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Clinical characteristics, late effects and outcomes in pineoblastomas in children: a single center experience
Ömer Görgün1, Başak Koç1, Rejin Kebudi1
1Division of Pediatric Hematology-Oncology, İstanbul University Oncology Institute, İstanbul, Turkey.
Insights
Pineoblastomas are rare pediatric brain tumors lacking a standard treatment. This study analyzed outcomes, revealing aggressive tumor behavior and the need for improved therapeutic strategies and long-term survivor monitoring.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Central Nervous System Tumors
Background:
- Pineoblastomas are rare, aggressive central nervous system tumors predominantly affecting children.
- Current standard of care for pineoblastomas lacks consensus.
- This study aimed to analyze treatment outcomes in pediatric pineoblastoma patients.
Purpose of the Study:
- To retrospectively evaluate the clinical outcomes of children diagnosed with pineoblastoma.
- To identify demographic, diagnostic, and treatment-related factors influencing survival.
- To inform the development of more effective treatment strategies for this rare tumor.
Main Methods:
- Retrospective analysis of six pediatric patients diagnosed with pineoblastoma between 1990 and 2012.
- Data collected included demographics, age at diagnosis, presenting symptoms, tumor location, diagnostic methods, metastasis, treatment regimens, and survival status.
- All patients received chemotherapy and craniospinal irradiation (CSI) post-diagnosis, with three undergoing subtotal resection.
Main Results:
- Median follow-up was 5.5 years (range: 1-19 years).
- Two patients achieved no evidence of disease survival at 7.5 and 10 years, respectively.
- One long-term survivor developed a secondary malignancy (papillary thyroid carcinoma) 9.5 years post-treatment.
Conclusions:
- Pineoblastomas are highly aggressive pediatric tumors requiring more effective treatment approaches.
- Long-term survivors necessitate vigilant follow-up for potential late effects, including secondary malignancies and endocrine deficiencies.
Background:
Pineoblastomas (PB) are rare tumors of the central nervous system and are more common in children. There is no consensus about standard of care. The objective of this study is to analyze the outcome of children with PB.
Methods:
Six patients with PB who were diagnosed between 1990-2012 were evaluated retrospectively. Demographics, age of diagnosis, first complaint, tumor region, diagnosis type, seeding metastasis to the spinal axis or cerebrospinal fluid (CSF), treatment and survival of these patients were recorded.
Results:
Three patients had subtotal resection and all patients received chemotherapy and craniospinal irradiation (CSI) after diagnosis. Median follow-up after treatment was 5.5 (range:1-19) years. Two patients are alive with no evidence of disease for 7.5 and 10 years, one of whom was diagnosed with papillary thyroid carcinoma 9.5 years after treatment. One of the patients who died had lived for 19 years after diagnosis.
Conclusions:
Pineoblastomas are rare but very aggressive tumors; more effective treatment strategies are needed. Survivors should be followed up for late effects such as second malignancies and endocrine deficiencies.
