title: Biological behavior and tumorigenesis of subependymal giant cell astrocytomas.
abstract: In spite of the benign nature of subependymal giant cell astrocytomas (SEGAs), some show massive hemorrhage, rapid growth, and tumor recurrence. This led us to investigate the biological behavior, cell dynamics, and tumorigenesis of SEGAs. All patients (4 men and 3 women; age range, 6-27 years; mean, 13.6 years) had features of tuberous sclerosis complex and obstructive hydrocephalus. One patient had intratumoral bleeding. In two patients, sequential neuroimaging showed a subependymal nodule growing to become a SEGA. All underwent surgical resection without radiation therapy. One tumor recurred and was treated surgically. There were no postoperative deaths. The presence of cytologic atypia, mitoses and vascular proliferation had no implication in terms of the clinical course. MIB-1 labeling indices were low (mean, 0.9), indicating low proliferative potential. Unexpectedly, bcl-2 staining was sparse and bax staining predominated in majority of cases. However, the mean value of terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling index was low. Immunohistochemically, tumors were positive for both glial and neuronal markers. In the majority of our cases, the expression of p53 was low. Only one tumor was focally positive for tuberin. SEGAs have low proliferative potential and apoptotic activity, and exhibit features of mixed glial-neuronal differentiation. In contrast to p53, tuberin is suggested to be the tumor suppressor in this tumor.
authors.affiliation: Department of Neurosurgery, Seoul National University Hospital, Chongno-gu, Korea.
authors.forename: S K
authors.initials: SK
authors.lastname: Kim
authors.forename: K C
authors.initials: KC
authors.lastname: Wang
authors.forename: B K
authors.initials: BK
authors.lastname: Cho
authors.forename: H W
authors.initials: HW
authors.lastname: Jung
authors.forename: Y J
authors.initials: YJ
authors.lastname: Lee
authors.forename: Y S
authors.initials: YS
authors.lastname: Chung
authors.forename: J Y
authors.initials: JY
authors.lastname: Lee
authors.forename: S H
authors.initials: SH
authors.lastname: Park
authors.forename: Y M
authors.initials: YM
authors.lastname: Kim
authors.forename: G
authors.initials: G
authors.lastname: Choe
authors.forename: J G
authors.initials: JG
authors.lastname: Chi
mesh_terms: D000328:Adult; D034961:Antigens, Nuclear; D001254:Astrocytoma / Q000737:chemistry / Q000150:complications / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D014408:Biomarkers, Tumor / Q000032:analysis; D001932:Brain Neoplasms / Q000737:chemistry / Q000150:complications / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D002454:Cell Differentiation; D002543:Cerebral Hemorrhage / Q000209:etiology; D002648:Child; D004253:DNA Nucleotidylexotransferase / Q000032:analysis; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000032:analysis; D006261:Headache / Q000209:etiology; D006801:Humans; D006849:Hydrocephalus / Q000209:etiology; D020287:In Situ Nick-End Labeling; D019394:Ki-67 Antigen; D008297:Male; D009363:Neoplasm Proteins / Q000032:analysis; D009364:Neoplasm Recurrence, Local; D009419:Nerve Tissue Proteins / Q000032:analysis; D019006:Neural Cell Adhesion Molecules / Q000032:analysis; D009687:Nuclear Proteins / Q000032:analysis; D010751:Phosphopyruvate Hydratase / Q000032:analysis; D011518:Proto-Oncogene Proteins / Q000032:analysis; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000032:analysis; D012097:Repressor Proteins / Q000032:analysis; D012640:Seizures / Q000209:etiology; D014402:Tuberous Sclerosis / Q000150:complications / Q000235:genetics; D000077005:Tuberous Sclerosis Complex 2 Protein; D016159:Tumor Suppressor Protein p53 / Q000032:analysis; D025521:Tumor Suppressor Proteins; D051028:bcl-2-Associated X Protein
chemical_list: D034961:Antigens, Nuclear; C494826:BAX protein, human; D014408:Biomarkers, Tumor; D005904:Glial Fibrillary Acidic Protein; D019394:Ki-67 Antigen; D009363:Neoplasm Proteins; D009419:Nerve Tissue Proteins; D019006:Neural Cell Adhesion Molecules; D009687:Nuclear Proteins; D011518:Proto-Oncogene Proteins; D019253:Proto-Oncogene Proteins c-bcl-2; D012097:Repressor Proteins; C000624653:TSC2 protein, human; D000077005:Tuberous Sclerosis Complex 2 Protein; D016159:Tumor Suppressor Protein p53; D025521:Tumor Suppressor Proteins; D051028:bcl-2-Associated X Protein; D004253:DNA Nucleotidylexotransferase; D010751:Phosphopyruvate Hydratase