Subependymal Giant-Cell Astrocytomasin Pediatric Tuberous Sclerosis Disease

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.