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CC BY 4.0 · Journal of Child Science 2019; 09(01): e7-e10
DOI: 10.1055/s-0039-1683452
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Isolated Intramedullary Spinal Rosai–Dorfman Disease in a Child: A Case Report

Amgad Mohamed Abdelhady Moussa
1   Department of Radiodiagnosis, Faculty of Medicine, Ain Shams University, Cairo, Egypt
,
Mohamed Ismail Degheidy
1   Department of Radiodiagnosis, Faculty of Medicine, Ain Shams University, Cairo, Egypt
,
Noha Mohamed Osman
1   Department of Radiodiagnosis, Faculty of Medicine, Ain Shams University, Cairo, Egypt
,
Sara Makkeyah
2   Department of Pediatric, Hematology-Oncology Unit, Faculty of Medicine, Ain Shams University, Cairo, Egypt
,
Shaimaa Abdel Sattar
1   Department of Radiodiagnosis, Faculty of Medicine, Ain Shams University, Cairo, Egypt
› Author Affiliations
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Publication History

02 November 2018

28 January 2019

Publication Date:
15 March 2019 (online)

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Abstract

Rosai–Dorfman disease (RDD) is an uncommon benign histiocytic proliferative disorder commonly involving the cervical lymph nodes and, less frequently, extranodal sites. The histological hallmark of RDD is emperipolesis displayed by lesional histiocytes. Central nervous system involvement is rare and usually intracranial; intramedullary spinal involvement is even less common and, to our knowledge, rarely reported in children. Herein, we report a case of RDD with isolated intramedullary spinal involvement in a child, which, to our knowledge, is the first case reported in an infant.

Keywords

Rosai-Dorfman disease - histiocytic disorder - intramedullary spinal cord masses