Download PDF
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
Further Information

Publication History

02 November 2018

28 January 2019

Publication Date:
15 March 2019 (online)

   Permissions and Reprints

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
 

  • References

  • 1 Sciacca S, Barkas K, Heptinstall L, McNamara C, Shetty R. Rosai-Dorfman disease with spinal cord compression: a diagnostic challenge. Eur Spine J 2015; 24 (Suppl. 04) S529-S535
    CrossrefPubMedGoogle Scholar
  • 2 Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 1969; 87 (01) 63-70
    PubMedGoogle Scholar
  • 3 Gaitonde S. Multifocal, extranodal sinus histiocytosis with massive lymphadenopathy: an overview. Arch Pathol Lab Med 2007; 131 (07) 1117-1121
    CrossrefPubMedGoogle Scholar
  • 4 Destombes P. [Adenitis with lipid excess, in children or young adults, seen in the Antilles and in Mali. (4 cases)]. Bull Soc Pathol Exot 1965; 58 (06) 1169-1175
    PubMedGoogle Scholar
  • 5 Kozak B, Talbott J, Uzelac A, Rehani B. Rosai-Dorfman disease isolated to the thoracic epidural spine. J Radiol Case Rep 2015; 9 (11) 6-16
    PubMedGoogle Scholar
  • 6 Liu G, Wang H, Yang Z, Tang T, Zhang S. Is it a metastatic disease: a case report and new understanding of Rosai-Dorfman disease?. Am J Dermatopathol 2016; 38 (06) e72-e76
    CrossrefPubMedGoogle Scholar
  • 7 Huang BY, Liu HL, Yu CJ. Isolated intramedullary spinal Rosai-Dorfman disease: a case report and literature review. World Neurosurg 2016; 88: 694.e11-694.e15
    CrossrefPubMedGoogle Scholar
  • 8 Sandoval-Sus JD, Sandoval-Leon AC, Chapman JR. , et al. Rosai-Dorfman disease of the central nervous system: report of 6 cases and review of the literature. Medicine (Baltimore) 2014; 93 (03) 165-175
    PubMedGoogle Scholar
  • 9 Destombes P, Destombes M, Martin L. [Pseudotumoral lymph node lipidic histiocytosis. Further case in a young Martinique woman]. Bull Soc Pathol Exot 1972; 65 (03) 481-488
    PubMedGoogle Scholar
  • 10 Vaiselbuh SR, Bryceson YT, Allen CE, Whitlock JA, Abla O. Updates on histiocytic disorders. Pediatr Blood Cancer 2014; 61 (07) 1329-1335
    CrossrefPubMedGoogle Scholar
  • 11 Rocha-Maguey J, Felix-Torrontegui JA, Cabrera-López M, Gutiérrez-Castro M, Montante-Montes de Oca D. A new case of cervical intramedullary sinus histiocytosis causing paraplegia and review of the literature. Surg Neurol Int 2016; 7: 9
    CrossrefPubMedGoogle Scholar
  • 12 Yao K, Li TF, Zhu MW. , et al. An intramedullary cervical cord lesion in a 12-year-old girl. Neuropathology 2013; 33 (05) 582-585
    PubMedGoogle Scholar
  • 13 Huang BY, Zhang H, Zong WJ, Sun YH. Rosai-Dorfman disease of rare isolated spinal involvement: report of 4 cases and literature review. World Neurosurg 2016; 85: 367.e11-367.e16
    CrossrefPubMedGoogle Scholar
  • 14 di Dio F, Mariotti I, Coccolini E, Bruzzi P, Predieri B, Iughetti L. Unusual presentation of Rosai-Dorfman disease in a 14-month-old Italian child: a case report and review of the literature. BMC Pediatr 2016; 16: 62
    CrossrefPubMedGoogle Scholar
  • 15 Vadivelu S, Mangano FT, Miller CR, Leonard JR. Multifocal Langerhans cell histiocytosis of the pediatric spine: a case report and literature review. Childs Nerv Syst 2007; 23 (01) 127-131
    PubMedGoogle Scholar
  • 16 Badalian-Very G, Vergilio JA, Degar BA. , et al. Recurrent BRAF mutations in Langerhans cell histiocytosis. Blood 2010; 116 (11) 1919-1923
    CrossrefPubMedGoogle Scholar
  • 17 Emile JF, Abla O, Fraitag S. , et al; Histiocyte Society. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood 2016; 127 (22) 2672-2681
    CrossrefPubMedGoogle Scholar
  • 18 Dalia S, Sagatys E, Sokol L, Kubal T. Rosai-Dorfman disease: tumor biology, clinical features, pathology, and treatment. Cancer Contr 2014; 21 (04) 322-327
    PubMedGoogle Scholar
  • 19 Garces S, Medeiros LJ, Patel KP. , et al. Mutually exclusive recurrent KRAS and MAP2K1 mutations in Rosai-Dorfman disease. Mod Pathol 2017; 30 (10) 1367-1377
    PubMedGoogle Scholar
  • 20 El Molla M, Mahasneh T, Holmes SE, Al-Khawaja D. Rare presentation of Rosai-Dorfman disease mimicking a cervical intramedullary spinal cord tumor. World Neurosurg 2014; 81 (02) 442.e7-442.e9
    CrossrefPubMedGoogle Scholar