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Disseminated encephalomyelitis-like central nervous system neoplasm in childhood

Jianhui Zhao1, Xinhua Bao2, Na Fu3

  • 1Department of Pediatrics, Peking University First Hospital, Beijing, China Department of Neurological Rehabilitation, Qingdao Children's Hospital, Qingdao, China.

Journal of Child Neurology
|July 23, 2013
PubMed

Insights

Rare pediatric central nervous system (CNS) tumors with diffuse white matter changes can mimic acute disseminated encephalomyelitis (ADEM). Early biopsy is crucial for accurate diagnosis and appropriate treatment of CNS neoplasms in children.

Area of Science:

  • Pediatric Neurology
  • Neuro-oncology
  • Neuroradiology

Background:

  • Malignant central nervous system (CNS) neoplasms presenting with diffuse white matter changes on magnetic resonance imaging (MRI) are uncommon in pediatric populations.
  • These rare presentations can be misdiagnosed as acute disseminated encephalomyelitis (ADEM), a demyelinating inflammatory condition.
  • Accurate differentiation is critical for appropriate management and prognosis.

Observation:

  • This report details the experience with four pediatric patients (3 male, 1 female; aged 7-13 years) initially diagnosed with ADEM based on MRI findings of diffuse white matter lesions.
  • All patients received corticosteroid therapy, a common treatment for ADEM.
  • Subsequent brain biopsy revealed diverse CNS neoplasms including gliomatosis cerebri, primitive neuroectodermal tumor, and CNS lymphoma.

Findings:

  • Diffuse white matter lesions on pediatric MRI can represent CNS neoplasms, not solely inflammatory processes like ADEM.
  • Initial misdiagnosis of ADEM can lead to delayed or inappropriate treatment with corticosteroids.
  • Histopathological confirmation via brain biopsy is essential for definitive diagnosis of pediatric CNS tumors.

Implications:

  • Highlights the importance of considering CNS neoplasms in the differential diagnosis of pediatric patients with diffuse white matter abnormalities on MRI.
  • Emphasizes the limitations of MRI alone in differentiating neoplastic from inflammatory lesions in this context.
  • Underscores the critical role of prompt neurosurgical consultation and brain biopsy for accurate diagnosis and timely oncological management in children.
Keywords:
acute disseminated encephalomyelitiscentral nervous system lymphomagliomatosis cerebrimagnetic resonance spectroscopyprimitive neuroectodermal tumor

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