Extranodal Rosai-Dorfman Disease with Paranasal Sinuses and Intracranial Involvement: a case report

Reprint requests to: Dr. Shu-Hang Ng Department of Medical Imaging and Intervention, Chang Gung Memorial Hospital, Linkou and College of Medicine, Chang Gung University. No. 5, Fu Hsing Road, Gueishan , Taoyuan 333, Taiwan, R.O.C. Rosa i Dor fman d i sease ( R DD) i s 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 obstruct ion and subsequent headache. Pre-enhanced sinus CT showed dif fuse polypoid masses in the nasal cavity without bony destruction. MRI demonstrated a strongly enhanced, infiltrative lesion f ill ing the sinonasal cavity. The patient underwent surgical resection, and histopathology showed emperipolesis (lymphophagocytosis) and immunohistochemistry 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. Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder characterized by sinus hist iocy tosis and massive lymphadenopathy. It typically causes massive, painless cervical lymphadenopathy in children and young adults. Although lymph node involvement occurs most frequently, isolated extra-nodal form presents in 25% to 40% of cases; most commonly the skin, head and neck, paranasal sinus, orbit, upper respiratory tract, bone, testis, and rarely central nervous system (CNS) [1]. CNS involvement is uncommon and accounts for less than 5% of cases, with three fourths in the brain and the other one fourth in the spine [2]. Coexisting sinonasal and intracranial RDD is even rarer and only 4 cases have been reported [3-6]. Three of the reported cases presented as multi-focal involvement of the paranasal sinuses, intracranial lesion, spinal lesion and skin. All of the reported cases had synchronous sinonasal and int racranial lesions. Herein, we reported a case with metachronous sinonasal and intracranial RDD with MRI demonstration.