On the Treatment of Subependymal Giant Cell Astrocytomas and Associated Hydrocephalus in Tuberous Sclerosis

Abstract

Between 1980 and 1992, 10 children affected by tuberous sclerosis and intraventricular subependymal giant cell astrocytomas were surgically treated at the Institute of Neurosurgery, Section of Pediatric Neurosurgery, Catholic University of Rome. Nine patients presented with signs and/or symptoms of intracranial hypertension; in all of them the neuroradiological investigations demonstrated the presence of a space-occupying lesion in the region of the foramen of Monro with secondary ventricular dilation. In the remaining patient, a 5-month-old male infant, an intraventricular mass was discovered by means of an ultrasound examination performed after the first epileptic fit. Three patients underwent a ventriculoperitoneal CSF shunt as first surgical procedure; in 2 of them it was subsequently necessary to remove the intraventricular tumor due to the frequent occlusion of the CSF shunt device. Seven subjects underwent the direct surgical excision of the lesion. In all of them the procedure resulted in the control of the associated hydrocephalus. On the basis of such an experience, the authors conclude that the surgical removal of the intraventricular tumors in patients with tuberous sclerosis and hydrocephalus is the most appropriate treatment. In fact, in the series considered here, the removal of the tumor was not accompanied by significant morbidity, and was followed by improvement in clinical conditions. In particular, in cases in whom the occurrence of hydrocephalus was associated with a worsening in the seizure disorder, the tumor removal and the correction of intracranial hypertension was followed by a significant reduction in frequency or even by the disappearance of the seizures. However, in no case presenting with mental impairment was a significant improvement observed in mental performances as a consequence of the surgical treatment.

Management of subependymal giant cell tumors in tuberous sclerosis complex: the neurosurgeon’s perspective

“…This CSF proteins level may normalize after resection of the tumor. [10,13] This last fact corroborates the uselessness of ventriculoperitoneal shunt in hydrocephalic children with TSC before the resection of SGCTs. Finally, it is important to keep in mind that even after a complete removal of SGCTs, hydrocephalus can evolve on itself and even lead to postoperative deaths in 10% to 20% of removed SGCTs.…”

“…[7][8][9][10][11] Indeed, while symptomatic SGCTs in children with TSC do not require any debate concerning their surgical management, asymptomatic ones remain more problematic to handle even if a recent more "interventionist" tendency is emanating from the neurosurgical literature. [1,3,9,12,13] Thus, after a brief reminder of the main clinical features of TSC and a compilation of the current data concerning the pathogenesis of TSC and the management of SGCTs, the author tries to clarify the modest role of the neurosurgeon in the multidisciplinary management of SGCTs in children with TSC.…”

“…[3,37] Clinical series showed a mild male predominance, with a mean age at surgery of 11 years. [3,10,13,17] Habitually, these neoplasms evolve on several weeks or months. They are revealed by increased ICP symptoms, epilepsy recrudescence and behavioral modifi cations.…”

Gamma Knife surgery for subependymal giant cell astrocytomas

“…2,16,24,26 Despite recent advances in microsurgical or endoscopic techniques, the surgical treatment for intraventricular tumors can lead to considerable postoperative morbidity, including hemiparesis, 2,5,6,24 visual disturbance, 16 memory deficit, 3 and other cognitive dysfunction. 4 Attempts at total resection usually result in higher complication rates. 23 Because of these risks, several authors have used fractionated radiotherapy to control the tumor as either primary or adjuvant treatment, but none seems to document a positive response to radiotherapy.…”

Congenital subependymal giant cell astrocytomas in patients with tuberous sclerosis complex

“…They are typically located on the surface of the lateral ventricle of the brain, and thus, while growing, they extend into the lateral ventricle and can obstruct the foramen of Monro and flow of CSF, causing hydrocephalus [8, 10]. SEGAs usually grow slowly, and the mean age they present clinical symptoms or cause hydrocephalus is 9.7 years [16, 26, 29, 37].…”