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Endoscopic Third Ventriculostomy and Pineal Biopsy from a Single Entry Point
Published on: June 28, 2024
Pineal region tumors in children
Girish Dhall1, Soumen Khatua, Jonathan L Finlay
1Division of Pediatric Hematology-Oncology at Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, California 90027, USA. gdhall@chla.usc.edu
Purpose Of Review:
Pineal tumors are rare in children, with pineoblastoma and germ cell tumors (GCTs) being the most common. Here we discuss recent advances in treatment and controversies in the management of these tumors.
Recent Findings:
There is significant heterogeneity in the clinical behavior of pineoblastoma in children. We will discuss differences in outcome of children with pineoblastoma who are less than and greater than 3 years of age, and between pineoblastoma and nonpineal supratentorial primitive neuro-ectodermal tumors when treated with multiple different strategies. Significant controversies exist in the treatment of GCTs as well, including the levels of tumor markers in the blood and cerebrospinal fluid that are required to establish without biopsy the diagnosis of a GCT, the role of surgery in GCTs and the optimal treatment for germinomas as well as mixed malignant GCTs.
Summary:
Although pineoblastoma in infants and very young children still remains a therapeutic challenge, significant progress has been made in the treatment of pineal GCTs with treatment strategies using a combination of chemotherapy and reduced dose and volume irradiation, resulting in increased survival rates and reduced long-term morbidity.
Insights
Pediatric pineal tumors like pineoblastoma and germ cell tumors (GCTs) show varied outcomes. Advances in chemotherapy and irradiation improve survival for GCTs, though pineoblastoma in young children remains challenging.
Area of Science:
- Pediatric oncology
- Neuro-oncology
- Tumor biology
Background:
- Pineal tumors are rare in children.
- Pineoblastoma and germ cell tumors (GCTs) are the most common types.
- Management strategies and treatment outcomes are areas of active research.
Purpose of the Study:
- To review recent advances in the treatment of pediatric pineal tumors.
- To discuss controversies in the management of pineoblastoma and GCTs.
- To highlight differences in outcomes based on age and tumor type.
Main Methods:
- Review of current literature on pediatric pineal tumors.
- Analysis of treatment strategies for pineoblastoma and GCTs.
- Discussion of diagnostic controversies and therapeutic approaches.
Main Results:
- Pineoblastoma exhibits heterogeneous clinical behavior, with age-dependent outcomes.
- Controversies in GCT management include diagnostic criteria, role of surgery, and optimal treatment for specific subtypes.
- Treatment strategies for GCTs involving chemotherapy and reduced-dose irradiation show promise.
Conclusions:
- Pineoblastoma in infants and young children presents a therapeutic challenge.
- Significant progress has been made in treating pineal GCTs, improving survival rates.
- Combined chemotherapy and irradiation reduce long-term morbidity in pediatric GCT patients.
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