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Low-grade gliomas in children: single institutional experience in 198 cases
Magda Garzón1, Gemma García-Fructuoso, Mariona Suñol
1Department of Neurosurgery, Hospital Sant Joan de Déu, University of Barcelona, Passeig St. Joan de Déu, 2, 08950, Esplugues de Llobregat, Barcelona, Spain, dendrita07@gmail.com.
Insights
Complete surgical resection and cerebellar tumor location are key for better outcomes in pediatric low-grade gliomas (PLGG). Infants and those needing adjuvant therapy face poorer prognoses.
Area of Science:
- Pediatric Oncology
- Neurosurgery
- Clinical Research
Background:
- Pediatric low-grade gliomas (PLGG) are the most common brain tumors in children.
- Surgical removal is a primary treatment modality for the majority of PLGG cases.
Purpose of the Study:
- To identify prognostic factors influencing outcomes in pediatric low-grade gliomas.
- To evaluate the impact of surgical resection extent and adjuvant therapy on progression-free survival (PFS) and overall survival (OS).
Main Methods:
- Retrospective review of 198 pediatric patients diagnosed with PLGG between 1980 and 2010.
- Analysis of variables including age, tumor location, extent of surgical resection (gross total resection [GTR], subtotal resection [STR], biopsy), adjuvant therapy (AT), and histology.
- Statistical correlation of these factors with PFS and OS.
Main Results:
- Gross total resection (GTR) and subtotal resection (STR) were associated with significantly longer overall survival (OS) compared to biopsy alone.
- Patients younger than 12 months at onset had worse OS.
- Adjuvant therapy (AT) was linked to worse PFS and OS, suggesting its use in cases of tumor progression or recurrence.
Conclusions:
- The extent of tumor resection, particularly GTR, is a critical prognostic factor for pediatric low-grade gliomas.
- Tumor location, specifically cerebellar, and early age at onset (infants) are associated with poorer outcomes.
- Adjuvant therapy indicates a more aggressive disease course and is linked to worse survival rates.
Introduction:
In pediatric population (0-18 years), low-grade gliomas (PLGG) are the most frequent brain tumors and majority are amenable for surgical removal.
Patients And Methods:
A retrospective review of 198 children diagnosed with PLGG between 1980 and 2010 at HSJD was carried out. Several variables were studied to find prognostic factors related to the outcomes (progression-free survival (PFS) and overall survival (OS)).
Results:
Median age at onset was 88.8 months (3.1 to 214.5 months, SD 53). Surgery was performed in 175 patients (88.4%), achieving gross total resection (GTR) in 77 (44%), subtotal resection (STR) in 87 (49.7%), and 11 (6.3%) biopsies. Pathological review classified 84 tumors as WHO grade I (48%) and 89 as grade II (50.8%). Adjuvant therapy (AT) was given to 75 patients (37.9%), radiotherapy in 24 (12.1%), chemotherapy in 33 (16.7%), and combined in 18 (9.1%). Sixteen patients (8.1%) died, 89 (43.4%) are alive with no evidence of disease, and 93 (47%) alive with disease, median follow-up 65.2 months. Outcome is significantly correlated with age (p = 0001, worse OS for patients younger than 12 months) and extent of tumor resection (p < 0001). OS for GTR/STR/biopsy was >200, 154.3, and 101.9 months, respectively. Patients treated with AT presented worse OS/PFS (p < 0.001) than those not treated. Histology was non significantly related to outcomes.
Conclusion:
In our series of PLGG, the best prognostic markers are tumor location (cerebellar) and the extent of tumor resection (GTR). Infants and patients who require adjuvant therapy because of tumor progression or recurrence have worse outcome.
