Subependymal Giant Cell Astrocytomas in Children

Abstract

Between 1977 and 1991, at the Children’s Hospital of Philadelphia, 10 patients, 5–16 years of age, were diagnosed as having subependymal giant cell astrocytomas. These patients accounted for 1.4% of all pediatric brain tumors seen during that time interval. One patient received a course of radiation therapy, which was ineffective in preventing tumor growth. All underwent surgical resections with the goal being maximal tumor debulking, if not complete resection. In 6 patients this was accomplished by the frontal transventricular route and, in the more recent patients, surgery was performed using a transcallosal approach. There were 2 perioperative deaths, and 2 other patients died later of causes unrelated to tumor progression. The remaining 6 patients remain alive and stable at a mean of 6.7 years of follow-up (range 1.8–12.4). None of these patients has received additional radiation therapy. Two patients have no other evidence of tuberous sclerosis. The use of modern radiographic and surgical techniques has made the treatment of this disease safer than in the past.

Surgical timing of the subependymal giant cell astrocytoma (sega) with the patients of tuberous sclerosis complex. .

“…MRI should be performed at the time of diagnosis of tuberous sclerosis When there are nodules located near the foramen of Monro and if they measure>5 mm in diameter, are not or incompletely calcified, and are enhanced by gadolinium, MRI should be repeated every year until the age of 15 years. (1,3,5,9,11,17,22,27,31,32,34). In our study, subependymal nodule was the most frequent symptom detected in the cranial imaging.…”

“…They are associated with TSC and believed to originate from subependymal TS nodules. SEGAs are clinically significant because enlargement of these lesions may obstruct the flow of cerebrospinal fluid through the foramen of monro, causing obstructive hydrocephalus (5,6,13,31).…”

Rapid Regrowth of Solitary Subependymal Giant Cell Astrocytoma. Case Report.

“…However, mitoses, necrosis, or vascular proliferation in SEGA do not suggest a worse prognosis. 3,5,8,16,17) The MIB-1 labeling index of the specimen from the second operation was less than 1%. Therefore, no adjuvant therapy, such as radiotherapy or chemotherapy, was given after the total removal.…”

“…15) The present and several other cases of SEGA without any obvious features of TS 1,[3][4][5][6]14,17,19) have been described as à forme fruste' of TS, 5,12,16) but without the genetic studies, whether these neoplasms represent a`forme fruste' of TS or a spontaneous tumor unrelated to the genetic abnormality that causes TS remains unclear. 1,17) The recent identification of mutations in two tuberous sclerosis complex (TSC) genes (TSC1 and TSC2) that cause TS has led to rapid progress in understanding the molecular and cellular pathogenesis of this disorder. TSC1 (chromosome 9q34) and TSC2 (chromosome 16p13) encode distinct proteins, hamartin and tuberin, respectively, which are widely expressed in the brain and may interact as part of a cascade pathway that modulates cellular differentiation, tumor suppression, and intracellular signaling.…”

“…13) Whether SEGA occurs in the absence of TS remains unclear, but several cases of SEGA without any obvious features of TS have been reported. 1,[3][4][5][6]14,17,19) SEGA is thought to be slowly progressive. 11,16) We describe here a rare case of SEGA without TS which rapidly regrew one year after partial removal.…”