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.
Abstract

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