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Related Experiment Videos

Extradural extension of primitive neuroectodermal tumor--case report

T Kuroiwa1, K Moriwaki, T Ohta

  • 1Department of Neurosurgery, Osaka Medical College.

Neurologia Medico-Chirurgica
|June 1, 1994
PubMed
Summary

Primitive neuroectodermal tumors (PNET) can extend beyond the skull, presenting diagnostic challenges. This case highlights the aggressive nature and complex management of extracranial PNETs.

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Area of Science:

  • Neuro-oncology
  • Pediatric oncology
  • Radiology

Background:

  • Primitive neuroectodermal tumors (PNETs) are rare, aggressive central nervous system malignancies.
  • Extracranial extension of PNETs is uncommon, posing diagnostic and therapeutic challenges.

Observation:

  • A 13-year-old boy presented with a PNET exhibiting unusual extracranial involvement.
  • Imaging revealed a ring-enhancing lesion with a central low-density area suggestive of methemoglobin.
  • Histological examination confirmed PNET.

Findings:

  • The PNET demonstrated characteristic imaging features on CT and MRI, including T1 hypointensity and T2 hyperintensity.
  • The central high signal intensity on both T1 and T2 MRI sequences suggested the presence of free methemoglobin.

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  • Despite subtotal resection, irradiation, and further tumor removal, the patient had a poor prognosis with death at 6 months.

    • Implications:

    • This case underscores the potential for PNETs to manifest with extracranial extension.
    • Multimodality imaging is crucial for characterizing these complex tumors.
    • Management requires careful consideration of both intracranial and extracranial components for optimal patient outcomes.