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CORRESPONDENCE
Year : 2012  |  Volume : 8  |  Issue : 4  |  Page : 647-649

Extranodal Rosai-Dorfman Disease presenting as spinal extradural lesion: A case report with a review of the literature


1 Department of Radiotherapy, IPGMER and SSKM Hospital, Kolkata, West Bengal, India
2 Drs. Tridedi and Roy Diagnostic Laboratory, Department of Pathology, Kolkata, West Bengal, India

Date of Web Publication29-Jan-2013

Correspondence Address:
Animesh Saha
2/1A, Kalinath Munsi Lane, Kolkata, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.106588

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 > Abstract 

Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease (RDD) is a rare, but well-documented entity. We report a male patient who presented with progressive paraparesis, with thoracolumbar extradural lesion (from D11 to L2 level) on magnetic resonance imaging (MRI). He underwent D12-L2 laminectomy followed by total removal of extradural spinal space-occupying lesion (SOL). Histopathological diagnosis of the lesion was RDD. Four weeks after surgery, he was treated with external beam radiotherapy, total dose: 50.4GY in 28 fractions. On three-month follow-up, he did not have any neurological deficits. There was no evidence of other extranodal or lymph node involvement. This case has been reported on account of rare presentation of this disease as spinal extradural lesion. Pertinent literature has been reviewed.

Keywords: Radiotherapy, Rosai-Dorfman disease, spinal extradural lesion


How to cite this article:
Roy C, Saha A, Roy S, Ghosh A. Extranodal Rosai-Dorfman Disease presenting as spinal extradural lesion: A case report with a review of the literature. J Can Res Ther 2012;8:647-9

How to cite this URL:
Roy C, Saha A, Roy S, Ghosh A. Extranodal Rosai-Dorfman Disease presenting as spinal extradural lesion: A case report with a review of the literature. J Can Res Ther [serial online] 2012 [cited 2019 Oct 20];8:647-9. Available from: http://www.cancerjournal.net/text.asp?2012/8/4/647/106588


 > Introduction Top


Rosai-Dorfman disease (RDD) is a rare, benign histiocytic disorder initially described as a separate entity in 1969 by Rosai and Dorfman under the term sinus histiocytosis with massive lymphadenopathy (SHML). [1] Typical clinical presentation is massive painless cervical lymphadenopathy, but extranodal involvement is rare, seen in 30-40% of the cases, with skin, upper respiratory tract, orbit and eye, salivary gland, bone, and testis being reported. [1] In less than 5% of the cases, the central nervous system (CNS) is involved. [2] Involvement of the spine has been described in only 20-25% of those with CNS involvement [3],[4] and is generally not accompanied by lymphadenopathy. [3] We report a rare case of extranodal RDD presenting with extradural spinal cord compression, and briefly review the literature.


 > Case Report Top


A 32-year-old male presented with a two-month history of an insidious onset and gradually progressive low back pain and asymmetric spastic paraparesis without any sphincter disturbance. Neurological examination revealed hypertonia of both lower limbs. He had a motor power of 3/5 in the right lower limb and 2/5 in left lower limb, with decreased sensation below D12. Deep tendon reflexes were brisk and bilateral plantar response was extensor. No lymph node was palpable on clinical examination. Complete hemogram, liver function test, and renal function test were normal. Chest X-ray posteroanterior (PA) view and ultrasonography (USG) of the whole abdomen and neck did not reveal any abnormality. Magnetic resonance imaging (MRI) of lumbosacral spine (plain and contrast) revealed a long biconvex enhancing extramedullary extradural lesion (about 9.3 cm×1.2 cm) seen in the posterior aspect of the thoracolumbar spinal canal (from D11 to L2 level), which had pushed the lower cord anteriorly and had compressed the adjacent spinal canal. The lesion was isointense to mildly hyperintense in T1 and hyperintense in T2 and STIR (STIR: short TI inversion recovery). There was early lumbar spondylosis with degenerative disc disease with disc desiccation at L4/L5 along with mild disc desiccation at L3/L4. There was an annular tear at L4/L5 with associated mild posterior disc protrusion [Figure 1]. He underwent D12-L2 laminectomy followed by total removal of the extradural spinal space-occupying lesion (SOL). Histopathology revealed a lesion composed of dilated spaces filled with histiocytes admixed with lymphocytes and plasma cells, but no granuloma found [Figure 2]. The histiocytic cells expressed S-100 protein [Figure 3], confirming the diagnosis of extranodal RDD. Special stains for micro-organisms were negative. Postoperatively, the patient improved gradually with physiotherapy. Four weeks after surgery, the patient was treated with external beam radiotherapy, at a total dose of 50.4 Gy in 28 fractions (1.8 Gy per fraction, five fractions per week). At the last three-month postradiotherapy follow-up, the patient was doing well. There was no evidence of other extranodal or lymph node involvement.
Figure 1: MRI of lumbosacral spine (plain and contrast) showing a long biconvex enhancing extramedullary lesion (about 9.3 cm×1.2 cm) in the posterior aspect of thoracolumbar spinal canal (from D11 to L2 level), which has pushed the lower cord anteriorly and has compressed the adjacent spinal canal. The lesion is isointense to mildly hyperintense in T1 and hyperintense in T2 and STIR. There is early lumbar spondylosis with degenerative disc disease and disc desiccation at L4/L5 along with mild disc desiccation at L3/L4. There is annular tear at L4/L5 with associated mild posterior disc protrusion

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Figure 2: Histopathology shows a lesion composed of dilated spaces filled with histiocytes admixed with lymphocytes and plasma cells. No granuloma is found (hematoxylin and eosin, 400x)

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Figure 3: The histiocytic cells express S-100 protein (immunoperoxidase, 400x)

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 > Discussion Top


CNS RDD can present at any age, but does so most commonly in the third and fourth decade, with a slight male predominance. [1] Over 90% of CNS RDD involves the leptomeninges and is seen in neuroimaging as a dural-based, contrast-enhancing lesion, mimicking a meningioma. [2],[3] Although thought to be benign, reversal of neurological deficits is mostly incomplete, and deaths have been reported due to infiltration of the vital organs. [1] The etiology of RDD is presumed to be reactive in nature, as no evidence of clonality has been demonstrated by molecular analysis. A possible association with infective agents like the human herpes virus 6 (HHV-6) and parvovirus B19 (Parvo B19) have been suggested in a few studies. [5] The current hypothesis is that RDD is a subtle, yet undefined immunological defect, which promotes monocyte recruitment from the circulation into the nodal or extranodal sites followed by transformation into the immunophenotypically distinct RDD histiocytes demonstrating emperipolesis with functional uniqueness in terms of the cytokine expression profile. [6] Histologically, lymph nodes show pericapsular fibrosis and dilated sinuses, heavily infiltrated with large histiocytes, lymphocytes, and plasma cells. The presence of emperipolesis, or the engulfment of lymphocytes and erythrocytes by histiocytes that express S-100, is considered diagnostic of RDD, although not uniquely. Cytologically, emperipolesis is a consistent finding in nodal disease, which is less apparent in extranodal sites, where fibrosis is commonly seen. Apart from S-100 antigen positivity, immunohistochemical stains of RDD cells are also positive for CD68, CD163, α1-antichymotrypsin, α1-antitrypsin, fascin, and HAM-56, whereas CD1a is typically negative. [7] A biopsy remains the gold standard for diagnosis and the differential diagnosis includes hematopoietic and primary CNS lesions that are accompanied by dense inflammatory infiltrates, such as lymphoplasmacytic meningioma, Langerhans histiocytosis, nodular sclerosing variant of Hodgkin's disease, intracranial plasmacytoma, and plasma cell granulomas. Immunohistochemistry aids in differentiating these from the other Langerhans and non-Langerhans cell histiocytosis. The possibility that many of the earlier reported cases of CNS plasma cell granulomas or inflammatory pseudotumors are RDD, has also been suggested. [8] There is no established treatment protocol for systemic RDD as it is often self-limiting except in the event of the compression of vital organs or airway obstruction. Local recurrence is possible following surgical resection. [1],[4] Steroids often resolve fever and reduce lymph node size. The role of radiotherapy and chemotherapy is controversial. There have been some reports of success in treating RDD with a combination of cytotoxic agents such as alkylating agents, vinca alkaloids, and prednisone. [9] A thorough search of the literature revealed at least 35 previously reported cases with spinal involvement, as depicted in [Figure 4], although isolated spinal involvement was seen in only six of them. Apart from this, there was an isolated reported case with spinal involvement by Maiti et al. in 2011. [1] In our case, a significant challenge was encountered in diagnosing as well as formulating a further line of treatment, in view of the exclusive extranodal involvement of this disease, which resulted in gradually progressive paraparesis. Early diagnosis of the extranodal intraspinal RDD can help to avoid a permanent long-term neurologic sequel only if there is a high index of suspicion on the part of clinicians and pathologists, of this very rare cause of spinal cord compression. [10]
Figure 4: Literature review of published cases of spinal RDD

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 > Acknowledgment Top


The authors acknowledge the co-operation of the patient's relatives for providing the reports and related material for our study.

 
 > References Top

1.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. 1
[PUBMED]  Medknow Journal  
2.Ma J, Xiao J, Wang L. Extranodal Rosai-Dorfman disease with multilevel lumbar spinal lesions. J Neurosurg Spine 2008;9:55-7.  Back to cited text no. 2
[PUBMED]    
3.Hargett C, Bassett T. Atypical presentation of sinus histiocytosis with massive lymphadenopathy as an epidural spinal cord tumor: A case presentation and literature review. J Spinal Disord Tech 2005;18:193-6.  Back to cited text no. 3
[PUBMED]    
4.Raslan O, Ketonen LM, Fuller GN, Schellingerhout D. Intracranial Rosai-Dorfman disease with relapsing spinal lesions. J Clin Oncol 2008;26:3087-9.  Back to cited text no. 4
[PUBMED]    
5.Mehraein Y, Wagner M, Remberger K, Fuzesi L, Middel P, Kaptur S, et al. Parvovirus B19 detected in Rosai-Dorfman disease in nodal and extranodal manifestations. J Clin Pathol 2006;59:1320-6.  Back to cited text no. 5
    
6.Middel P, Hemmerlein B, Fayyazi A, Kaboth U, Radzun HJ. Sinus histiocytosis with massive lymphadenopathy: Evidence for its relationship to macrophages and for a cytokine-related disorder. Histopathology 1999;35:525-33.  Back to cited text no. 6
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7.Juskevicius R, Finlay JL. Rosai-Dorfman disease of the parotid gland, cytologic and histopathologic findings with immunohistochemical correlation. Arch Pathol Lab Med 2001;125:1348-50.  Back to cited text no. 7
    
8.Mirra SS, Tindall SC, Check IJ, Brynes RK, Moore WW. Inflammatory meningeal masses of unexplained origin. An ultrastructural and immunological study. J Neuropathol Exp Neurol 1983;42:453-68.  Back to cited text no. 8
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9.Konca C, Ozkurt ZN, Deger M, Aki Z, Yagci M. Extranodal multifocal Rosai-Dorfman disease: Response to 2-chlorodeoxyadenosine treatment. Int J Hematol 2009;89:58-62.  Back to cited text no. 9
    
10.Dran G, Rasendrarijao D, Vandenbos F, Paquis P. Rosai-Dorfman disease causing spinal cord compression: Case report. Neurosurgery 2008;62:977-8.  Back to cited text no. 10
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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