Isolated Intraventricular Rosai-Dorfman Disease

Abstract

Rosai-Dorfman disease (RDD) is a benign histioproliferative disorder characterized by generalized lymphadenopathy, weakness, anemia, and rarely extranodal involvement. The disease affects the central nervous system (CNS) very rarely and at a relatively older age. Isolated intracranial involvement is very rare. RDD tends to present as dural masses mimicking meningioma, though very rare case reports describe isolated parenchymal involvement in the CNS. Only one case of intraventricular RDD has been described so far to the best of our knowledge. We present a very rare presentation of this disease as an isolated intraventricular presentation in the region of the atria of the lateral ventricles.

Keywords: Extranodal; Rosai Dorfman; intraventricular.

Figure 1

(a and b) Magnetic resonance imaging reveals an intensely enhancing lobulated mass expanding the atrium and the occipital horn of the lateral ventricle with mild perilesional vasogenic edema

Figure 2

Gross examination: the mass is firm, nodular, gray white with the few yellowish areas

Figure 3

Microscopy showed: (a) histiocytes with prominent emperipolesis admixed with mature plasma cells and lymphocytes (H and E, ×40). (b) Histiocytes with emperipolesis are seen. (c) Mature plasma cells with Russell bodies are seen. (d) Macrophages are CD68 positive. (e) Macrophages are S100 positive. (f) Lymphocytes are heterogeneous and consist of both CD3 positive and (g) CD20 positive cells. (h) Kappa and lambda (i) staining by IHC show no light chain restriction