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Dysembryoplastic neuroepithelial tumour

M Tatke1, A Sharma, V Malhotra

  • 1Department of Pathology, G. B. Pant Hospital, New Delhi, India.

Child'S Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery
|September 3, 1998
PubMed
Summary

This report details a rare dysembryoplastic neuroepithelial tumor (DNET) in a young Indian boy with complex partial seizures. Surgical resection is the only effective treatment for this uncommon brain tumor.

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Area of Science:

  • Neuro-oncology
  • Pediatric Neurology
  • Neurosurgery

Background:

  • Dysembryoplastic neuroepithelial tumors (DNETs) are rare congenital tumors of the central nervous system (CNS).
  • First described in 1988, DNETs are incorporated into the WHO classification of CNS tumors.
  • Fewer than 100 cases of DNETs have been reported globally.

Observation:

  • A 13-year-old boy presented with intractable partial complex seizures originating from the left frontal lobe, with onset at age 3.
  • The patient was diagnosed with a dysembryoplastic neuroepithelial tumor.
  • This case represents the first reported instance of a DNET in India.

Findings:

  • Dysembryoplastic neuroepithelial tumors are rare brain tumors.
  • Surgical intervention is the primary curative treatment for DNETs.

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  • Chemotherapy and radiotherapy are not indicated for DNETs.

    • Implications:

    • Early recognition and diagnosis of DNETs are crucial for effective management.
    • Accurate histological diagnosis guides treatment decisions, emphasizing surgical resection.
    • This case highlights the importance of reporting rare neurological conditions to expand global understanding and clinical experience.