Abstract
Rosai-Dorfman disease (RDD) is a rare, benign pseudolymphatous condition, predominantly
involving lymph nodes. Although several cases of extra-nodal involvement have been
reported previously, central nervous system involvement, particularly in the absence
of nodal disease is extremely rare. Extranodal large RDD presenting as a single lesion
involving sino-orbital and anterior cranial fossa has rarely been described previously.
We report a case of incisional biopsy proved RDD in a young lady who presented with
nasal obstruction and subsequent proptosis with visual diminution. Radiography of
head and paranasal sinus demonstrated a strongly enhanced, diffuse polypoid lesion
filling the bilateral sinonasal cavity and orbit with extension to the anterior cranial
fossa by way of splaying the bony foramina. Pre-operative low dose steroid therapy
had resulted in decreased size of the mass which facilitate gross-total surgical resection.
RDD was confirmed by histopathology (emperipolesis) and immuno-histochemistry (S-100
positivity). The follow-up computed tomography 3 months later showed minimal tumor
residue in left parasellar region with complete sinonasal decompression.
Key words
Emperipolesis - extranodal - intracranial - Rosai-Dorfman disease - sino-orbital
