Extranodal Rosai-Dorfman disease with paranasal sinuses and intracranial involvement: A case report

Rosai-Dorfman disease (RDD) is a rare, benign pseudolymphatous condition, predominantly involving lymph nodes. Extranodal RDD presenting as metachronous nasal and intracranial masses has not previously been described. Herein, we reported such a case of RDD in a 49 year-old female who presented with nasal obstruction and subsequent headache. Pre-enhanced sinus CT showed diffuse polypoid masses in the nasal cavity without bony destruction. MRI demonstrated a strongly enhanced, infiltrative lesion filling the sinonasal cavity. The patient underwent surgical resection, and histopathology showed emperipolesis (lymphophagocytosis) and immuno- histochemistry showed S-100 positivity. The follow-up MRI one year later showed sinonasal tumor recurrence. In addition, it revealed a newly developed intracranial dura-based mass that exhibited hypointense foci on T2-weighted imaging. Recognition of these imaging findings is helpful in suggesting the diagnosis of RDD even in the absence of lymphadenopathy.