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Pediatric cerebellar astrocytoma: a review
Christopher M Bonfield1, Paul Steinbok2
1Division of Neurosurgery, Department of Surgery, BC Children's Hospital and University of British Columbia, 4480 Oak Street, Vancouver, BC, V6H 3 V4, Canada. bonfieldcm@gmail.com.
Insights
Low-grade cerebellar astrocytomas (CA) in children have excellent survival rates, with surgery as the primary treatment. Careful management is crucial to preserve neurologic function for optimal long-term outcomes.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Tumor Biology
Background:
- Cerebellar astrocytomas (CA) are frequent posterior fossa tumors in children.
- Most pediatric CAs are low-grade with a favorable long-term survival prognosis.
Purpose of the Study:
- To provide an updated overview of cerebellar astrocytomas.
- To cover epidemiology, pathology, molecular biology, diagnosis, management, and outcomes.
Main Methods:
- Comprehensive review of recent literature on cerebellar astrocytomas.
- Synthesis of information on various aspects of CA.
Main Results:
- Surgical resection is the primary treatment, aiming for complete tumor removal.
- Subtotal resection can lead to tumor stability or spontaneous regression.
- Adjuvant chemotherapy is for progressive tumors; personalized approaches are emerging.
- Radiotherapy is generally not recommended but may be considered for relapsed/progressive cases.
- Long-term neurologic deficits are common, but quality of life and cognitive function are generally good.
Conclusions:
- Low-grade cerebellar astrocytomas are primarily surgical conditions with high survival rates.
- Preserving neurologic function during surgery and adjuvant therapy is essential for optimal long-term patient outcomes.
Introduction:
Cerebellar astrocytomas (CA) are one of the most common posterior fossa tumors in children. The vast majority is low grade, and prognosis for long-term survival is excellent.
Methods:
Recent literature about CA was reviewed to provide an up to date overview of the epidemiology, pathology, molecular and cell biology, diagnosis, presentation, management, and long-term outcomes.
Results:
Surgical resection remains the first-line treatment with complete removal of the tumor the goal. However, even when only subtotal resection has been achieved, there is a significant chance that the tumor will remain stable or will regress spontaneously. Adjuvant chemotherapy is reserved for those tumors that progress despite surgery, and more personalized chemotherapy is being pursued with better understanding of the molecular genetics of this tumor. Radiotherapy has generally not been recommended, but stereotactic radiotherapy and conformal proton beam radiotherapy may be reasonable options in the setting of relapse or progression. In the long term, permanent neurologic deficits, mainly cerebellar dysfunction, are common, but quality of life and cognitive function are generally good.
Conclusions:
Low-grade CA remains primarily a surgical disease, with excellent survival rates. Care must be taken with surgery and adjuvant treatments to preserve neurologic function to allow for optimal outcomes in the long term.
