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Aggressive surgical management of recurrent intracranial juvenile xanthogranuloma: case report and review of the literature

  • Salman AlQazlan
  • Abdulrahman AlbakrEmail author
  • Abdullah Al Towim
  • Yazeed Alsaadan
  • Hamdy Hassan
  • Khaldoon Aljerian
  • Sherif Elwatidy
Case Report

Abstract

Introduction

Juvenile xanthogranuloma (JXG) with the central nervous system (CNS) involvement is a rare disease entity that remains poorly understood, especially when the condition develops following treatment for Langerhans cell histiocytosis (LCH).

Case report

A 21-year-old man who was diagnosed with LCH at age 2, several years following which he developed signs and symptoms of CNS involvement. Magnetic resonance imaging (MRI) of the brain revealed JXG with bilateral choroid plexus involvement. As radiation therapy for the intraventricular masses proved unsuccessful, he underwent two surgical resections. In the following years, he developed another large JXG in the meninges, which was managed conservatively until he required surgery due to symptom progression. Twelve years after the first surgery, the patient is in stable condition with no evidence of recurrence.

Conclusion

Due to the rarity of JXG in the CNS, optimal treatment strategies and the precise duration of therapy remain to be determined. Future studies should aim to develop an appropriate treatment algorithm for such rare cases.

Keywords

Juvenile xanthogranuloma Langerhans cell histiocytosis Dura Choroid plexus Central nervous system 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflicts of interest.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.College of MedicineKing Saud UniversityRiyadhSaudi Arabia
  2. 2.Division of Neurosurgery, Department of Surgery, College of MedicineKing Saud UniversityRiyadhSaudi Arabia
  3. 3.Division of Neurosurgery, Department of NeurosciencesUniversity of CalgaryCalgaryCanada
  4. 4.Department of NeurosurgeryPrince Sultan Military Medical CityRiyadhSaudi Arabia
  5. 5.Department of Radiology and Medical Imaging, College of MedicineKing Saud UniversityRiyadhSaudi Arabia
  6. 6.Department of Pathology, College of MedicineKing Saud UniversityRiyadhSaudi Arabia

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