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Table of Contents    
Year : 2016  |  Volume : 64  |  Issue : 5  |  Page : 1064-1065

Intracranial Rosai–Dorfman disease: An rare entity with an uncommon presentation

1 Department of Pathology, Kovai Medical Centre and Hospital, Coimbatore, Tamil Nadu, India
2 Department of Neurosurgery, Kovai Medical Centre and Hospital, Coimbatore, Tamil Nadu, India

Date of Web Publication12-Sep-2016

Correspondence Address:
Preithy Uthamalingam
Department of Pathology, Kovai Medical Centre and Hospital, Coimbatore, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.190301

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How to cite this article:
Uthamalingam P, Mehta S, Duraisamy G. Intracranial Rosai–Dorfman disease: An rare entity with an uncommon presentation. Neurol India 2016;64:1064-5

How to cite this URL:
Uthamalingam P, Mehta S, Duraisamy G. Intracranial Rosai–Dorfman disease: An rare entity with an uncommon presentation. Neurol India [serial online] 2016 [cited 2020 May 29];64:1064-5. Available from:


Sinus histocytosis with massive lymphadenopathy, known as Rosai–Dorfman Disease (RDD), is a benign histiocytic proliferation affecting the lymph nodes. Central nervous system (CNS) manifestations, particularly in the absence of nodal disease, are rare.[1]

A 59-year-old male patient presented with progressive loss of vision for one year. He had only perception of light in both the eyes. There were no other positive systemic findings. Contrast enhanced computed tomography (CECT) [Figure 1] of the brain showed multiple mild-to-moderately enhancing extra-axial masses in the anterior and posterior fossae. A radiological impression of “en-plaque” meningiomatosis was made. A frontal craniotomy with optic nerve decompression was performed.
Figure 1: Radiological features: Contrast enhanced computed tomography images at the sellar level (axial plane, a) and mid-sagittal plane (b) show moderately enhancing lobulated extra-axial masses in the sellar, suprasellar, parasellar and tentorial regions

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On histopathological examination, the lesion was composed of large pale histiocytes with admixed lymphoplasmacytic cells, eosinophils, and neutrophils. On careful searching, a few foci of emperipolesis were noted [Figure 2]. There were no granulomas or multinucleated giant cells. These histiocytes were found to be strongly positive for CD68 and S-100 [Figure 2], and were negative for CD1a and epithelial membrane antigen (EMA). A final diagnosis of RDD was given.
Figure 2: Histopathological features: The lesion exhibited a pale, vaguely lobulated architecture (a, hematoxylin and eosin, ×40) with an admixture of large histiocytic cells, plasma cells, eosinophils and lymphocytes (b, Hematoxylin and eosin, ×400). Foci of emperipolesis (presence of intact lymphocytes within the cytoplasm of the histiocytes) were noted (c, Hematoxylin and eosin, ×400). These histiocytes were uniformly positive for S-100 protein (d, Immunohistochemistry, ×200)

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The patient was started on steroids for the residual disease with no progress of the disease at a 2-year follow up.

Isolated intracranial RDD without nodal involvement is rare.[1],[2] In the available literature, most of the central nervous system RDD lesions are intracranial and dural based,[1] and the most common radiological differential diagnosis is a meningioma.[1],[2]

Histopathologically, emperipolesis is often less apparent in CNS lesions.[3] The most common differential diagnosis includes tuberculosis, especially in India, with obvious therapeutic implications.[2] Other differentials include lymphoplasmacytic meningioma, inflammatory pseudotumor and Langerhans cell histiocytosis (LCH). Immunostaining for epithelial membrane antigen and CD1a along with the histology and the classical cytological features help in differentiating lymphoplasmacytic meningioma and LCH, respectively. IgG4-related intracranial lesions, another radiological differential for meningioma, can be differentiated histologically based on the histiocytic contribution to the lesion and immunostaining for IgG4.[4]

The prognosis of a completely resected intracranial RDD is favorable, especially in the absence of nodal disease.[2] Controversies exist in the treatment of the residual disease.[2] The index patient was started on steroids and has non-progressive disease till date. However, his long-term outcomes is unpredictable at this stage.

This case illustrates the importance of an accurate histopathological diagnosis of RDD in a neurosurgical patient.[5] This is because the differential diagnoses are only a few but all of them are significantly different in terms of prognostication and further management of the patient.

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Conflicts of interest

There are no conflicts of interest

  References Top

Andriko JA, Morrison A, Colegial CH, Davis BJ, Jones RV. Rosai-Dorfman disease isolated to the central nervous system: A report of 11 cases. Mod Pathol 2001;14:172-8.  Back to cited text no. 1
Symss NP, Cugati G, Vasudevan MC, Ramamurthi R, Pande A. Intracranial Rosai Dorfman Disease: Report of three cases and literature review. Asian J Neurosurg 2010;5:19-30.  Back to cited text no. 2
[PUBMED]  Medknow Journal  
Foucar E, Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Review of the entity. Semin Diagn Pathol 1990;7:19-73.  Back to cited text no. 3
Nishino T, Toda J, Nakatsuka T, Kimura T, Inaoka T, Terada H. IgG4-related inflammatory pseudotumors mimicking multiple meningiomas. Jpn J Radiol 2013;31:405-7.  Back to cited text no. 4
Krishnamoorthy V, Parmar CF, Panikar D. Isolated intracranial Rosai Dorfman disease. Neurol India 2011; 59: 443-446.  Back to cited text no. 5
[PUBMED]  Medknow Journal  


  [Figure 1], [Figure 2]


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