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Table of Contents    
Year : 2019  |  Volume : 67  |  Issue : 3  |  Page : 896-898

Primary spinal Rosai–Dorfman disease: Report of an unusual extradural pathology

1 Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, Whitefield, Bengaluru, Karnataka, India
2 Department of Pathology, Sri Sathya Sai Institute of Higher Medical Sciences, Whitefield, Bengaluru, Karnataka, India

Date of Web Publication23-Jul-2019

Correspondence Address:
Dr. Sumit Thakar
Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, EPIP Area, Whitefield, Bengaluru - 560 066, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.263188

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How to cite this article:
Atal AA, Thakar S, Ghosal N, Hegde A. Primary spinal Rosai–Dorfman disease: Report of an unusual extradural pathology. Neurol India 2019;67:896-8

How to cite this URL:
Atal AA, Thakar S, Ghosal N, Hegde A. Primary spinal Rosai–Dorfman disease: Report of an unusual extradural pathology. Neurol India [serial online] 2019 [cited 2020 Jun 6];67:896-8. Available from:


Rosai–Dorfman disease (RDD) is an uncommon non-neoplastic lymphoproliferative disorder.[1] Given its rarity as well as nonspecific symptoms and imaging findings, the disorder is often underdiagnosed on imaging studies. Isolated intraspinal involvement in RDD without accompanying systemic manifestations is anecdotal.[2]

A 45-year old man presented with progressive spastic paraparesis and urge incontinence for 3 months. Neurological examination revealed spastic lower limbs with grade 1/5 power, exaggerated deep tendon reflexes, and upgoing plantars. Magnetic resonance imaging [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d showed a D8-9 extradural lesion that was isointense on T1 and T2 sequences and was homogenously enhancing with contrast. It had compressed the cord anteriorly and to the right side. He underwent D8, 9 laminectomy and excision of the lesion. Histopathological examination [Figure 2]a and [Figure 2]b showed numerous histiocytes with lymphoplasmacytic infiltrates, prominent emperipolesis, immunopositivity for S-100 protein, and immunonegativity for CD1a. After ruling out systemic disease, the overall diagnosis was that of primary spinal RDD. Postoperatively, there was improvement in power in the lower limbs to grade 3/5 bilaterally. He was referred to a hematologist for further management.
Figure 1: Sagittal MRI sequences: (a) T2-weighted and (b) T1-weighted image demonstrating an isointense extradural lesion at D8, 9 levels with (c) gadolinium enhanced T1 sequence showing homogenous uptake of contrast. (d) T1 axial image with contrast showing the lesion displacing the cord ventrally and to the right. MRI = magnetic resonance imaging

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Figure 2: Paraffin section showing (a) numerous histiocytes with lymphoplasmacytic infiltrate and (b) emperipolesis by the histiocytes (straight arrow) and (Inset B) immunopositivity for S-100 protein (Inset). [Hematoxylin and Eosin: (a) ×100; (b) ×400] [B-Inset × 100: Avidin Biotin Immune Complex]

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Rosai and Dorfman first described this disease and labeled it “sinus histiocytosis with massive lymphadenopathy.” The pathogenesis of the disease is related to cytokine-mediated migration of monocytes and histiocyte accumulation and activation triggered by different stimuli in the setting of coexistent autoimmune diseases, hematological malignancies, or viral infections, such as Herpes virus 6 and Epstein-Barr virus. The most frequent clinical presentation of RDD is a massive bilateral and painless cervical lymphadenopathy with fever, night sweats, and weight loss. Extranodal involvement by RDD has been documented in 43% of cases.[3] Neurological manifestations of the disease are exceedingly uncommon, with extranodal involvement of the spinal cord being reported in <5% of all the cases.

RDD often has a benign course, with treatment being required only in a minority of cases. Active therapy in the form of surgery and high-dose corticosteroids is required for patients with extranodal RDD having vital organ involvement or those with nodal disease causing life-threatening complications.[4],[5] Surgery is generally limited to biopsy, but debulking may be required in patients with vital organ compromise.[2] Radiotherapy is indicated in resistant cases or whenever surgery is not feasible, whereas chemotherapy is reserved for patients with life-threatening disease or in nonresponsive and/or relapsing cases.

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  References Top

Russo N, Giangaspero F, Beccaglia MR, Santoro A. Intracranial dural histiocytosis. Br J Neurosurg 2009;23:449-54.  Back to cited text no. 1
Deodhare SS, Ang LC, Bilbao JM. Isolated intracranial involvement in Rosai- Dorfman disease: A report of two cases and a review of the literature. Arch Pathol Lab Med 1998;122:161-5.  Back to cited text no. 2
Foucar E, Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): A review of the entity. Semin Diagn Pathol 1990;7:19-73.  Back to cited text no. 3
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. 4
Kumar R, Garg K, Dash C, Sharma MC. Rosai–Dorfman disease: An unusual isolated cavernous sinus lesion. Neurol India 2016;64:1331-2.  Back to cited text no. 5
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