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Table of Contents    
LETTER TO EDITOR
Year : 2016  |  Volume : 64  |  Issue : 6  |  Page : 1331-1332

Rosai–Dorfman disease: An unusual isolated cavernous sinus lesion


Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication11-Nov-2016

Correspondence Address:
Rajinder Kumar
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.193820

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How to cite this article:
Kumar R, Garg K, Dash C, Sharma MC. Rosai–Dorfman disease: An unusual isolated cavernous sinus lesion. Neurol India 2016;64:1331-2

How to cite this URL:
Kumar R, Garg K, Dash C, Sharma MC. Rosai–Dorfman disease: An unusual isolated cavernous sinus lesion. Neurol India [serial online] 2016 [cited 2019 Jul 19];64:1331-2. Available from: http://www.neurologyindia.com/text.asp?2016/64/6/1331/193820


Sir,

Rosai–Dorfman disease (RDD) commonly presents as a massive lymphadenopathy; an isolated central nervous system (CNS) involvement is rare.[1],[2] We present a case of isolated cavernous sinus lesion that was diagnosed as trigeminal schwannoma based on the preoperative radiology, and which turned out to be RDD on histopathological examination.

An 18-year-old boy presented with complaints of lancinating pain on the left side of face for the past 1 year. He also complained of decreased sensation over the left side of face for the last 6 months. His physical examination was unremarkable. The neurological examination revealed about 70% loss to touch and pain sensation in the left V1 distribution and 40% loss in the left V2 distribution. Rest of the neurological examination, including that of the III, IV, and VI cranial nerves, was normal. Magnetic resonance imaging revealed an extraaxial lesion in the left cavernous sinus region with an infratentorial extension [Figure 1]. The lesion was isointense on both T1 weighted (W) and T2W images. After gadolinium administration, the lesion showed a homogenous brilliant contrast enhancement. The patient underwent a left temporal craniotomy and excision of the lesion. Intraoperatively, the lesion was firm and adherent to the dura. Histopathological examination revealed the lesion to be RDD [Figure 2].
Figure 1: (a) T2 weighted image, axial section, showing an isointense lesion in the left cavernous sinus region; (b) T1 weighted axial image after gadolinium administration showing the brilliantly enhancing lesion in the left cavernous sinus region, and (c) T1 weighted coronal image after gadolinium administration showing the brilliantly enhancing lesion in the left cavernous sinus region

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Figure 2: Photomicrographs showing an admixture of lymphocytes, neutrophils, and histiocytes (a, Hematoxylin and eosin [H and E] ×200). Higher magnification showing emperipolesis of lymphocytes in the histiocytes (b, H and E × 400). These histiocytes are immunopositive on S-100 and CD68 staining (c and d, ×400 each)

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Rosai and Dorfman first described RDD in 1969.[3] It is also known as sinus histiocytosis. It is a benign histiocytic proliferative disease with pathognomonic histological and immunohistochemical features. It commonly presents as massive lymphadenopathy, with cervical lymph nodes being the most commonly involved nodes. However, the disease can be extranodal in some cases, and even the extranodal disease is mostly associated with lymphadenopathy. Isolated central nervous system (CNS) involvement is very rare with fewer than 40 reported cases.[1],[2] Children or young adults are most commonly affected.[3] On histological examination, RDD consists of proliferation of foamy histiocytes and B and T lymphocytes, as well as phagocytosis of lymphocytes by histiocytes. The pathogenesis is not yet clear; however, most theories implicate an aberrant response to an unknown antigen, possibly an infectious organism.[3],[4] RDD can occur anywhere in the CNS; 77% of cases present with intracranial lesions, 14% with spinal involvement, and 9% with both spinal and intracranial disease.[5] Most of the lesions are extra-axial and are attached to the dura; few lesions are parenchymal and intramedullary. RDD mimicking a trigeminal schwannoma has never been reported in the literature. The classical presenting features of the disease are cervical lymphadenopathy (seen in 90% of cases) with fever, malaise, weight loss, mild anemia, leukocytosis, polyclonal hypergammaglobulinemia, and an elevated sedimentation rate.[4] The disease may be persistent and progressive in some cases, although it has been commonly reported as a benign disorder.[5],[6] Mortality due to the disease has been reported in a few patients in the available literature.

On computed tomography scan, the lesion may appear as a homogeneous isodense or hyperdense mass, with perilesional edema and marked contrast enhancement. There may be erosion of the adjacent bone.[7] The lesions may appear homogeneously isointense to hypointense on T1WI, with strong inhomogeneous or homogeneous enhancement after gadolinium injection.[7] On T2WI, lesions always appear heterogeneously hypointense to isointense with an intensity similar to that of the adjacent dura, which is intensely enhanced with gadolinium chelate contrast agents.[7]

There are no well-defined treatment protocols for RDD. Surgery and radical resection when feasible, are usually the treatment of choice for the management of CNS lesions.[8],[9] Some surgeons have tried adjuvant radiotherapy. Chemotherapy, although it is of no proven role,[8] has also been used in patients with CNS lesions with extensive systemic involvement. There are certain reporting the successful use of corticosteroids and azathioprine in the management of this lesion.[10],[11]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Camp SJ, Roncaroli F, Apostolopoulos V, Weatherall M, Lim S, Nandi D. Intracerebral multifocal Rosai-Dorfman disease. J Clin Neurosci 2012;19:1308-10.  Back to cited text no. 1
    
2.
Griffiths SJ, Tang W, Parameswaran R, Kelsey A, West CG. Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child. Br J Neurosurg 2004;18:293-7.  Back to cited text no. 2
    
3.
Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 1969;87:63-70.  Back to cited text no. 3
    
4.
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. 4
    
5.
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. 5
    
6.
Le Guenno G, Galicier L, Uro-Coste E, Petitcolin V, Rieu V, Ruivard M. Successful treatment with azathioprine of relapsing Rosai-Dorfman disease of the central nervous system. J Neurosurg 2012;117:486-9.  Back to cited text no. 6
    
7.
La Barge DV 3rd, Salzman KL, Harnsberger HR, Ginsberg LE, Hamilton BE, Wiggins RH 3rd, et al. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Imaging manifestations in the head and neck. AJR Am J Roentgenol 2008;191:W299-306.  Back to cited text no. 7
    
8.
Pulsoni A, Anghel G, Falcucci P, Matera R, Pescarmona E, Ribersani M, et al. Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Report of a case and literature review. Am J Hematol 2002;69:67-71.  Back to cited text no. 8
    
9.
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. 9
    
10.
Le Guenno G, Galicier L, Uro-Coste E, Petitcolin V, Rieu V, Ruivard M. Successful treatment with azathioprine of relapsing Rosai-Dorfman disease of the central nervous system. J Neurosurg 2012;117:486-9.  Back to cited text no. 10
    
11.
McPherson CM, Brown J, Kim AW, DeMonte F. Regression of intracranial Rosai-Dorfman disease following corticosteroid therapy. Case report. J Neurosurg 2006;104:840-4.  Back to cited text no. 11
    


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