J Korean Soc Spine Surg.  2006 Mar;13(1):64-68. 10.4184/jkss.2006.13.1.64.

Rosai-Dorfman Disease Occurred in Epidural Space of the Thoracic Spinal Canal

• Shin BJ
• Lee JC
• Nam JS
• Yoon ES
• Kim DW
• Cho YI
• Kim YI

• KMID: 2040783
• DOI: http://doi.org/10.4184/jkss.2006.13.1.64

Abstract

Rosai-Dorfman disease is a rare, non-neoplastic lymphoproliferative disorder that is characterized by its specific histological features. However, it is uncommon for it to involve the thoracic spinal cord as a site of extranodal disease. A 36 year-old man developed progressive paraparesis 2 weeks prior to admission. On an MRI study, the spinal cord was compressed at the T4 and T5 levels posteriorly by an epidural mass. A decompressive laminectomy and removal of the mass were performed and Rosai-Dorfman disease was confirmed histologically. After the operation, additional high-dose radiotherapy was performed. The motor weakness and hypesthesia in the lower extremities resolved completely and there was no evidence of recurrence at the final follow-up examination.

Keyword

Thoracic spinal canal; Rosai-Dorfman disease; Laminectomy

MeSH Terms

Adult
Epidural Space*
Follow-Up Studies
Histiocytosis, Sinus*
Humans
Hypesthesia
Laminectomy
Lower Extremity
Lymphoproliferative Disorders
Magnetic Resonance Imaging
Paraparesis
Radiotherapy
Recurrence
Spinal Canal*
Spinal Cord

Figure

• 

  Fig. 1. (A)Sagittal T2-weighted magnetic resonance images of thoracic spine showing extradural mass lesion extending from T2, T3, T4 area. (B)Axial T2-weighted magnetic resonance images at the level of 4th thoracic spine showing extradural mass(arrows) and compression of the cord from central to left side.

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  Fig. 2. Preoperative bone scan showing tear drop shaped increased uptake of upper thoracic spine from T1 to T4.

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  Fig. 3. Histologic findings. (A)A large numbers of histiocytes are interrupted by lymphocytes and plasma cells (B)The histiocytes are positive for S-100 protein.

Reference

1). Sartoris DJ, Resnick D. Osseous involvement in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Eur J Pediatr. 1986; 145:238–40.

2). Park JK, Cho MY, Park KH Pyon JS. Isolated intracranial Rosai-Dorfman disease - a case report. J Kor Pathol. 2004; 38:430–433.

3). 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; 32:236–239.

4). Raveenthiran V, Dhanalakshmi M, Hayavadana Rao PV, Viswanathan P. Rosai-Dorfman disease: report of a 3-year-old girl with critical review of treatment options. Eur J Pediatr Surg. 2003; 13:350–354.

5). 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; 18:193–196.

6). Geara AR, Ayoubi MA, Achram MC, Chamseddine NM. Rosai-Dorfman disease mimicking neurofibromato -sis: case presentation and review of the literature. Clin Radiol. 2004; 59:625–630.

7). Osenbach RK. Isolated extranodal sinus histiocytosis presentimg as an intramedullary spinal cord tumor with paraplegia. J Neurosurg. 1996; 85:692–696.