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Intracranial solitary juvenile xanthogranuloma in an infant
Lian-Ping Sun1, Hui-Ming Jin, Bo Yang
1Department of Pediatric Neurosurgery, Xin Hua Hospital Affiliated to Shanghai Jiaotong University, Shanghai, 200092, China. slp1128@126.com
Insights
Juvenile xanthogranuloma (JXG) is a rare histiocyte disorder. Complete surgical removal of intracranial JXG tumors offers a curative outcome for affected children.
Area of Science:
- Pediatric Pathology
- Neuropathology
- Histiocytosis
Background:
- Juvenile xanthogranuloma (JXG) is a rare histiocytic proliferation disorder.
- While typically presenting as solitary cutaneous lesions, systemic JXG involvement is uncommon and associated with significant morbidity.
- Intracranial JXG can be challenging to diagnose preoperatively, potentially leading to misdiagnosis.
Observation:
- A 5-month-old infant presented with an elevated anterior fontanel.
- Brain Magnetic Resonance Imaging (MRI) revealed a large mass in the right parietal region.
- No other significant abnormalities were noted on admission.
Findings:
- Surgical resection of the intracranial mass was performed, including removal of the infiltrated meninges.
- Pathological examination confirmed the diagnosis of Juvenile xanthogranuloma (JXG) in the right parietal region.
- The resected tumor was identified as a JXG.
Implications:
- Complete surgical excision of intracranial JXG may be a curative treatment.
- Maintaining normal vital signs and neurological function during and after surgery is crucial for a favorable outcome.
- Early and accurate diagnosis of intracranial JXG is important for timely and effective management.
Background:
Juvenile xanthogranuloma (JXG) is a disorder of histiocyte proliferation. Most cases present with a solitary cutaneous lesion. JXG with systemic involvement is rare with significant morbidity. Intracranial solitary JXG may be misdiagnosed before operation.
Methods:
A 5-month-old boy showed an elevated anterior fontanel but no other abnormalities on admission. Brain MRI showed a large mass in the right parietal region.
Results:
The tumor was removed with the encroached meninges. A JXG in the right parietal region was diagnosed pathologically.
Conclusion:
Total excision of the tumor may be curative with a prerequisite of ensuring normal vital signs and nervous function.
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