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Neurology India
Medknow Publications on behalf of the Neurological Society of India
ISSN: 0028-3886 EISSN: 1998-4022
Vol. 59, Num. 3, 2011, pp. 329-330

Neurology India, Vol. 59, No. 3, May-June, 2011, pp. 329-330


Rosai-Dorfman Disease and neurological manifestations

C Sundaram

Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India

Correspondence Address: C Sundaram Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh India

Date of Submission: 24-May-2011
Date of Decision: 24-May-2011
Date of Acceptance: 24-May-2011

Code Number: ni11103

PMID: 21743156

DOI: 10.4103/0028-3886.82705

Rosai-Dorfman Disease (RDD), sinus histocytosis with massive lymphadenopathy is an idiopathic histocytic proliferation affecting the lymph nodes in its classic form. It may affect many diverse sites with or without nodal involvement; the estimated incidence of classic nodal presentation of RDD is approximately 100 cases per year. The extranodal disease is seen in about 43% of cases and central nervous system (CNS) involvement is seen in less than 5% of cases. [1],[2] A review of the literature on CNS involvement in RDD from 1969-2008 observed a sudden increase in the reports of RDD of CNS from 2002-2008. [3] Review of Indian literature between 1996 and 2010 revealed report of 20 cases of RDD of CNS, mostly from south India, 17 cases. [4],[5],[6],[7],[8],[9],[10],[11],[12] This included 18 patients with intracranial lesions, one patient with spinal, and one patient with both intracranial and spinal lesions. There were two patients with multiple intraparenchymal lesions and one patient with dural-based lesions involving multiple levels. The intracranial dural-based lesion involved bone in two patients and there was erosion of bone in two patients. All the cases of RDD were isolated involvement of CNS and confirmation of diagnosis was made by histopathology and immunohistochemistry. None of the patients were diagnosed preoperatively. [4],[5],[6],[7],[8],[9],[10],[11],[12] CNS involvement in RDD affects all the age groups with a mean age of 39.3 years. [3] About 70% patients with intracranial RDD had no lymphadenopathy and 52% had no systemic disease [3],[12] In this issue of the journal, Venkidesh et al., [13] report a patient with isolated intracranial RDD without nodal involvement. Intracranial lesions were reported in 77%, spinal lesions in 14% and both intracranial and spinal lesions in 9% of patients. [3],[14],[15] Majority (85%) of the intracranial lesions were dural-based lesions mimicking meningioma located in the convexity or skull base. Intraparenchymal lesions are rarely reported. The spinal lesions may be cervical, thoracic or lumbar. [3] Maiti et al., [16] has reported a patient with spinal extradural lesion in this issue of the journal. Intramedullary involvement is rare. Lesions may be single or multiple. There may be invasion of the dura, bone or sinuses. Multiple intraparenchymal lesions are extremely rare. [5],[7]

Neuroimaging features are often non-specific and a preoperative diagnosis is often difficult. On computed tomography (CT), the lesion may appear as a homogenous lobulated hyperattenuating mass with marked contrast enhancement. There may be moderate to marked perilesional edema with mass effect. There can be associated bone erosion but not calcification. [14],[15] On magnetic resonance imaging (MRI), the lesions are lobulated, isointense on T1W1 with homogenous intense enhancement on gadolinium injection. On T2W1 the lesions appear hypo- to isointense. MR spectroscopy with elevated choline may improve the specificity of preoperative diagnosis and help in differential diagnosis from meningioma. [17]

Histopathology forms the mainstay of diagnosis. Classically, the lesions are composed of lymphoplasmacytic infiltrate and scattered Russel bodies. [1],[2] There is characteristic histiocytic proliferation and emperipolesis. Though the features are essentially similar to nodal disease, emperipolesis is less pronounced and fibrosis is more in extranodal disease. Immunohistochemistry demonstrates positivity for CD 68 and S100 due to the histiocytic nature of the lesion and RDD needs to be differentiated from other inflammatory and neoplastic lesions of the CNS, especially dural-based lesions. These include Langerhans cell histiocytosis, Hodgkin's lymphoma, plasmacytoma, plasma cell granuloma or inflammatory pseudotumor. [18] The emperipolesis and immunohistochemical evaluation will help to rule out other diagnostic possibilities, especially in the isolated form where there is no lymphadenopathy or other system involvement. The etiology of RDD is still uncertain. Molecular studies have demonstrated that RDD is a polyclonal disorder. A definite infectious agent has not been isolated though various studies have been carried out to isolate an etiological agent.

In conclusion CNS involvement in RDD is still rare, though there is an increase in the documentation of CNS-RDD. However, because of the ubiquity of its involvement of the entire neuraxis and its ability to mimic meningeal and primary brain tumors, it is essential to be aware of this entity and consider RDD in the differential diagnosis of various lesions of the CNS.


1.Foucar E, Rosai J, Dorfman RF, Brynes RK. The neurologic manifestations of sinus histiocytosis with massive lymphadenopathy. Neurology 1982;32:365-72.   Back to cited text no. 1  [PUBMED]  
2.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. 2  [PUBMED]  
3.Adeleye AO, Amir G, Fraifeld S, Shoshan Y, Umansky F, Spektor S. Diagnosis and management of Rosai-Dorfman disease involving the central nervous system. Neurol Res 2010;32:572-8.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Panicker NK, Sabhikhi AK, Rai R. Rosai-Dorfman disease presenting as a meningiomas. Indian J Cancer 1996;33:192-4.  Back to cited text no. 4  [PUBMED]  
5.Purav P, Ganapathy K, Mallikarjuna VS, Annapurneswari S, Kalyanaraman S, Reginald J, et al. Rosai-Dorfman disease of the central nervous system. J Clin Neurosci 2005;12;656-9.  Back to cited text no. 5    
6.Sharma MS, Padma MD, Jha AN. Rosai-Dorfman disease mimicking a sphenoid wing meningiomas. Neurol India 2005:53:110-1.   Back to cited text no. 6    
7.Sundaram C, Uppin SG, Prasad BC, Sahu BP, Devi MU, Prasad VS, et al. Isolated Rosai Dorfman disease of the central nervous system presenting as dural based and intra parenchymal lesions. Clin Neuropathol 2005;24:112-7.   Back to cited text no. 7  [PUBMED]  
8.Bhandari A, Patel PR, Parel MP. Extra nodal Rosai Dorfman disease with multiple spinal lesions: a rare presentation. Surg Neurol 2006;65:308-11.  Back to cited text no. 8    
9.Ghosal N, Murthy G, Visvanathan K, Sridhar M, Hegde AS. Isolated intracranial Rosai Dorfman disease masquerading as meningiomas: A case report. Indian J Pathol Microbiol 2007;50:382-4.  Back to cited text no. 9  [PUBMED]  
10.Gupta DK, Suri A, Mahapatra AK, Mehta VS, Garg A, Sarkar C, et al. Intracranial Rosai Dorfman disease in a child mimicking bilateral giant petroclival meningiomas: A case report and review of literature. Childs Nerv Syst 2006;22:1194-200.   Back to cited text no. 10  [PUBMED]  [FULLTEXT]
11.Kumar KK, Menon G, Nair S, Radhakrishnan VV. Rosai-Dorfman disease mimicking chronic subdural hematoma. J Clin Neurosci 2008;15:1-2.  Back to cited text no. 11    
12.Ambedkar S, Somanna S, Bhat D, Ranjan M. Isolated cranio-spinal involvement of Rosai Dorfman disease: Case report. Br J Neurosurg 2010;25:297-9.  Back to cited text no. 12    
13.Krishnamoorthy V, Parmar CF, Dilip P. Isolated intracranial Rosai Dorfman disease: Case report. Neurol India 2011;59:443-6.  Back to cited text no. 13  [PUBMED]  
14.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. 14  [PUBMED]  [FULLTEXT]
15.Kattner KA, Stroink AR, Roth TC, Lee JM. Rosai-Dorfman disease mimicking parasagittal meningiomas: Case presentation and review of literature. Surg Neurol 2000;53:452-7.   Back to cited text no. 15  [PUBMED]  [FULLTEXT]
16.Maiti TK, Gangadharan J, Mahadevan A, Arivazhagan A, Chandramouli BA, Shankar SK. Rosai-Dorfman disease presenting as cervical extradural lesion: A case report with review of literature. Neurol India 2011;59:438-42.  Back to cited text no. 16  [PUBMED]  
17.Baruah D, Guleria S, Chandra T. Rosai-Dorfman disease with extensive extra nodal involvement. Eur J Radiol 2007;62:31-3.  Back to cited text no. 17    
18.Johnson MD, Powell SZ, Boyer PJ. Weil RJ, Moots PL. Dural lesions mimicking meningiomas. Hum Pathol 2002;33:1211-26.  Back to cited text no. 18    

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