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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. 443-446

Neurology India, Vol. 59, No. 3, May-June, 2011, pp. 443-446

Case Report

Isolated intracranial Rosai Dorfman disease

Venkidesh Krishnamoorthy1, Chirag F Parmar2, Dilip Panikar1

1 Department of Neurosurgery, Amrita Institute of Medical Sciences, Kochi, Kerala, India
2 Department of Pathology, Amrita Institute of Medical Sciences, Kochi, Kerala, India

Correspondence Address: Venkidesh Krishnamoorthy Manas, Madathilparambu, Palace Ward, Alappuzha - 688 011, Kerala India

Date of Submission: 20-Oct-2010
Date of Decision: 21-Oct-2010
Date of Acceptance: 04-Feb-2011

Code Number: ni11127

PMID: 21743180

DOI: 10.4103/0028-3886.82770


Rosai Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a benign histiocytic proliferative disorder mainly affecting the lymph nodes. Although several cases of extra-nodal involvement have been reported previously, central nervous system involvement, particularly in the absence of nodal disease is extremely rare. We report a case of isolated intracranial RDD occurring in a relatively elder patient, which was shown by histological examination to have a dura-based involvement.

Keywords: Emperipolesis, histiocytosis, lymphadenopathy, Rosai-Dorfman


Sinus histiocytosis with massive lymphadenopathy (SHML), a rare but well-defined clinicopathological entity first described in 1969, affects children and adolescents predominantly. The most common presentation is bilateral painless cervical lymphadenopathy. [1],[2] An extra-nodal manifestation of the disease occurs in up to 43% of the patients; and occurs in the absence of lymphadenopathy in 23% of the patients. The most common extra-nodal sites affected are the soft tissues of the head and neck and the paranasal sinuses and nasal cavity. [2] Isolated central nervous system (CNS) involvement without nodal involvement is extremely rare; only few cases have been reported in literature so far.

Case Report

A 51-year-old male presented with unsteadiness of gait, of four months' duration, occasional episodes of low grade fever, of three months' duration, and a recent onset of blurring of vision, of three weeks' duration. On neurological examination there was left-sided spastic hemiparesis. Clinically he had no lymph node enlargement. Non-contrast computerized tomography (CT) of the brain revealed an ill-defined, extra-axial mass lesion involving the right frontal region with peri-lesional edema. Contrast magnetic resonance imaging (MRI) revealed a dural-based, brightly enhancing lesion, involving the right frontal region, suggestive of convexity meningioma [Figure - 1]a-c. CT of the chest and abdomen did not reveal any lymph node enlargement. Ultrasound examination of the neck was unremarkable. He underwent right frontal craniotomy and excision of the lesion. Pre-operatively the dura was found to be very much thickened, with a nodular inner surface that was impinging on the brain surface. There was a good pial plane between the tumor and the brain surface. The postoperative course was uneventful. Microscopic examination [Figure - 2]a and b revealed dura infiltrated with sheets of histiocytes, lymphocytes, and plasma cells, and focally by polymorphs. The histiocytes showed features of emperipolesis. There was no nuclear grooving or convolutions in the histiocytes. No eosinophils or granuloma or meningothelial cell clusters were seen. Immunohistochemistry for the S-100 protein and CD68 antibody confirmed the presence of histiocytes. Based on these histological features a diagnosis of Rosai Dorfman disease was made. He was discharged on low-dose steroids for a month. MRI done at the end of three months showed no evidence of any residual or recurrent lesion [Figure - 3] a-c and his spasticity involving the left upper and lower limb had significantly improved. [3],[4]


The term SHML was first introduced by Ronald F. Dorfman and Juan Rosai based on four cases, published in 1969, in the Archives of Pathology journal, with the title 'Sinus histiocytosis with massive lymphadenopathy: A newly recognized benign clinicopathologic entity'. [1] The synonym of this condition is Destombes-Rosai-Dorfman syndrome, named after Pierre-Paul Louis Lucien Destombes, Juan Rosai, and Ronald F. Dorfman.

The disease is characterized by massive, painless, bilateral cervical lymphadenopathy, often associated with fever, leukocytosis, mild anemia, elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia. [2] Although extra-nodal involvement has been reported in diverse sites, CNS involvement, particularly in the absence of nodal disease is rare . [2] The most common presentations of CNS involvement, in the largest series, include headaches, seizures, numbness, and paraplegia. [5],[6] Frequent lesion locations include cerebral convexities, para-sagittal, supra-sellar, cavernous sinus, and petroclival regions. [5],[7],[8],[9],[10],[11] Purely intraparenchymal lesions have also been reported. [5],[12] There are isolated case reports involving the orbit, pituitary, cerebellar, and cervical extradural involvement. [13],[14],[15],[16],[17] The other extra-nodal sites include skin, soft tissues, respiratory system, genitourinary system, bones, orbit, thyroid, and breast. [2],[18],[19],[20],[21],[22],[23] Male to female ratio is about 1.4 : 1. [18],[19] The etiology, pathogenesis, and natural history of Rosai-Dorfman disease are still unknown. Although an infection has been suggested as an underlying cause, a definitive agent has never been isolated. [2] Molecular studies using polymorphic regions of the human androgen receptor locus have demonstrated that RDD is a polyclonal disorder, [1],[2] in contrast to Langerhans cell histiocytosis, which is a monoclonal disorder. [24]

Histologically, Rosai Dorfman disease is characterized by large histiocytes, which often show apparently viable and intact hematopoietic cells (mostly lymphocytes) within the cytoplasm without undergoing degenerative changes, a phenomenon known as Emperipolesis (lymphophagocytosis), derived from the Greek word 'em + peripolesis' = going about. On immunohistochemical examination, these are positive for S-100 protein and negative for CD1a, a marker of Langerhans histiocytosis. [25],[26]

There are no definitive treatment guidelines for Rosai Dorfman disease. A majority of these undergo spontaneous regression. The mortality rate is approximately low, 7%. Major complications include airway obstruction, immunological disorder, and infection. Usually, concomitant immune dysfunction is present. [27] The prognosis of CNS Rosai Dorfman disease is not bad. Petzold et al. found tumor regrowth or recurrence of symptoms in 14% of the 29 patients, with a mean follow-up of 10.1 years. Only 52% of these patients had undergone brain imaging at follow-up. They concluded that a five-year follow-up with brain imaging was essential and advocated low-dose radiation to treat patients with sub-total resection and recurrence. Although a variety of treatment modalities had been used, including steroid therapy and radiation, surgical resection appeared to be the most appropriate approach. [28],[29],[30] There have been some reports of success in treating Rosai-Dorfman disease with a combination of cytotoxic agents such as alkylating agents, vinca alkaloids, and prednisone. [31] Decision to follow-up the patient with serial imaging is an accepted modality. Our patient was maintained on low steroids postoperatively, for a month. A repeat MRI done at the end of three months did not reveal any residual or recurrence involving the operative bed.


1.Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: A newly recognized benign clinicopathologic entity. Arch Pathol 1969;87:63-70.  Back to cited text no. 1    
2.Foucar E, Rosai J, Dorfman RF. 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    
3.Foss HD, Herbst H, Araujo I, Hummel M, Berg E, Schmitt-Gräff A, et al. Monokine expression in Langerhans' cell histiocytosis and sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). J Pathol 1996;179:60-5.  Back to cited text no. 3    
4.Paulli M, Bergamaschi G, Tonon L, Viglio A, Rosso R, Facchetti F, et al. Evidence for a polyclonal nature of the cell infiltrate in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Br J Haematol 1995;91:415-8.  Back to cited text no. 4    
5.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. 5    
6.Petzold A, Thom M, Powell M, Plant GT. Relapsing intracranial Rosai-Dorfman disease J Neurol Neurosurg Psychiatry 2001;71:538-41.   Back to cited text no. 6    
7.Kattner KA, Stroink AR, Roth TC, Lee JM. Rosai-Dorfman disease mimicking parasagittal meningioma: Case presentation and review of literature. Surg Neurol 2000;53:452-7.  Back to cited text no. 7    
8.Huang HY, Huang CC, Lui CC, Chen HJ, Chen WJ. Isolated intracranial Rosai-Dorfman disease: Case report and literature review. Pathol Int 1998;48:396-402.  Back to cited text no. 8    
9.Wu M, Anderson AE, Kahn LB. A report of intracranial Rosai-Dorfman disease with literature review. Ann Diagn Pathol 2001;5:96-102.  Back to cited text no. 9    
10.Kitai R, Sato K, Kubota T, Kabuto M, Kawano H, Kobayashi H, et al. Meningeal sinus histiocytosis mimicking lymphoplasmacyte-rich meningioma. J Neurosurg 1996;84:1051-4.  Back to cited text no. 10    
11.Deodhare SS, Ang LC, Bilbao JM. Isolated intracranial involvement in Rosai-Dorfman disease: A report of two cases and review ofthe literature. Arch Pathol Lab Med 1998;122:161-5.  Back to cited text no. 11    
12.Juriæ G, Jakiæ-Razumoviæ J, Rotim K, Zarkoviæ K. Extranodal sinus histiocytosis (Rosai-Dorfman disease) of the brain parenchyma. Acta Neurochir (Wien) 2003;145:145-9.  Back to cited text no. 12    
13.Ng HK, Poon WS. Sinus histiocytosis with massive lymphadenopathy localized to the sella. Br J Neurosurg 1995;9:551-5.  Back to cited text no. 13    
14.Kelly WF, Bradey N, Scoones D. Rosai-Dorfman disease presenting as a pituitary tumour. Clin Endocrinol 1999;50:133-7.  Back to cited text no. 14    
15.Rotondo F, Munoz DG, Hegele RG, Gray B, Khatun N, Bonert M, et al. Rosai-Dorfman disease involving the neurohypophysis. Pituitary 2010;13:256-9.  Back to cited text no. 15    
16.Gaetani P, Tancioni F, Di Rocco M, Rodriguez y Baena R. Isolated cerebellar involvement in Rosai-Dorfman disease: case report. Neurosurgery 2000;46:479-81  Back to cited text no. 16    
17.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. 17    
18.Brenn T, Calonje E, Granter SR, Leonard N, Grayson W, Fletcher CD, et al. Cutaneous Rosai-Dorfman disease is a clinical entity. Am J Dermatopathol 2002;24:385-91.  Back to cited text no. 18    
19.George J, Stacy G, Peabody T, Montag A. Rosai-Dorfman disease manifesting as a solitary lesion of the radius in a 41 year old woman. Skeletal Radiol 2003;32:236-9. Epub 2003 Mar 08.  Back to cited text no. 19    
20.McAlister WH, Herman T, Dehner LP. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Pediatr Radiol 1990;20:425-32.  Back to cited text no. 20    
21.Deshmukh RR, Kumar V, Kumbhari D. Sinus histiocytosis of the thyroid with massive lymphadenopathy (Rosai-Dorfman Disease). J Indian Med Assoc 2003;101:597-8.  Back to cited text no. 21    
22.Ture U, Seker A, Bozkurt SU, Uneri C, Sav A, Pamir MN. Giant intracranial Rosai-Dorfman disease. J Clin Neurosci 2004;11:563-6.  Back to cited text no. 22    
23.Green I, Dorfman RF, Rosai J. Breast involvement by extranodal Rosai-Dorfman disease: Report of seven cases. Am J Surg Pathol 1997;21:664-8.  Back to cited text no. 23    
24.Willman CL, Busque L, Griffith BB, Favara BE, McClain KL, Duncan MH, et al. Langerhans'-cell histiocytosis (histiocytosis X)-a clonal proliferative disease. N Engl J Med 1994;331:154-60.  Back to cited text no. 24    
25.Eisen RN, Buckley PJ, Rosai J. Immunophenotypic characterization of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Semin Diagn Pathol 1990;7:74-82.  Back to cited text no. 25    
26.Warnke RA, Weiss LM, Chan JK, Cleary ML, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). In: Rosai J, editors. Atlas of Tumor Pathology, 3rd Series: Tumors of the Lymph Nodes and Spleen. Washington: Armed Forces Institute of Pathology; 1995. p. 349-60.  Back to cited text no. 26    
27.Wang E, Anzai Y, Paulino A, Wong J. Rosai-Dorfman Disease Presenting with Isolated Bilateral Orbital Masses: Report of Two Cases. AJNR Am J Neuroradiol 2001;22:1386-8.   Back to cited text no. 27    
28.Kim M, Provias J, Bernstein M. Rosai-Dorfman disease mimicking multiple meningioma: Case report. Neurosurgery 1995;36:1185-7.  Back to cited text no. 28    
29.Udono H, Fukuyama K, Okamoto H, Tabuchi K. Rosai-Dorfman disease presenting multiple intracranial lesions with unique findings on magnetic resonance imaging. Case report. J Neurosurg 1999;91:335-9.  Back to cited text no. 29    
30.Sharma MS, Padua MD, Jha AN. Rosai-Dorfman disease mimicking a sphenoid wing meningioma, Neurol India 2005;53:110-1.   Back to cited text no. 30    
31.Komp DM. The treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Semin Diagn Pathol 1990;7:83-6.  Back to cited text no. 31    

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