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Child's Nervous System
Published in: Child's Nervous System 11/2005

01-11-2005 | Case-based Update

Spinal cord compression as a result of Rosai–Dorfman disease of the upper cervical spine in a child

Authors: R. Shane Tubbs, David R. Kelly, Elizabeth C. Mroczek-Musulman, Yuki A. Hammers, Roger L. Berkow, W. Jerry Oakes, Paul A. Grabb

Published in: Child's Nervous System | Issue 11/2005

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Abstract

Background

Rosai–Dorfman disease is characterized by massive painless cervical lymphadenopathy, but can also include nasal obstruction, tonsillar enlargement, or hearing abnormalities. The disease occurs most often in the third and fourth decades. Most authors have suggested that it represents either an autoimmune disease or a reaction to an infectious agent that has yet to be discovered. Less than 50 cases of central nervous system involvement have been reported.

Case illustration

We report a child with cervical spinal cord compression due to Rosai–Dorfman disease of the cervical spine.

Prognosis

This disease has variable outcomes from relatively benign to insidious over decades. Our case is unusual in that the patient was a child and had involvement of the spine with resultant cord compression, a combination that has been reported rarely.
Literature
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Foucar E, Rosai J, Dorfman R (1990) Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol 7:19–73PubMed
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Eisen RN, Buckley P, Rosai J (1990) Immunophenotypic characterization of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Semin Diagn Pathol 7:74–82
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Bernard F, Sarran N, Serre I, Baldet P, Callamand P, Margueritte G, Astrue J (1999) Sinus histiocytosis (Destombes-Rosai-Dorfman disease) revealed by extranodal spinal involvement. Arch Pediatr 6:173–177CrossRef
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Hunger RE, Sieling PA, Ochoa MT, Sugaya M, Burdick AE, Rea TH, Brennan PJ, Belisle JT, Blauvelt A, Porcelli SA, Modlin RL (2004) Langerhans cells utilize CD1a and langerin to efficiently present nonpeptide antigens to T cells. J Clin Invest 113:701–708CrossRef
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Paulli M, Bergamaschi G, Tonon L, Viglio A, Rosso R, Facchetti F, Geerts ML, Magrini U, Cazzola M (1995) Evidence for a polyclonal nature of the cell infiltrate in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Br J Haematol 91:415–418
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Foucar E, Rosai J, Dorfman RF, Brynes RK (1982) The neurologic manifestations of sinus histiocytosis with massive lymphadenopathy. Neurology 32:365–372
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Sato A, Sakurada K, Sonoda Y, Saito S, Kayama T, Jokura H, Yoshimoto T, Nakazato Y (2003) Rosai-Dorfman disease presenting with multiple intracranial and intraspinal masses: a case report. No Shinkei Geka 31:1199–1204
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Metadata
Title
Spinal cord compression as a result of Rosai–Dorfman disease of the upper cervical spine in a child
Authors
R. Shane Tubbs
David R. Kelly
Elizabeth C. Mroczek-Musulman
Yuki A. Hammers
Roger L. Berkow
W. Jerry Oakes
Paul A. Grabb
Publication date
01-11-2005
Publisher
Springer-Verlag
Published in
Child's Nervous System / Issue 11/2005
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-004-1121-1

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