Intracranial Rosai–Dorfman disease: Case report and literature review
ABSTRACT Rosai–Dorfman disease (RDD) is an idiopathic, non-neoplastic, lympho-histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy. Intracranial RDD is mainly a disease characterized by dura-based masses and/or diffuse meningeal thickening and included a broad differential diagnosis radiologically. The authors report an unusual case of a 38-year-old male patient presenting with bilateral multiple intracranial dura-based nodules and diffuse meningeal thickening as well as intra-spinal canal involvement. The MR imaging and histo-pathological features of the disease are discussed. Histology with immunohistochemical analysis, in which it demonstrates emperipolesis (lymphophagocytosis) feature and that most of the histiocytes stained with strong positivity for S100 and CD68 proteins, is essential for a definitive diagnosis.
Archives of pathology 02/1969; 87(1):63-70.
American Journal of Ophthalmology 03/2007; 143(3):544. DOI:10.1016/j.ajo.2007.01.010 · 4.02 Impact Factor
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ABSTRACT: Rosai-Dorfman disease (RDD) is a rare idiopathic histoproliferative disease affecting the systemic lymph nodes. Although an extranodal lesion has also been recognized, central nervous system involvement is extremely rare. To the authors' knowledge, only 20 cases of intracranial lesions have been reported previously. Intracranial RDD is clinically and radiologically difficult to distinguish from meningioma, and histological examination is essential for a definitive diagnosis. The authors treated a large frontal lobe tumor associated with multiple meningeal nodules in a 67-year-old patient presenting with diplopia and headache. Radiological examination indicated that the mass was an inflammatory lesion rather than a meningioma. Microscopically the lesion consisted of mixed inflammatory infiltrate with marked emperipolesis, which is characteristic of RDD. A review of the literature and a discussion of the differential diagnosis of this rare lesion are also presented.Journal of Neurosurgery 09/1999; 91(2):335-9. DOI:10.3171/jns.1999.91.2.0335 · 3.23 Impact Factor