Pontine Rosai-Dorfman disease in a child

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• 作者：Ali Varan ; Hilal ?en ; Nejat Akalan ; Kader K. O?uz ; Arzu Sa?lam…
• 关键词：Pons ; Rosai ; Dorfman disease ; Treatment ; Surgery
• 刊名：Child's Nervous System
• 出版年：2015
• 出版时间：June 2015
• 年：2015
• 卷：31
• 期：6
• 页码：971-975
• 全文大小：6,609 KB
• 参考文献：1.Rosai J, Dorfman RF (1969) Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathological entity. Arch Pathol 87:63-0PubMed
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  16.Sharma MS, Padua MD, Jha AN (2005) Rosai-Dorfman disease mimicking a sphenoid wing meningioma. Neurol India 53:110-11View Article PubMed
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• 作者单位：Ali Varan (1)
  Hilal ?en (1)
  Nejat Akalan (2)
  Kader K. O?uz (3)
  Arzu Sa?lam (4)
  Canan Akyüz (1)
  
  1. Department of Pediatric Oncology, Cancer Institute, Hacettepe University, 06100, Ankara, Turkey
  2. Department of Neurosurgery, Faculty of Medicine, Ac?badem University, Ankara, Turkey
  3. Department of Radiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
  4. Department of Pathology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
  
• 刊物类别：Medicine
• 刊物主题：Medicine & Public Health
  Neurosurgery
  Neurosciences
  
• 出版者：Springer Berlin / Heidelberg
• ISSN：1433-0350

文摘

Introduction Rosai-Dorfman Disease (RDD) is a benign histiocytic disorder that commonly presents with massive lymphadenopathy. Central nervous system (CNS) involvement is only 5?%; isolated CNS involvement is much rarer. The patient presented here has been diagnosed with isolated pontine RDD. Case report A 5-year-old child was admitted to the hospital with the history of double vision and ptosis of the left eye. Magnetic resonance imaging revealed presence of a mass at the pontomesencephalic junction. Subtotal tumor excision was performed. Microscopic examination of the lesion showed histologic features consistent with RDD. The patient was followed up without medication, with a small residue. Conclusion RDD is rare in childhood, and isolated CNS involvement is also quite rare. The tumor is located in pons in our case. The treatment of choice is gross total resection. Even total excision is not possible, follow up without medication may be an option because of the indolent and self-limited course of the disease.