conclusions: Central nervous system RDD is a diagnostic challenge preoperatively depending on the radiologic findings. Immunohistochemical confirmation is indispensable for the definite diagnosis. Total resection remains the most effective treatment to date. Adjuvant treatment such as chemotherapy, radiotherapy, and steroids can be administrated in cases of incomplete resection, recurrence, or multiple foci.

Keywords: Cavernous sinus; Central nervous system; Rosai-Dorfman disease; Sinus histiocytosis with massive lymphadenopathy; Treatment.