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Cerebral primitive neuroectodermal tumors in childhood.
T Tomita1, D G McLone, M Yasue
1Division of Pediatric Neurosurgery, Children's Memorial Hospital, Chicago, Illinois.
Journal of Neuro-Oncology
|November 1, 1988
Summary
Pediatric cerebral primitive neuroectodermal tumors (PNETs) have a poor prognosis despite multimodal treatment. Aggressive therapy including gross total resection, neuraxis radiation, and chemotherapy is recommended for improved outcomes in children with these rare brain tumors.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Tumor Biology
Background:
- Cerebral primitive neuroectodermal tumors (PNETs) are rare and aggressive brain tumors in children.
- Nosological challenges exist in classifying these undifferentiated neuroepithelial tumors.
Purpose of the Study:
- To review a series of pediatric cerebral PNETs.
- To evaluate treatment outcomes and identify prognostic factors.
Main Methods:
- Computed tomography (CT) detection of 17 pediatric cerebral PNET cases.
- Review of pertinent medical literature.
- Analysis of treatment modalities including surgery, radiation, and chemotherapy.
Main Results:
- Overall poor prognosis for cerebral PNETs, with only 2/17 patients surviving >4 years.
- Histological features and extent of resection did not significantly impact survival.
- Gross total resection improved local control but distant cerebrospinal fluid metastases were common.
Conclusions:
- Cerebral PNETs in children have a dismal prognosis.
- Recommended treatment involves radical gross total resection, postoperative neuraxis radiation, and aggressive chemotherapy.
- Further research is needed to improve survival rates for these challenging tumors.