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At least 19 recordsLinked to original sources
PubMed1990

[Histogenesis of subependymal glioma in Bourneville's tuberous sclerosis].

The phenotypic characteristics of 7 subependymal giant cell astrocytomas (GSECG) (6 of these being associated with tuberous sclerosis) are studied using morphological and immunohistochemical methods with antiserums against vimentine, glial fibrillary acid protein (GFA), S100 protein, and neurofilaments. The glycoproteic secretion of the tumor cells was also analyzed after exposure to Concanavalin A (CON A) by a direct fluorescent method. Our results suggest that some GSECG originate from specialized ependyma (circum-ventricular organs). They have the same location (foramen of Monro), present some common ultrastructural features (cytoplasm with lumen containing cilia), are positive with certain immunohistochemical markers (staining with S100 protein in 4 cases, with vimentin in 3 cases) and show a strong glycoproteic secretion (positive with CON A). Therefore, some GSECG might be considered hamartomas of specialized ependyma, with a reduced evolutivity potential.

Adolescent↗
PubMed1978

[Experimental brain tumors produced transplacentally by ethylnitrosourea (IV): ultrastructure studied by using transmission and scanning electron microscope (author's transl)].

The fine structure of rat gliomas induced transplacentally with a single i.p. dose of 50 mg/kg of Ethylnitrosourea has been studied by using transmission and scanning electron microscope. The subependymal matrix layers of the fetus which was affected by ENU have showed irregular and rough arrangements with expanded extracellular spaces as compared with that of control rats. The cells of subependymal layer seemed to form the microtumor. A so-called "microtumor", which was found in a 8 week old, has been composed of small round cells. The fine structures of these cells have showed the characteristics in primitive oligodendroglioma. The characteristics of the fine structure of astrocytoma cells was identified by both TEM and SEM. The fine structure of subependymal glioma cells was often pleomorphic. These gliomas contained a mixture of primitive oligodendrocytes and ependymal cells together with anaplastic glial cells. With increasing size, the glioma has become more pleomorphic with a mixture of neoplastic oligodendrocytes, astrocytes and ependymal cells, and ependymoma like cells have showed neither cilia nor junctional complex. Abnormal vascular structure in the tumor has been reconfirmed by injection replica scanning electron microscope method. The fine structure of the separated single tumor cell surface was also studied by scanning electron microscope. The differences of the cells surface between that of astrocytoma cell and oligodendroglioma cells were clearly noticed.

Animals↗
PubMed1984

Long-term neurological sequelae in achondroplasia.

Two children with achondroplasia and neurologic sequelae secondary to foramen magnum compression were found to have syringomyelia. In one patient, the cyst was associated with a subependymal glioma. These findings suggest that foramen magnum stenosis may lead to irreversible spinal cord damage.

Achondroplasia↗
PubMed2003

Nonmalignant pediatric brain tumors.

Brain tumors are the most common solid neoplasms in the pediatric population. Each year in the United States, approximately 1500 to 2000 children are affected by one of these tumors. About 50% of pediatric brain tumors are malignant. Nonmalignant pediatric brain tumors comprise an eclectic group of pathologic entities that have fascinating clinical features. Many of these tumors demonstrate a favorable prognosis. In this report, we review the epidemiology, histopathology, genetics, clinical presentation, neuroimaging, and treatment of the common nonmalignant pediatric brain tumors, including low grade astrocytomas (such as cerebellar astrocytoma and optic pathway glioma), subependymal giant cell astrocytomas, central neurocytomas, dysembryoplastic neuroepithelial tumor, choroid plexus papilloma, and craniopharyngioma. Advances in the therapeutic management of patients with these tumors have considerably improved their prognosis.

Adolescent↗
PubMed2003

Subependymal giant cell astrocytoma in children with tuberous sclerosis.

METHODS: Out of 105 patients with tuberous sclerosis (TS) admitted to the Hospital Nacional de Pediatría "Juan P. Garrahan" (Buenos Aires, Argentina), we surgically treated 17 children between January 1988 and December 2000. Two patients were operated on because of epilepsy and 15 patients because of an intraventricular tumor (subependymal giant cell astrocytoma [SGCA]). In this report we focus on tumors. Twelve of the 15 patients presented with hydrocephalus but none of them had a preoperative shunt. All tumors were surgically resected using frontal transventricular or transcallosal routes. Total removal was achieved in 12 out of 15 and subtotal removal in 3 out of 15 patients (resection of 70-95%). RESULTS: Exeresis of the tumor was not accompanied by significant morbidity and there was no perioperative mortality. Seizures and mental retardation did not improve after tumor resection. It was necessary to insert a postoperative shunt a long time after surgery in only one patient. There were no recurrences of SGCA that were totally removed after a mean follow-up of 51.7 months. CONCLUSIONS: We encourage surgery as soon as a lesion is diagnosed as a tumor. The "transformation" of subependymal nodules (SEN) into tumors (SGCA) may be considered controversial.

Adolescent↗
PubMed2004

Tuberin and hamartin expression is reduced in the majority of subependymal giant cell astrocytomas in tuberous sclerosis complex consistent with a two-hit model of pathogenesis.

Subependymal giant cell astrocytomas are distinctive brain tumors that are seen only in tuberous sclerosis complex. Although histologically benign, they cause both moribidity and occasional mortality owing to progressive growth in some patients. Tuberous sclerosis complex is an autosomal dominant genetic disorder with a high sporadic case rate that is due to mutations in either of two genes, TSC1 and TSC2, encoding hamartin and tuberin, respectively. The pathogenesis of subependymal giant cell astrocytomas in tuberous sclerosis complex is uncertain. In this study, we examined the expression of tuberin and hamartin in subependymal giant cell astrocytomas from nine patients with tuberous sclerosis complex by immunohistochemistry with confocal microscopy. Loss of hamartin expression was seen in all subependymal giant cell astrocytomas, including five from patients with germline TSC2 mutations and two from patients with germline TSC1 mutations. The subependymal giant cell astrocytomas of six of nine patients had no expression of tuberin as well, whereas three patients retained some tuberin expression. Tuberin expression was seen in one patient with a TSC2 germline mutation and two patients whose mutational status was not determined. Overall, these data indicate a loss of both tuberin and hamartin expression in the subependymal giant cell astrocytomas of patients with both TSC1 and TSC2 mutations and are consistent with a two-hit disease pathogenesis model for the development of subependymal giant cell astrocytomas.

Antibodies, Monoclonal↗
PubMed1997

Resolution of subependymal cysts in neonatal holocarboxylase synthetase deficiency.

Holocarboxylase synthetase deficiency is typically a biotin responsive disorder that presents with lactic acidosis, tachypnea, temperature instability, and shock in neonates (Briones et al.1989 and Fuchshuber et al. 1992). The primary defect in cases studied to date appears to be the decreased affinity of HCS for its substrate, biotin (Gompertz et al. 1971). Supplemental biotin can provide sufficient substrate to increase HCS enzymatic function and thereby permit biotinylation of the four carboxylase apoenzymes (Briones et al. 1989). We report an infant with HCS deficiency who presented with lactic acidosis, shock, and hypertonia. Subependymal cysts were identified on cranial ultrasound and subsequently confirmed by MRI. Six months following biotin supplementation, she is developmentally normal and MRI of the brain shows complete resolution of the cysts.

Biotin↗
PubMed1994

Familial occurrence of subependymoma. Report of two cases.

Subependymomas are unusual tumors believed to arise from the bipotential subependymal cell. Previous reports of familial occurrence of subependymoma have involved monozygous twins and siblings. The authors describe the first reported occurrence of fourth ventricular subependymoma in a father and son, suggesting the possibility of direct inheritance.

Adult↗
PubMed1995

Surgical treatment of intraventricular tumors associated with tuberous sclerosis.

Six children with intraventricular tumors associated with tuberous sclerosis (TS) were treated at the Children's Health Center, Department of Pediatric Neurosurgery, in the period 1987-1992. The age of the patients ranged from 7 to 15 years. TS was diagnosed according to Gomez diagnostic criteria. Computer tomography (CT) and magnetic resonance imaging showed intraventricular tumors associated with ventricular enlargement and multiple subependymal nodules commonly observed in cases of TS. All tumors were removed totally through frontal transcortical approach, with uneventful postoperative recovery. One patient, with two parallel tumors in the two frontal horns, underwent one-stage surgery with successful total removal. Histopathological examination in all cases showed subependymal giant cell astrocytoma (SGCA). The growth pattern of SGCA associated with TS, documented by sequential CT scans over several years, is described. The diagnosis and surgical treatment of the tumor are discussed, and periodic CT scanning, at least every 2 years, is recommended for patients with TS.

Adolescent↗
PubMed2004

Subependymal giant cell astrocytoma: a clinicopathological study of 23 cases with special emphasis on proliferative markers and expression of p53 and retinoblastoma gene proteins.

AIMS: To gain a better insight into the biological behaviour of subependymal giant cell astrocytoma (SEGA), tumour suppressor gene protein expression and various proliferative indices were studied in these tumours and correlated with histological features and clinical outcome. METHODS: We studied 23 cases of SEGA, 19 from our own Institute and four from the National Institute of Mental Health and Neurological Sciences (NIMHANS), Bangalore, India. Immunohistochemical staining for various glial and neuronal markers, proliferative markers (MIB-1, Topoisomerase II alpha PCNA) and tumour suppressor gene protein expression of p53 and retinoblastoma (Rb) were performed. RESULTS: Nineteen cases of SEGA were collected over a period of 23 years (January 1978-December 2001), which accounted for 0.16% of all intracranial tumours and 0.51% of all gliomas reported at this centre. Ages ranged from 4 to 37 years (mean 13.2 years) with a male preponderance. Nine of the 23 cases were associated with tuberous sclerosis (TS), six at the time of diagnosis, while three developed TS during the follow-up period. Treatment consisted of surgical resection (total in nine cases and subtotal in 14 cases) followed by radiotherapy in seven cases. Except for two patients who died in the immediate post-operative period of surgical complications, the remaining patients were all alive in the follow-up period (mean 37.1 months). One patient experienced recurrence 22 years after the first surgery and a second patient after 2 years. Necrosis and/or mitoses were observed in five cases. Immunohistochemically, tumours were positive for both glial and neuronal markers. Interspersed inflammatory cells were a mixture of mast cells and lymphocytes of T immunophenotype. The MIB-1 labelling index (LI) ranged from 0 to 8% (mean 3.0%), topoisomerase II alpha (topo II alpha) LI from 0 to 9.5% (mean 2.9%) and PCNA LI from 10 to 59% (mean 32.5%). The difference in the labelling indices of tumours with and without mitoses and/or necrosis was not statistically significant. None of the tumours revealed loss of Rb gene protein expression. p53 immunopositivity was seen in 14 cases (labelling indices ranged from 1 to 7.3% with a mean of 2.4%). The correlation between the MIB-1 LI and topo II alpha LI, and topo II alpha LI and PCNA LI was significant (P<0.05) but not so with other parameters like p53 protein expression, duration of survival and morphological features such as mitoses and necrosis. CONCLUSIONS: SEGAs are rare intraventricular tumours associated with TS and express both neuronal and glial markers. They have a low proliferative potential. Mitoses and necrosis are not associated with a worse prognosis. In view of the low proliferative indices and long survival of these patients without recurrence, the role of post-operative radiotherapy is questionable. These patients should be followed up closely as many of them develop stigmata of tuberous sclerosis at a later stage.

Adolescent↗
PubMed2004

201Tl-SPECT and 1H-MRS study of benign lateral ventricle tumors: differential diagnosis of subependymoma.

The introduction of computed tomography (CT) and magnetic resonance (MR) imaging has resulted in the detection of increasing numbers of asymptomatic intraventricular tumors. Establishing the correct preoperative diagnosis is important to prevent unnecessary surgical intervention. Our study includes nine cases of benign lateral ventricle tumors including two cases of central neurocytoma, two of subependymal giant cell astrocytoma, two of pilocytic astrocytoma and three of subependymoma treated surgically between 1996 and 2003. MR imaging, proton MR spectroscopy ((1)H-MRS) and thallium-201 single photon emission computed tomography ((201)Tl-SPECT) were performed in all patients. All three types of tumor demonstrated heterogeneous enhancement on MR imaging with gadolinium-diethylenetriamine pentaacetic acid (Gd-DTPA) and increased choline (Cho) peak and decreased N-acetyl aspartate (NAA) and creatine (Cre) peaks on (1)H-MRS. (201)Tl-SPECT showed high uptake of (201)Tl without wash out in all cases of central neurocytoma, subependymal giant cell astrocytoma and pilocytic astrocytoma, but no uptake in cases of subependymoma. Absence of (201)Tl uptake in contrast with enhancement on MR imaging and the (1)H-MRS features of modest elevation of the Cho/Cre ratio, reduction of the NAA peak and presence of lactate/lipid peaks are characteristic features of subependymomas and useful to establish a preoperative diagnosis.

Adolescent↗
PubMed2000

Traumatic subependymal hematoma during endoscopic third ventriculostomy in a patient with a third ventricle tumor: case report.

Endoscopic third ventriculostomy has become a routine intervention for the treatment of non-communicating hydrocephalus. This technique is largely considered safe and a very low incidence of complications is reported. However, hemorrhage in the course of neuroendoscopy is still a problem difficult to manage. The authors present a case in which endoscopic third ventriculostomy and tumor biopsy were performed in a young patient with a huge tumor growing in the posterior part of the third ventricle. The surgical approach to realize the stoma was difficult because the tumor size reduced the third ventricle diameter. Surgical manipulation produced a traumatic subependymal hematoma. This hematoma drained spontaneously after few minutes into the ventricle and the blood was washed away. The postoperative neurological course was uneventful and the ventriculostomy showed to work well by reducing the size of the lateral ventricles and the intracranial pressure in three days. This complication during endoscopic third ventriculostomy has never been reported before. We emphasize the difficulty of endoscopic procedures in patients with huge tumors in the third ventricle. Where reduction in size of the third ventricle and of the foramen of Monro ist present we suggest a careful approach to the third ventricle.

Adolescent↗