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[Intracranial primitive neuroectodermal tumor in an infant: a case report]
K Yoshizato1, S Yoshioka, T Tamai
1Department of Neurosurgery, Oita Prefectural Hospital, Japan.
No Shinkei Geka. Neurological Surgery
|April 6, 1999
Summary
A supratentorial primitive neuroectodermal tumor (PNET) in an infant was successfully removed and treated with chemotherapy. The child remains disease-free after 12 months, suggesting effective treatment for this rare brain tumor.
Area of Science:
- Neuro-oncology
- Pediatric oncology
- Molecular pathology
Background:
- Primitive neuroectodermal tumors (PNETs) are rare and aggressive central nervous system neoplasms.
- Distinguishing between central and peripheral PNETs is crucial for prognosis and treatment, but can be challenging based solely on location.
Observation:
- A 2-month-old infant presented with a supratentorial PNET extending into the skull.
- The tumor exhibited histological features of poorly differentiated neuroectodermal cells.
- Immunohistochemical analysis revealed reactivity for GFAP, synaptophysin, neuron-specific enolase, and vimentin.
Findings:
- A significant finding was moderate immunoreactivity for the HBA71 antigen (p30/32M1C2), a marker typically associated with peripheral PNETs.
- Despite its intracranial location, the tumor's immunophenotype suggests a classification as a peripheral PNET.
- The patient underwent complete tumor resection followed by chemotherapy, achieving a 12-month recurrence-free survival.
Implications:
- This case highlights the potential for intracranial tumors to exhibit characteristics of peripheral PNETs.
- The HBA71 antigen may serve as a valuable diagnostic marker in differentiating PNET subtypes, even in unusual locations.
- Successful management with surgery and chemotherapy offers hope for similar pediatric cases.