Salman AlQazlan1, Abdulrahman Albakr2,3, Abdullah Al Towim4, Yazeed Alsaadan1, Hamdy Hassan5, Khaldoon Aljerian6, Sherif Elwatidy7. 1. College of Medicine, King Saud University, Riyadh, Saudi Arabia. 2. Division of Neurosurgery, Department of Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia. Dr.aalbakr@gmail.com. 3. Division of Neurosurgery, Department of Neurosciences, University of Calgary, Foothills Medical Centre, Calgary, Alberta, Canada. Dr.aalbakr@gmail.com. 4. Department of Neurosurgery, Prince Sultan Military Medical City, Riyadh, Saudi Arabia. 5. Department of Radiology and Medical Imaging, College of Medicine, King Saud University, Riyadh, Saudi Arabia. 6. Department of Pathology, College of Medicine, King Saud University, Riyadh, Saudi Arabia. 7. Division of Neurosurgery, Department of Surgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
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.
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.