Primary isolated intracranial Rosai–Dorfman disease: Report of a rare case and review of the literature

Abstract

BackgroundIntracranial involvement is an uncommon manifestation of Rosai–Dorfman disease (RDD) and had been rarely reported. In this study, we explore clinical characteristics, imageology manifestations and pathological features of primary intracranial RDD so as to improve the understanding for this disease. MethodsOne case (16-years-old boy) with primary intracranial RDD was analyzed and studied retrospectively by MRI features, histopathological observation and immunohistochemical staining, and the related literatures were reviewed. ResultsThe case was single lesion and involved the dura of the left middle cranial fossa base, which was iso-hypo signal intensity on T1WI and hypointense on T2WI and FLAIR image. The lesion was a homogeneous contrast enhancement mass with dural tail sign and had peritumoral brain edema. Pathological analysis showed the lesion consisted of variable numbers of mature lymphocytes, plasma cells and neutrophils. The characteristic histiocytes were emperipolesis and positively expressed for S-100 and CD-68 and negatively expressed for CD-1a by immunohistochemical analysis. Based on clinical presentations and histological findings after surgical excision, a final diagnosis of primary intracranial RDD was made. ConclusionPrimary intracranial RDD, especially located in the cranial base, is exceptionally rare, which hard to be distinguished with meningoma by imageology and clinical manifestations, but could be diagnosed by pathological and immunohistochemical examinations. Surgery is of the most importance treatment and prognosis is optimistic for this disease.