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Adult supratentorial extra-pineal primitive neuro-ectodermal tumors
Yun-Cong Zheng1, Shih-Ming Jung2, Shih-Tseng Lee1
1Department of Neurosurgery, Chang Gung Memorial Hospital, Linkuo, School of Medicine, Chang Gung University, No. 5, Fu-hsin Street, Kweishan, Taoyuan 333, Taiwan.
Summary
Extra-pineal supratentorial primitive neuro-ectodermal tumors (PNET) in adults are rare. This study identified multiplicity, CSF seeding, and tumor differentiation as negative prognostic factors for these brain tumors.
Area of Science:
- Neuro-oncology
- Pediatric and Adult Oncology
- Radiology
Background:
- Primitive neuro-ectodermal tumors (PNET) are rare, undifferentiated malignant brain tumors.
- Extra-pineal supratentorial PNET in adults represent a particularly uncommon presentation.
- Lack of established guidelines before 2011 led to varied management at the study institute.
Purpose of the Study:
- To review clinical profiles and radiologic images of adult patients with extra-pineal supratentorial PNET.
- To identify prognostic factors influencing survival in this rare patient population.
- To provide insights for future research and clinical management.
Main Methods:
- Retrospective review of 22 consecutive adult patients diagnosed between 1994-2008.
- Evaluation of clinical data and radiological imaging.
- Pathological review based on 2007 WHO criteria and survival analysis.
Main Results:
- Overall survival rates at 1 and 3 years were 64% and 32%, respectively.
- Negative prognostic factors identified: multiplicity at onset, initial cerebrospinal fluid (CSF) seeding, and tumor differentiation.
- Age, extent of resection, radiation, and chemotherapy did not show strong significance for overall survival in analysis.
Conclusions:
- Multiplicity, CSF seeding, and tumor differentiation are suggested negative prognostic factors for adult extra-pineal supratentorial PNET.
- Further research into the genetic/molecular basis of these tumors is warranted.
- Detailed investigations are needed to improve understanding and clinical outcomes.
