Neurological Presentations and CNS Pathology of Rosai-Dorfman disease: A 28-Year Report and Review from Single Tertiary Referral Institute in Thailand
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Abstract
Rosai-Dorfman Disease (RDD) is a benign systemic histio-proliferative disorder with a variable clinical presentation, depending on the sites of the lesion. Intracranial RDD is a form of sporadic extranodal type of RDD and usually occurs without lymphadenopathy. Most intracranial lesions are attached to the dura mimic meningioma; therefore, pathology is necessary to diagnose this rare entity. Pathology of RDD is characterized by numerous large histiocytic cells engulf the intact lymphocytes (Emperipolesis). Immunohistochemical stains of histiocytic cells are positive for CD68, S-100 protein but negative for CD1a. Surgery is the first line of treatment and post-operative corticosteroids or chemotherapy may be recommended in some cases. This article describes and summarizes the four cases of intracranial RDD encountered by the Neurological Institute of Thailand from 1992 to 2019 and highlights the clinical presentations, pathology, plan of treatments and outcome of disease in each occurrence.
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