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Subependymal giant-cell astrocytomas in pediatric tuberous sclerosis disease: when should we operate?
Sandrine de Ribaupierre1, Georg Dorfmüller, Christine Bulteau
1Division of Pediatric Neurosurgery, Fondation Ophtalmologique Adolphe de Rothschild, Paris, France.
Insights
Subependymal giant-cell astrocytomas in tuberous sclerosis patients have a good prognosis with complete surgical removal. Early surgical intervention is recommended for lesions showing clear evidence of growth to prevent complications.
Area of Science:
- Neuro-oncology
- Pediatric Neurosurgery
- Genetics
Background:
- Subependymal giant-cell astrocytomas (SEGAs) are tumors associated with tuberous sclerosis.
- Early diagnosis and management are crucial due to potential morbidity and mortality.
- The optimal timing for surgical intervention in pediatric SEGAs remains debated.
Purpose of the Study:
- To analyze surgical outcomes for pediatric subependymal giant-cell astrocytomas.
- To establish consensus on the benefits of early versus late surgical intervention.
- To evaluate prognostic factors influencing SEGA management.
Main Methods:
- Retrospective review of 19 pediatric patients with intraventricular tumors.
- Analysis of published pediatric SEGA case series from 1980 to 2006.
- Comparison of outcomes based on surgical timing and tumor characteristics.
Main Results:
- Macroscopically total resection of SEGAs is associated with a good prognosis.
- Residual lesions may enlarge, necessitating careful follow-up due to late recurrences.
- Larger or symptomatic SEGAs present higher morbidity risks.
Conclusions:
- SEGAs meeting specific criteria (near foramen of Monro, >5mm, incomplete calcifications) warrant surgical removal.
- Intervention should occur upon confirmed evidence of tumor growth.
- Proactive surgical management of growing SEGAs is advised.
Objective:
A small percentage of tuberous sclerosis patients will develop a subependymal giant-cell astrocytoma. Given the morbidity and mortality when such a lesion is left undiagnosed, successive follow-up imaging in pediatric patients has been recommended. Surgical removal of the lesion has become the procedure of choice; however, the timing of this surgery is still a controversial subject. By analyzing our own series of data, as well as other published series, we have attempted to reach a consensus on the benefits of early versus late surgery.
Methods:
We retrospectively reviewed 19 patients treated surgically for intraventricular tumors in Foch Hospital and at the Fondation Adolphe de Rothschild in Paris, France, and we analyzed published pediatric reports from 1980 to 2006.
Results:
The results from our own population, as well as from other published pediatric series (15 series), indicate that subependymal giant-cell astrocytomas have a good prognosis when a macroscopically total resection has been performed. In our series, residual lesions tended to enlarge, but residual tumors remaining stable have been reported. Careful follow-up examination should be undertaken because late recurrences do occur. Larger or symptomatic lesions tend to have a higher morbidity.
Conclusion:
We think that any lesion fulfilling the criteria for a subependymal giant-cell astrocytoma as previously described in the literature (lesion around the foramen of Monro, greater than 5 mm, with incomplete calcifications) should be removed as soon as clear evidence of growth has been confirmed.
