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A 5-year-old child presenting with tumor-like primary angiitis of the central nervous system
Xiuwei Zhuo1, Weixing Feng1, Ji Zhou1
1Department of Neurology Beijing Children's Hospital Capital Medical University National Center for Children's Health Beijing China.
Insights
Primary angiitis of the central nervous system (PACNS) can mimic brain tumors in children. Early diagnosis and immunotherapy for this rare condition improve outcomes and avoid surgery.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Vascular Neurology
Background:
- Primary angiitis of the central nervous system (PACNS) is a rare vasculitis affecting only the brain and spinal cord.
- Tumor-like presentations of PACNS are uncommon, particularly in pediatric cases.
Observation:
- A 5-year-old girl presented with intermittent headaches and a space-occupying lesion on brain imaging.
- Stereotactic brain biopsy confirmed PACNS with a lymphocytic pattern.
Findings:
- The patient's condition significantly improved following immunotherapy.
- This case highlights a rare pediatric presentation of PACNS.
Implications:
- Pediatricians should consider PACNS in children with intracranial tumor-like lesions.
- Timely diagnosis and immunotherapy can lead to better long-term prognoses and potentially prevent surgical intervention for tumor-like PACNS.
Introduction:
Primary angiitis of the central nervous system (PACNS) is a vasculitis confined to the CNS. A small proportion of the lesions may present as a tumor-like mass, which is rarely seen in children.
Case Presentation:
A 5-year-old girl was admitted to our hospital because of an intermittent headache. Brain imaging suggested a space-occupying lesion in the right cerebral hemisphere. The final diagnosis was PACNS with a lymphocytic pattern by stereotactic brain biopsy. Her condition improved after immunotherapy.
Conclusion:
Pediatricians should consider the possibility of PACNS when encountering intracranial tumor-like lesions. Early diagnosis of tumor-like PACNS and prompt immunotherapy could improve the long-term prognosis and avoid surgery.
