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Pineoblastoma-The Experience at St. Jude Children's Research Hospital
Kara A Parikh1,2, Garrett T Venable2,3, Brent A Orr4
1School of Medicine, Louisiana State University Health Science Center, New Orleans, Louisiana.
Background:
Pineoblastomas are rare, supratentorial, primitive neuroectodermal tumors.
Objective:
To document outcomes with multimodal therapy and evaluate the impact that the degree of surgical resection has on outcome.
Methods:
A departmental brain tumor database was queried to identify all patients with pathologically proven pineoblastoma who were treated from January 1997 to June 2015 at St. Jude Children's Research Hospital. For each patient, we recorded demographic, pathological, radiological, surgical, and clinical follow-up data. The effect of degree of surgical resection on survival outcomes was analyzed.
Results:
Forty-one patients (21 male, 20 female) treated for pineoblastoma were identified. The median age at diagnosis was 5.5 years (range 0.4-28.1) and the median follow-up was 34.5 months. Nineteen patients experienced tumor relapse with a median progression-free survival of 11.3 months, and 18 ultimately succumbed to their disease. Patients who died or experienced treatment failure were younger (median, 2.69 vs 6.5 years, P = .026) and more likely to have metastatic disease at diagnosis (12 [63.2%] vs 5 [22.7%], P = .012). When analyzing only patients 5 years of age or older with focal disease at presentation, those who had a gross total resection or near-total resection-compared with subtotal resection or biopsy-had greater overall survival (75.18 vs 48.57 months), with no patients dying as a result of their cancer.
Conclusion:
Poor prognostic variables for children with pineoblastoma include young age, metastatic disease at presentation, and tumor relapse. For patients older than 5 years with focal disease, maximal tumor resection should be the goal.
Insights
Maximal surgical resection improves survival for children over 5 with focal pineoblastoma. Younger age and metastatic disease at diagnosis are poor prognostic factors for this rare brain tumor.
Area of Science:
- Neuro-oncology
- Pediatric oncology
- Cancer research
Background:
- Pineoblastomas are rare, aggressive, supratentorial primitive neuroectodermal tumors.
- These tumors primarily affect children, posing significant treatment challenges.
Purpose of the Study:
- To document treatment outcomes for pineoblastoma using multimodal therapy.
- To evaluate the impact of surgical resection extent on patient survival.
Main Methods:
- Retrospective analysis of 41 pineoblastoma patients treated between 1997 and 2015.
- Data collected included demographics, pathology, radiology, surgical details, and clinical follow-up.
- Statistical analysis focused on the relationship between surgical resection degree and survival outcomes.
Main Results:
- Younger patients (median 2.69 years) and those with metastatic disease at diagnosis had significantly worse outcomes.
- For patients aged 5 years and older with focal disease, gross total or near-total resection led to improved overall survival (75.18 months) compared to subtotal resection or biopsy (48.57 months).
- No deaths occurred in the maximal resection group for older patients with focal disease.
Conclusions:
- Young age at diagnosis, metastatic disease, and tumor relapse are poor prognostic indicators for pineoblastoma.
- Maximal safe surgical tumor resection should be prioritized for patients over 5 years old with focal pineoblastoma.
- Multimodal therapy remains crucial for managing this rare pediatric brain tumor.
