Early Diagnosis of Subependymal Giant Cell Astrocytoma in Patients With Tuberous Sclerosis

Abstract

We present 19 patients with tuberous sclerosis complex and subependymal giant cell astrocytoma. The mean age at the time of tumor diagnosis was 9.4 years (range, 1.5 to 21 years). Computed cranial tomography (CT) or cranial magnetic resonance imaging (MRI) identified the lesion which was resected in all cases. Seven patients had hydrocephalus and there was an interval increase in the tumor size or a large tumor without hydrocephalus in 12 patients. Surgical criteria included: (1) presence of hydrocephalus; (2) interval increase in tumor size; (3) new focal neurologic deficit attributable to the tumor; and/or (4) symptoms of increased intracranial pressure. Eight patients were identified through a surveillance program involving annual computed cranial tomography. All of these eight patients had their tumor removed prior to the development of symptoms, none had neurologic deficits which persisted after surgery, and none has so far developed recurrent subependymal giant cell astrocytoma. In contrast, of the 11 patients from the non-surveillance group 7 were symptomatic at tumor diagnosis, 1 had a complicated postoperative course, 2 developed recurrent giant cell astrocytoma, and 1 had an extensive lesion that could not be completely excised. Periodic cranial imaging may help to identify subependymal giant cell astrocytomas in tuberous sclerosis patients before they become symptomatic. Earlier diagnosis and treatment could reduce surgical morbidity and the risk of tumor recurrence.

Neurologic manifestations of tuberous sclerosis complex

“…SEGAs tend to grow in childhood and often obstruct the flow of spinal fluid leading to hydrocephalus, and are often located in the region of the foramen of Monro, but not invariably so [7]. An active area of study is how to distinguish these two lesions early [8,9]. Early surgical intervention before hydrocephalus and increased intracranial pressure develop may lead to less morbidity and complications.…”

Advances in the management of subependymal giant cell astrocytoma

“…Furthermore, as SENs are present in nearly all patients with TSC and cannot be radiographically differentiated from SEGAs with confidence, target selection becomes challenging [7]. The application of radiosurgery is further complicated by the fact that irradiation of SEGAs may promote malignant degeneration and development of glioblastoma [20,21]. Although GK-SRS may be beneficial for SEGAs not amenable to surgical resection, at present, there are limited data demonstrating its safety and efficacy.…”

Tuberous sclerosis

“…If fully resected the prognosis is good with a low recurrence risk. 42 These tumours are rarely responsive to radiotherapy and there are reports suggesting that irradiation may induce glioblastomas. For this reason, radiotherapy is contraindicated if the lesion has been completely removed and its use in other situations needs careful consideration.…”

“…There are reports suggesting benefit from regular cranial imaging to screen for SEGAs. 42,43 The expert panel at the TS Consensus Conference in 1998 concluded that early detection and treatment increased the chances of complete excision and recommended CT or MRI scanning every 1 -3 years in children and less often in adults. 35 This has not been universally adopted and more studies are needed to determine the value of screening and the criteria for surgical intervention.…”