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Management of posterior fossa gliomas in children
K Sridhar1, R Sridhar, G Venkatprasanna
1Department of Neurosurgery, Fortis Malar Hospital, Chennai, India.
Insights
Pediatric posterior fossa tumors, including cerebellar astrocytomas and brain stem gliomas, are common. Surgical excision is key for survival, with histology not impacting prognosis for these childhood brain tumors.
Area of Science:
- Pediatric neuro-oncology
- Childhood brain tumors
- Posterior fossa tumors
Background:
- Brain tumors are the most common solid tumors in children, with over 50% located in the infratentorial region.
- Cerebellar astrocytomas and brain stem gliomas are the most frequent posterior fossa glial tumors in pediatric patients.
- Low-grade gliomas constitute the majority of cerebellar gliomas.
Purpose of the Study:
- To review the characteristics, presentation, and management of pediatric posterior fossa tumors.
- To highlight the role of surgical excision in the prognosis of these tumors.
- To discuss the classification and histological subtypes of brain stem gliomas.
Main Methods:
- Review of literature on pediatric posterior fossa tumors.
- Analysis of clinical presentation and radiological findings.
- Discussion of treatment modalities, focusing on surgical resection.
Main Results:
- Cerebellar astrocytomas often present with obstructive hydrocephalus and raised intracranial pressure.
- Surgical excision is the primary treatment and a critical factor for progression-free survival and long-term outcomes.
- Brain stem gliomas can be classified into diffuse, focal, exophytic, and cervicomedullary types, with diffuse gliomas being high-grade and malignant.
Conclusions:
- Surgical management is paramount for pediatric posterior fossa tumors, irrespective of tumor histology.
- While cerebellar astrocytomas are often low-grade, brain stem gliomas encompass a spectrum from indolent to malignant lesions.
- Further research into specific subtypes and targeted therapies may improve outcomes for challenging brain stem gliomas.
Abstract:
Brain tumours form the most common type of solid tumour in children and more that 50% of these are infratentorial. Cerebellar astrocytomas and brain stem gliomas are the commonest posterior fossa glial tumours in children. Cerebellar astrocytomas represent up to 10% of all primary brain tumours and up to 25% of posterior fossa tumors in children, with Low grade gliomas forming the commonest of the cerebellar gliomas. They commonly present with symptoms and signs of raised intracranial pressure due to obstructive hydrocephalus. Radiologically they may be solid or cystic with or without a mural nodule. Surgical excision is the mainstay of treatment and forms the most consistent factor influencing progression free and long term survival. While majority of the tumours are pilocytic astrocytomas, they may also be fibrillary astrocytomas or even high grade tumours. Tumour histology does not appear to be an independent factor in the prognosis of these children, and therefore no palliative treatment after surgery is advocated. Brain stem gliomas account for approximately 10% of all pediatric brain tumours. Cranial nerve signs, ataxia and cerebellar signs with or without symptoms and signs of raised intracranial pressure are classically described symptoms and signs. Radiographic findings and clinical correlates can be used to categorize brain stem tumours into four types: diffuse, focal, exophytic and cervicomedullary. Histologically most brain stem gliomas are fibrillary astrocytomas. Diffuse brain stem gliomas are the most commonly seen tumour in the brain stem. These lesions are malignant high grade fibrillary astrocytomas. Focal tumours of the brain stem are demarcated lesions generally less than 2 cms in size, without associated edema. Most commonly seen in the midbrain or medulla, they form a heterogeneous pathological group, showing indolent growth except when the lesion is a PNET. Dorsally exophytic tumours lie in the fourth ventricle, while cervicomedullary lesions are similar to spinal intramedullary tumours. Expanding lesions are the only lesions amenable for excision while infiltrative and ventral lesions are not.
