The first case of intracranial rosai dorfman disease.
Spaulding J, Saraf S, Essad K, and Christianson M. The first case of intracranial rosai dorfman disease. Neurology 2016; 86(16)
Objective: to report an interesting and informative case of an atypical presentation of a rare condition, Rosai Dorfman Disease (RDD) Background: RDD previously known as Sinus Histiocytosis with massive lymphadenopathy (SHML), is a rare, idiopathic disease seen mainly in children and young adults. It typically presents with painless lymphadenopathy, fever, leukocytosis, elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia. To date only 5 cases of isolated epibulbar disease have been described in the literature. Case, Methods: An 80 year-old African-American woman presented with a history of a right orbital mass and complaints of worsening blurred vision and photophobia in the left eye. Her vision was NLP in the right and 20/60 in the left eye. She had a large, pink lesions under the superior bulbar conjunctiva of both eyes with concomitant cell and flare. MRI orbits, biopsies of right subconjunctival lesion and both retrobulbar lesions. Pathology showed histiocytes with abundant atypical reticular cytoplasm with phagocytosed lymphocytes. Immunohistochemical staining was S-100 positive, CD1a negative, and CD68 positive for histiocytes. Results: Enhancing soft tissue masses posterior to both globes, and masses in the cavernous sinus, cribriform plate, crista Galli, and interhemispheric fissure on either side of the falx, the left optic nerve sheath Conclusions: RDD is associated with massive cervical lymphadenopathy in 90[percnt] of patients, lymphadenopathy is also present inguinal (26[percnt]), axillary (24[percnt]), and mediastinal (15[percnt]). Up to 43[percnt] of patients presented with extranodal pathology, most frequently involving the skin, upper respiratory system, eyelid, orbit, bone, salivary glands, and the central nervous system. Our case is classified as RDD with orbital, uveal, epibulbar and intracranial involvement. The age of presentation is atypical, suggesting that RDD may masquerade as several entities and should be kept in the differential for patients with any combination of orbital masses, idiopathic uveitis, and subconjunctival lesions.