Related Experiment Videos
Focal tectal tumors: management and prognosis
L A Squires1, J C Allen, R Abbott
1Department of Neurology, New York University Medical Center, NY.
Insights
Midbrain tectal tumors in children often present with hydrocephalus and are managed with ventriculoperitoneal (VP) shunts. This review suggests that most tectal gliomas are benign and may be treated with a VP shunt alone.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Pediatric Neurosurgery
Background:
- Midbrain tectal tumors are rare in children.
- These tumors can cause obstructive hydrocephalus and increased intracranial pressure.
- Tectal gliomas are considered a subset of brainstem gliomas.
Purpose of the Study:
- To review the clinical course and management of pediatric midbrain tectal tumors.
- To evaluate the efficacy of ventriculoperitoneal (VP) shunts in managing these tumors.
- To assess the long-term outcomes and survival rates for children with tectal gliomas.
Main Methods:
- Retrospective chart review of 12 pediatric patients with midbrain tectal tumors over 7 years.
- Analysis of presenting symptoms, imaging findings, and treatment modalities.
- Evaluation of clinical and radiographic progression, treatment responses, and survival data.
Main Results:
- All 12 children presented with increased intracranial pressure and hydrocephalus, treated with VP shunts.
- Three patients showed disease progression; two received radiation and chemotherapy, one received radiation alone.
- All patients remained alive with a median follow-up exceeding 4 years.
- Median progression-free survival was at least 24 months; median overall survival was over 50 months.
Conclusions:
- Pediatric midbrain tectal gliomas represent a relatively benign variant of brainstem glioma.
- Ventriculoperitoneal (VP) shunting is an effective initial management strategy for hydrocephalus in these patients.
- The majority of children with tectal gliomas may be managed successfully with a VP shunt alone, with good long-term prognoses.
Abstract:
A 7-year review at our institution identified 12 children with midbrain tectal tumors. All presented with signs of increased intracranial pressure, had hydrocephalus on initial imaging, and were treated with ventriculoperitoneal (VP) shunts. Three had clinical and radiographic progression of disease. Two were treated with radiation and chemotherapy, with progression of disease in one. The third received radiation alone. All patients are alive, with a median follow-up of over 4 years. Median progression-free survival is at least 24 months and median total survival is beyond 50 months. The tectal glioma syndrome is a relatively benign variant of the brainstem glioma. The majority of patients may be managed with a VP shunt alone.