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Isolated intracranial Rosai–Dorfman disease in a child, a case report and review of the literature

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Abstract

Background

Rosai–Dorfman disease (RDD), otherwise known as sinus histiocytosis with massive lymphadenopathy (SHML), usually affects young adults and commonly presents with massive painless cervical lymphadenopathy. Extranodal disease is present in a third of patients, and it is recognised that this can involve the central nervous system. Intracranial RDD is rare in adults and fewer than 10 paediatric cases have been reported.

Case

A 10-year-old boy with isolated intracranial RDD presents with a painless forehead mass. The management is discussed and the literature reviewed.

Conclusion

This case of isolated intracranial RDD highlights the importance of considering RDD in the differential of paediatric intracranial mass lesions and outlines the diagnostic and treatment challenges faced when managing this rare condition.

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Authors and Affiliations

  1. Department of Neurosurgery, Atkinson Morley Wing, St. George’s Hospital, Blackshaw Road, London, SW17 0QT, UK

    E. C. Maratos & A. J. Martin

  2. Department of Cellular Pathology, St. George’s Hospital, London, UK

    L. R. Bridges

  3. Department of Neuroradiology, Atkinson Morley Wing, St. George’s Hospital, London, UK

    A. D. MacKinnon & J. B. Madigan

  4. Department of Paediatric Oncology, Royal Marsden Hospital, London, UK

    A. Atra

Authors
  1. E. C. Maratos
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  2. L. R. Bridges
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  3. A. D. MacKinnon
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  4. J. B. Madigan
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  5. A. Atra
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  6. A. J. Martin
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Correspondence to E. C. Maratos.

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Maratos, E.C., Bridges, L.R., MacKinnon, A.D. et al. Isolated intracranial Rosai–Dorfman disease in a child, a case report and review of the literature. Childs Nerv Syst 30, 1595–1600 (2014). https://doi.org/10.1007/s00381-014-2437-0

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  • DOI: https://doi.org/10.1007/s00381-014-2437-0

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