Predictors of tumor progression among children with gangliogliomas (original) (raw)
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Clinical article
Mostafa El Khashab
Mostafa El Khashab Department of Neurosurgery, Advanced Neurosurgery Associates, Hackensack University Medical Center, New Jersey;
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M.D., Ph.D.
,
Lynn Gargan
Lynn Gargan Departments of Neuro-Oncology and
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Ph.D.
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Linda Margraf
Linda Margraf Departments of Pathology,
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Farideh Nejat
Farideh Nejat Department of Neurosurgery, Children's Hospital Medical Center, Medical Sciences/University of Tehran, Iran
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M.D., M.P.H.
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Dale Swift
Dale Swift Pediatric Neurosurgery, Children's Medical Center, Dallas;
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Bradley Weprin
Bradley Weprin Pediatric Neurosurgery, Children's Medical Center, Dallas;
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Daniel C. Bowers
Daniel C. Bowers Pediatrics, University of Texas Southwestern Medical School at Dallas, Texas; and
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Object
Few reports describe the outcome and prognostic factors for children with gangliogliomas. The objective of this report was to describe the progression-free survival (PFS) for children with low-grade gangliogliomas and identify risk factors for tumor progression.
Methods
A retrospective study was performed in children with low-grade gangliogliomas who were evaluated and treated in the neuro-oncology department between 1986 and 2006 to determine risk factors for subsequent tumor progression.
Results
A total of 38 children with newly diagnosed gangliogliomas were included in this report. Thirty-four children were treated with surgery alone, 3 with subtotal resection and radiation therapy, and 1 with subtotal resection and chemotherapy. The follow-up ranged from 4 months to 15.8 years (mean 5.7 ± 4.2 years [± SD]). Seven children have experienced tumor progression, and 1 child died after his tumor subsequently underwent malignant transformation. The 5-year PFS was calculated to be 81.2% using Kaplan-Meier survival analysis. Initial presentation with seizures (p = 0.004), tumor location in the cerebral hemisphere (p = 0.020), and complete tumor resection (p = 0.035) were associated with prolonged PFS. Further analysis of the above significant variables by a Cox regression model identified initial presentation with seizures as being associated with prolonged PFS (p = 0.028).
Conclusions
The PFS and overall survival of children with gangliogliomas are good. Tumors located in the cerebral hemispheres, the achievement of total resection, and seizures at presentation were associated with prolonged PFS. Cox regression analysis identified presenting symptoms including seizures as significant predictive factors of PFS. Prospective studies with larger numbers of children are needed to define the significant factors of tumor progression.
Abbreviations used in this paper:
DIG = desmoplastic infantile ganglioglioma ; GTR = gross-total resection ; PFS = progression-free survival .