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1. de Carvalho Neto A, Gasparetto EL, Bruck I: Subependymal giant cell astrocytoma with high choline/creatine ratio on proton MR spectroscopy. Arq Neuropsiquiatr; 2006 Sep;64(3B):877-80
Hazardous Substances Data Bank. CHOLINE CHLORIDE .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Subependymal giant cell astrocytoma with high choline/creatine ratio on proton MR spectroscopy.
  • OBJECTIVE: To report a case of subependymal giant cell astrocytoma (SEGA) in a patient with tuberous sclerosis, emphasizing the proton MR spectroscopy (MRS) findings.
  • MRI showed many cortical tubers and subependymal nodules.
  • CONCLUSION: The MRS performed at the SEGA may show high Cho/Cr and low NAA/Cr ratios, similar to the other brain neoplasms.
  • As a consequence, MRS may be a valuable tool for the early detection of neoplastic transformation of subependymal nodules near the foramina of Monro in patients with tuberous sclerosis.
  • [MeSH-major] Astrocytoma / diagnosis. Brain Neoplasms / diagnosis. Choline / analysis. Creatine / analysis. Tuberous Sclerosis / complications
  • [MeSH-minor] Biomarkers, Tumor / analysis. Child, Preschool. Humans. Magnetic Resonance Spectroscopy. Male


2. Koenig MK, Butler IJ, Northrup H: Regression of subependymal giant cell astrocytoma with rapamycin in tuberous sclerosis complex. J Child Neurol; 2008 Oct;23(10):1238-9
Hazardous Substances Data Bank. SIROLIMUS .

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  • [Title] Regression of subependymal giant cell astrocytoma with rapamycin in tuberous sclerosis complex.
  • The authors present a 21-year-old woman who has been receiving rapamycin for 5 months for bilateral subependymal giant cell astrocytomas.

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  • [Cites] Pediatr Res. 2005 Jan;57(1):67-75 [15557109.001]
  • [Cites] BMC Dermatol. 2008;8:1 [18226258.001]
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  • [Cites] Ann Neurol. 2006 Mar;59(3):490-8 [16453317.001]
  • (PMID = 18952591.001).
  • [ISSN] 1708-8283
  • [Journal-full-title] Journal of child neurology
  • [ISO-abbreviation] J. Child Neurol.
  • [Language] ENG
  • [Grant] United States / NCRR NIH HHS / RR / KL2 RR024149; United States / NCRR NIH HHS / RR / RR024149-05S1; United States / NCRR NIH HHS / RR / KL2 RR024149-05S1
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; W36ZG6FT64 / Sirolimus
  • [Other-IDs] NLM/ NIHMS282750; NLM/ PMC3072698
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3. Pascual-Castroviejo I, Pascual-Pascual SI, Velázquez-Fragua R, Viaño J, Carceller F, Hernández-Moneo JL, Gutiérrez-Molina M, Morales C: [Subependymal giant cell astrocytoma in tuberous sclerosis complex. A presentation of eight paediatric patients]. Neurologia; 2010 Jun;25(5):314-21
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  • [Title] [Subependymal giant cell astrocytoma in tuberous sclerosis complex. A presentation of eight paediatric patients].
  • [Transliterated title] Astrocitoma subependimario de células gigantes en el complejo de esclerosis tuberosa. Presentación de ocho pacientes infantiles.
  • OBJECTIVE: Presentation of 8 patients with subependymal giant-cell astrocytomas (SGCA) associated with tuberous sclerosis complex (TSC).
  • Only one patient developed the tumour suddenly from pre-existing subependymal nodules from the childhood and they had to be removed at 24 years of age.
  • By contrast, 32 patients with TSC and images of subependymal nodules whose CT or MR progress was followed up for between 10 and 30 years did not develop a tumour.
  • [MeSH-major] Astrocytoma / etiology. Astrocytoma / pathology. Brain Neoplasms / etiology. Brain Neoplasms / pathology. Tuberous Sclerosis


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4. Takata K, Gasparetto EL, Leite Cda C, Lucato LT, Reed UC, Matushita H, Aguiar PH, Rosemberg S: [Subependymal giant cell astrocytoma in patients with tuberous sclerosis: magnetic resonance imaging findings in ten cases]. Arq Neuropsiquiatr; 2007 Jun;65(2A):313-6
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  • [Title] [Subependymal giant cell astrocytoma in patients with tuberous sclerosis: magnetic resonance imaging findings in ten cases].
  • [Transliterated title] Astrocitoma subependimário de células gigantes em pacientes com esclerose tuberosa: achados em ressonância magnética de dez casos.
  • OBJECTIVE: To report the magnetic resonance imaging (MRI) findings in 10 patients with subependimal giant cell astrocytoma (SGCA) and tuberous sclerosis (TS).
  • All patients underwent MRI, which was analyzed by two radiologists, final diagnosis was reached by consensus.
  • Eight lesions were found near the foramen of Monro and two in the body of the lateral ventricles.
  • [MeSH-major] Astrocytoma / pathology. Brain Neoplasms / pathology. Cerebral Ventricles / pathology. Tuberous Sclerosis / pathology


5. Stavrinou P, Spiliotopoulos A, Patsalas I, Balogiannis I, Karkavelas G, Polyzoidis K, Selviaridis P: Subependymal giant cell astrocytoma with intratumoral hemorrhage in the absence of tuberous sclerosis. J Clin Neurosci; 2008 Jun;15(6):704-6
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  • [Title] Subependymal giant cell astrocytoma with intratumoral hemorrhage in the absence of tuberous sclerosis.
  • Subependymal giant cell astrocytoma (SEGA) is an uncommon tumor that usually occurs in the setting of tuberous sclerosis (TS) syndrome.
  • We report a rare case of an intratumoral and a small intraventricular hemorrhage complicating a SEGA in an adult patient without any signs of TS.
  • Although pre-operative CT and MRI findings for the tumor were typical of SEGA, SEGA was not considered in the differential diagnosis because the patient was lacking any symptoms of TS.
  • This is the second report of intraventricular and intratumoral hemorrhage complicating a SEGA and the first case in which these complications occurred in an adult patient in whom there was no previous suspicion of systemic disease.
  • [MeSH-major] Astrocytoma / complications. Brain Neoplasms / complications. Hemorrhage / etiology

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  • (PMID = 18395450.001).
  • [ISSN] 0967-5868
  • [Journal-full-title] Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
  • [ISO-abbreviation] J Clin Neurosci
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Scotland
  • [Chemical-registry-number] 0 / Glial Fibrillary Acidic Protein
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6. Ichikawa T, Wakisaka A, Daido S, Takao S, Tamiya T, Date I, Koizumi S, Niida Y: A case of solitary subependymal giant cell astrocytoma: two somatic hits of TSC2 in the tumor, without evidence of somatic mosaicism. J Mol Diagn; 2005 Oct;7(4):544-9
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  • [Title] A case of solitary subependymal giant cell astrocytoma: two somatic hits of TSC2 in the tumor, without evidence of somatic mosaicism.
  • Subependymal giant cell astrocytoma (SEGA) is a unique brain tumor arising in tuberous sclerosis complex (TSC), an autosomal dominant inherited phacomatosis.
  • There are several case reports of solitary SEGA without any other manifestations of TSC.
  • Here, we report a 20-year-old woman with a brain tumor.
  • Pathological diagnosis was consistent with SEGA, but comprehensive clinical screening found no other lesions indicative of TSC.
  • Molecular analysis of the tumor revealed loss of heterozygosity and allelic mutation (5228G>A, R1743Q) of TSC2.
  • According to these results, this patient should be considered as having SEGA that developed from two somatic hit mutations in TSC2, rather than being a TSC2 patient with a very small fraction of somatic mosaicism.
  • [MeSH-major] Astrocytoma / genetics. Astrocytoma / pathology. Brain Neoplasms / genetics. Brain Neoplasms / pathology. Mosaicism. Tumor Suppressor Proteins / genetics

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  • (PMID = 16237225.001).
  • [ISSN] 1525-1578
  • [Journal-full-title] The Journal of molecular diagnostics : JMD
  • [ISO-abbreviation] J Mol Diagn
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Tumor Suppressor Proteins; 4JG2LF96VF / tuberous sclerosis complex 2 protein
  • [Other-IDs] NLM/ PMC1888498
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7. Berhouma M: Management of subependymal giant cell tumors in tuberous sclerosis complex: the neurosurgeon's perspective. World J Pediatr; 2010 May;6(2):103-10
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  • [Title] Management of subependymal giant cell tumors in tuberous sclerosis complex: the neurosurgeon's perspective.
  • BACKGROUND: Tuberous sclerosis complex (TSC), an autosomal dominant genetic disorder, can lead to the development of hamartomas in various organs, including the heart, lungs, kidneys, skin and brain.
  • The management of subependymal giant cell tumors (SGCTs) is still controversial, and peri- and/or intraventricular neoplasms may lead to life-threatening hydrocephalus.
  • The data were collected after a bibliography made using PubMed/Medline with these terms: subependymal, subependymal giant cell astrocytoma, subependymal giant cell tumor, and tuberous sclerosis complex.
  • RESULTS: SGCTs are shown to be generated from a glioneuronal lineage, but their filiation with subependymal nodules (SENs) is still under debate.
  • CONCLUSIONS: An earlier diagnosis of SGCT in neurologically asymptomatic children with TSC may allow a precocious surgical removal of the tumor before the installation of increased intracranial pressure signs, an attitude that is being progressively adopted to lessen the morbimortality rate.
  • [MeSH-major] Astrocytoma / complications. Brain Neoplasms / complications. Tuberous Sclerosis / complications
  • [MeSH-minor] Algorithms. Child. Humans. Hydrocephalus / etiology. Intracranial Hypertension / etiology. Mutation. Neurosurgical Procedures. Tumor Suppressor Proteins / genetics

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  • (PMID = 20490765.001).
  • [ISSN] 1867-0687
  • [Journal-full-title] World journal of pediatrics : WJP
  • [ISO-abbreviation] World J Pediatr
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Tumor Suppressor Proteins; 0 / tuberous sclerosis complex 1 protein; 4JG2LF96VF / tuberous sclerosis complex 2 protein
  • [Number-of-references] 60
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8. Krueger DA, Care MM, Holland K, Agricola K, Tudor C, Mangeshkar P, Wilson KA, Byars A, Sahmoud T, Franz DN: Everolimus for subependymal giant-cell astrocytomas in tuberous sclerosis. N Engl J Med; 2010 Nov 4;363(19):1801-11
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  • [Title] Everolimus for subependymal giant-cell astrocytomas in tuberous sclerosis.
  • BACKGROUND: Neurosurgical resection is the standard treatment for subependymal giant-cell astrocytomas in patients with the tuberous sclerosis complex.
  • METHODS: Patients 3 years of age or older with serial growth of subependymal giant-cell astrocytomas were eligible for this open-label study.
  • The primary efficacy end point was the change in volume of subependymal giant-cell astrocytomas between baseline and 6 months.
  • Everolimus therapy was associated with a clinically meaningful reduction in volume of the primary subependymal giant-cell astrocytoma, as assessed on independent central review (P<0.001 for baseline vs. 6 months), with a reduction of at least 30% in 21 patients (75%) and at least 50% in 9 patients (32%).
  • There were no new lesions, worsening hydrocephalus, evidence of increased intracranial pressure, or necessity for surgical resection or other therapy for subependymal giant-cell astrocytoma.
  • CONCLUSIONS: Everolimus therapy was associated with marked reduction in the volume of subependymal giant-cell astrocytomas and seizure frequency and may be a potential alternative to neurosurgical resection in some cases, though long-term studies are needed. (Funded by Novartis; ClinicalTrials.gov number, NCT00411619.).
  • [MeSH-major] Astrocytoma / drug therapy. Brain Neoplasms / drug therapy. Intracellular Signaling Peptides and Proteins / antagonists & inhibitors. Protein-Serine-Threonine Kinases / antagonists & inhibitors. Seizures / drug therapy. Sirolimus / analogs & derivatives. Tuberous Sclerosis / drug therapy


9. Buccoliero AM, Franchi A, Castiglione F, Gheri CF, Mussa F, Giordano F, Genitori L, Taddei GL: Subependymal giant cell astrocytoma (SEGA): Is it an astrocytoma? Morphological, immunohistochemical and ultrastructural study. Neuropathology; 2009 Feb;29(1):25-30
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  • [Title] Subependymal giant cell astrocytoma (SEGA): Is it an astrocytoma? Morphological, immunohistochemical and ultrastructural study.
  • Subependymal giant-cell astrocytoma (SEGA) is a rare intra-ventricular low-grade tumor which frequently occurs as a manifestation of tuberous sclerosis complex.
  • The histogenesis of SEGA is controversial and its astrocytic nature has been doubted.
  • First studies suggested the astrocytic nature of SEGA while several recent reports demonstrate its glio-neuronal nature.
  • In spite of this, in the recently revised WHO classification of the CNS tumors, SEGA has been still included in the group of astrocytomas.
  • Eight patients (89%) had a solitary lesion located in the lateral ventricle close to of the head of the caudate nucleus, the remaining patient (11%) had two tumors, one located close to the head of the left caudate nucleus and the other in the central part of the right lateral ventricle.
  • In all four there were glial cell processes filled with intermediate filaments.
  • Our results emphasize the glio-neuronal nature of SEGA.
  • We suggest moving it into the group of mixed glio-neuronal tumors under the denomination of subependymal giant cell tumor.
  • [MeSH-major] Astrocytoma / pathology. Neoplasms, Multiple Primary / pathology
  • [MeSH-minor] Adolescent. Brain Neoplasms / chemistry. Brain Neoplasms / classification. Brain Neoplasms / pathology. Brain Neoplasms / ultrastructure. Cerebral Ventricle Neoplasms / chemistry. Cerebral Ventricle Neoplasms / classification. Cerebral Ventricle Neoplasms / pathology. Cerebral Ventricle Neoplasms / ultrastructure. Child. Child, Preschool. Female. Humans. Immunohistochemistry. Infant. Intermediate Filaments / ultrastructure. Male. Nerve Tissue Proteins / analysis. Neurofilament Proteins / analysis. Neuroglia / pathology. Neuroglia / ultrastructure. Phosphopyruvate Hydratase / analysis. Synaptophysin / analysis

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  • (PMID = 18564101.001).
  • [ISSN] 1440-1789
  • [Journal-full-title] Neuropathology : official journal of the Japanese Society of Neuropathology
  • [ISO-abbreviation] Neuropathology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Nerve Tissue Proteins; 0 / Neurofilament Proteins; 0 / Synaptophysin; EC 4.2.1.11 / Phosphopyruvate Hydratase
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10. Kim SH, Lee KG, Kim TS: Cytologic characteristics of subependymal giant cell astrocytoma in squash smears: morphometric comparisons with gemistocytic astrocytoma and giant cell glioblastoma. Acta Cytol; 2007 May-Jun;51(3):375-9
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  • [Title] Cytologic characteristics of subependymal giant cell astrocytoma in squash smears: morphometric comparisons with gemistocytic astrocytoma and giant cell glioblastoma.
  • OBJECTIVE: To evaluate the squash smear features of subependymal giant cell astrocytoma (SEGA) in comparison with gemistocytic astrocytoma and giant cell glioblastoma.
  • STUDY DESIGN: We compared the squash smear features of 3 cases of SEGA, 9 cases of gemistocytic astrocytoma and 3 cases of giant cell glioblastoma with the morphometric findings.
  • RESULTS: SEGA had, on average, a 15.84 +/- 5.03-microm nucleus, 33.22 +/- 12.05-microm cytoplasm and 0.50 +/- 0.12 nuclear/cytoplasmic ratio in squash smears.
  • In addition, SEGA showed hairlike processes distributed along the squash direction like strap cells.
  • While the gemistocytic astrocytoma had several tumor cells showing a vertically located nucleus, the tumor cells of SEGA showed nuclei oriented mainly in parallel.
  • CONCLUSION: These squash cytologic features of SEGA can be very helpful in the differential diagnosis by excluding mimics.
  • [MeSH-major] Astrocytoma / pathology. Brain Neoplasms / pathology. Glioblastoma / pathology
  • [MeSH-minor] Adolescent. Adult. Cell Nucleus / pathology. Child. Child, Preschool. Cytoplasm / pathology. Diagnosis, Differential. Female. Humans. Male


11. Chen H, Sun XF, Wu JS: [Clinicopathologic study of subependymal giant cell astrocytoma]. Zhonghua Bing Li Xue Za Zhi; 2006 Nov;35(11):656-9
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  • [Title] [Clinicopathologic study of subependymal giant cell astrocytoma].
  • OBJECTIVE: To study the clinicopathologic features of subependymal giant cell astrocytoma.
  • METHODS: The clinical and pathologic characteristics of 18 cases of subependymal giant cell astrocytoma were retrospectively analyzed.
  • The tumor often occurred in the lateral ventricles (16/18, 88.9%).
  • MRI revealed isointense mass lesion on T1WI (7/11, 63.6%), highly intense lesion on T2WI (10/11, 90.9%) and contrast enhancement in some cases (9/11, 81.8%).
  • CONCLUSIONS: Subependymal giant cell astrocytoma is a benign brain tumor with distinctive histopathologic features.
  • The tumor typically affects children and young adults.
  • [MeSH-major] Astrocytoma / pathology. Cerebral Ventricle Neoplasms / pathology

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  • (PMID = 17374208.001).
  • [ISSN] 0529-5807
  • [Journal-full-title] Zhonghua bing li xue za zhi = Chinese journal of pathology
  • [ISO-abbreviation] Zhonghua Bing Li Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Glial Fibrillary Acidic Protein; 0 / Synaptophysin
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12. You H, Kim YI, Im SY, Suh-Kim H, Paek SH, Park SH, Kim DG, Jung HW: Immunohistochemical study of central neurocytoma, subependymoma, and subependymal giant cell astrocytoma. J Neurooncol; 2005 Aug;74(1):1-8
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  • [Title] Immunohistochemical study of central neurocytoma, subependymoma, and subependymal giant cell astrocytoma.
  • For investigation of histogenesis of central neurocytomas (CNs), subependymoma (SEs), subependymal giant cell astrocytomas (SEGAs), we studied expression of various neuronal and glial biomarkers by immunohistochemical (IHC) study and reverse transcriptase-polymerase chain reaction (RT-PCR).
  • The materials for IHC were paraffin section of seven CNs, three SEs, and eight SEGAs and those for RT-PCR were frozen tissues of seven CNs, three SEs, and five SEGAs.
  • The neuronal biomarkers included nestin, chromogranin A (chrA), synaptophysin (SNP), neuronal cell adhesion molecule (NCAM), neuron specific enolase (NSE), neuronal nuclear antigen (NeuN), neurofilament (NF) and the glial marker was GFAP.
  • CNs expressed all neuronal markers except NF (0%), SNP (100%), NCAM (100%), NSE (100%), NeuN (100%), nestin (29%) and chrA (43%), but GFAP expression was found only in one case (14%).
  • SEGA coexpressed several neuronal markers and a glial marker; NeuN (100%), NSE (88%), NCAM (63%), nestin (100%), SNP (weakly and focally, 100%), and GFAP (100%), however, other neuronal markers including chrA, SNP and NF were all negative.
  • RT-PCR product of nestin was expressed in 29% of CNs (2/7cases), 60% of SEGAs (3/5 cases), 100% of SEs (3/3 cases), 80% of EPs (4/5 cases), and 25% of PAs (1/4 cases).
  • Conclusively, coexpression of neuronal and glial markers and expression of nestin in CNs, SEGAs and SEs suggested the origin of these tumor cells might be the stem cells being able to differentiate into both neuronal and glial phenotypes.
  • But CNs might be originated from rather neuronally committed stem cells and SEs from rather glially committed stem cells.
  • [MeSH-major] Astrocytoma / metabolism. Biomarkers, Tumor / analysis. Brain Neoplasms / metabolism. Glioma, Subependymal / metabolism. Neurocytoma / metabolism

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  • (PMID = 16078101.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Intermediate Filament Proteins; 0 / NES protein, human; 0 / Nerve Tissue Proteins; 0 / Nestin
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13. Ess KC, Kamp CA, Tu BP, Gutmann DH: Developmental origin of subependymal giant cell astrocytoma in tuberous sclerosis complex. Neurology; 2005 Apr 26;64(8):1446-9
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  • [Title] Developmental origin of subependymal giant cell astrocytoma in tuberous sclerosis complex.
  • Children with tuberous sclerosis complex (TSC) harbor developmental brain abnormalities (cortical tubers) and low-grade tumors (subependymal giant cell astrocytomas [SEGAs]).
  • Using gene expression profiling to identify neuroglial differentiation markers in Tsc1 conditional knockout mice, the authors demonstrate that giant cells of SEGAs aberrantly express similar neuroglial differentiation markers as do cortical tubers.
  • These results suggest that both tubers and SEGAs result from related defects in progenitor cell differentiation during brain development.
  • [MeSH-major] Astrocytoma / genetics. Astrocytoma / metabolism. Brain Neoplasms / genetics. Brain Neoplasms / metabolism. Cell Differentiation / genetics. Tuberous Sclerosis / genetics. Tuberous Sclerosis / metabolism
  • [MeSH-minor] Adolescent. Animals. Cell Lineage / genetics. Child. Disease Models, Animal. Female. Gene Expression Profiling. Gene Expression Regulation, Developmental / genetics. Genetic Markers / genetics. Humans. Infant. Male. Mice. Mice, Knockout. Nerve Tissue Proteins / genetics. Neurons / metabolism. Stem Cells / metabolism. Tumor Suppressor Proteins / genetics


14. Raju GP, Urion DK, Sahin M: Neonatal subependymal giant cell astrocytoma: new case and review of literature. Pediatr Neurol; 2007 Feb;36(2):128-31
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  • [Title] Neonatal subependymal giant cell astrocytoma: new case and review of literature.
  • Subependymal giant cell astrocytomas are one of the three major intracranial lesions found in tuberous sclerosis complex.
  • Subependymal giant cell astrocytomas are typically slow-growing tumors of mixed glioneuronal lineage which can become aggressive and cause obstructive hydrocephalus usually in older children and adolescents.
  • Neonatal subependymal giant cell astrocytomas are extremely rare, and their natural history and prognosis are poorly understood.
  • This report investigates an extremely large neonatal subependymal giant cell astrocytoma which was initially identified in utero at 19 weeks of gestation in a high-risk pregnancy with no family history of tuberous sclerosis complex.
  • [MeSH-major] Astrocytoma / pathology. Brain Neoplasms / pathology. Magnetic Resonance Imaging
  • [MeSH-minor] Adult. Fatal Outcome. Female. Humans. Infant, Newborn. Pregnancy. Pregnancy, High-Risk. Prenatal Diagnosis. Tuberous Sclerosis

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  • (PMID = 17275668.001).
  • [ISSN] 0887-8994
  • [Journal-full-title] Pediatric neurology
  • [ISO-abbreviation] Pediatr. Neurol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 16
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15. Phi JH, Park SH, Chae JH, Hong KH, Park SS, Kang JH, Jun JK, Cho BK, Wang KC, Kim SK: Congenital subependymal giant cell astrocytoma: clinical considerations and expression of radial glial cell markers in giant cells. Childs Nerv Syst; 2008 Dec;24(12):1499-503
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  • [Title] Congenital subependymal giant cell astrocytoma: clinical considerations and expression of radial glial cell markers in giant cells.
  • OBJECTS: Congenital Subependymal giant cell astrocytoma (SEGA), diagnosed in fetal and neonatal period, is extremely rare.
  • We encountered a patient diagnosed as congenital SEGA and report the surgical outcome along with interesting immuno-phenotypes of giant tumor cells.
  • CASE: Ventriculomegaly and a hypoechoic mass near the foramen of Monro were detected in a fetus on prenatal ultrasonography in the 35th week of gestation.
  • At postnatal 2 months, gross total resection of the tumor was achieved without complications.
  • The patient had been followed up for 1 year without tumor recurrence.
  • In double immunofluorescence, the prototype cells of SEGA expressed a variety of neural stem cell (nestin and Sox2) and radial glial cell markers (vimentin and brain lipid-binding protein), in addition to glutamate/aspartate transporter and glial fibrillary acidic protein.
  • CONCLUSIONS: Congenital SEGA can be successfully treated with judicious use of observation period and careful evaluation of general conditions.
  • Pathological findings support the concept that SEGA may originate from aberrant radial glial cells in the developing brain.
  • [MeSH-major] Astrocytoma / surgery. Cerebral Ventricle Neoplasms / surgery. Fetal Diseases / surgery
  • [MeSH-minor] Carrier Proteins / biosynthesis. Fatty Acid-Binding Protein 7. Female. Fluorescent Antibody Technique / methods. Humans. Infant, Newborn. Intermediate Filament Proteins / biosynthesis. Male. Nerve Tissue Proteins / biosynthesis. Nestin. Pregnancy. Prenatal Diagnosis. SOXB1 Transcription Factors / biosynthesis. Tumor Suppressor Proteins / biosynthesis. Ultrasonography, Prenatal. Vimentin / biosynthesis

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  • (PMID = 18629509.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Carrier Proteins; 0 / FABP7 protein, human; 0 / Fatty Acid-Binding Protein 7; 0 / Intermediate Filament Proteins; 0 / NES protein, human; 0 / Nerve Tissue Proteins; 0 / Nestin; 0 / SOX2 protein, human; 0 / SOXB1 Transcription Factors; 0 / Tumor Suppressor Proteins; 0 / Vimentin
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16. Takei H, Adesina AM, Powell SZ: Solitary subependymal giant cell astrocytoma incidentally found at autopsy in an elderly woman without tuberous sclerosis complex. Neuropathology; 2009 Apr;29(2):181-6
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  • [Title] Solitary subependymal giant cell astrocytoma incidentally found at autopsy in an elderly woman without tuberous sclerosis complex.
  • Subependymal giant cell astrocytoma (SEGA) is a benign, slowly growing tumor typically occurring in the setting of tuberous sclerosis complex (TSC).
  • However there are several reported cases in which patients with a solitary SEGA had no other stigmata of TSC.
  • We describe a case of SEGA in a 75-year-old woman representing the oldest patient reported to-date.
  • Postmortem examination of the brain revealed a single 2.1 x 1.0 x 0.8 cm intraventricular nodule in the lateral ventricle.
  • Histologically, it was composed of interlacing bundles of spindle-shaped tumor cells with thin delicate processes admixed with relatively large pleomorphic cells with abundant glassy eosinophilic cytoplasm, as seen in a SEGA.
  • Immunohistochemically, GFAP, S-100 protein, and neuron specific enolase were positive, and synaptophysin labeled a few tumor cells.
  • Also noted were rare isolated MM cells within the tumor (i.e., tumor-to-tumor metastasis).
  • The histopathological differential diagnosis was limited and included giant cell ependymoma and, much less likely, giant cell glioblastoma and pleomorphic xanthoastrocytoma.
  • This case illustrates that SEGA can be found incidentally in an elderly individual with no associated symptoms and also indicates that SEGA can occur outside the setting of TSC.
  • Tumor metastasis to an occult SEGA is extremely rare.
  • [MeSH-major] Astrocytoma / pathology. Brain Neoplasms / pathology
  • [MeSH-minor] Aged. Autopsy. Brain / pathology. Brain / physiopathology. Diagnosis, Differential. Female. Glial Fibrillary Acidic Protein / metabolism. Humans. Immunohistochemistry. Melanoma / pathology. Melanoma / physiopathology. Melanoma / secondary. Neoplasm Metastasis. Phosphopyruvate Hydratase / metabolism. S100 Proteins / metabolism

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  • (PMID = 18673443.001).
  • [ISSN] 1440-1789
  • [Journal-full-title] Neuropathology : official journal of the Japanese Society of Neuropathology
  • [ISO-abbreviation] Neuropathology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Glial Fibrillary Acidic Protein; 0 / S100 Proteins; EC 4.2.1.11 / Phosphopyruvate Hydratase
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17. Cai R, Di X: Combined intra- and extra-endoscopic techniques for aggressive resection of subependymal giant cell astrocytomas. World Neurosurg; 2010 Jun;73(6):713-8
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  • [Title] Combined intra- and extra-endoscopic techniques for aggressive resection of subependymal giant cell astrocytomas.
  • We report two cases of combined intra-/extra-axial endoscopic procedures--intraventricular solid tumor resection for subependymal giant cell astrocytoma.
  • METHODS: In 2007, two patients with subependymal giant cell astrocytoma with a long history of tuberous sclerosis underwent solely endoscopic, minimally invasive intraventricular tumor resection.
  • Pathologic diagnoses were subependymal giant cell astrocytoma.
  • EMIN is a completive, safe procedure for intraventricular subependymal giant cell astrocytoma.
  • [MeSH-major] Astrocytoma / surgery. Cerebral Ventricle Neoplasms / surgery. Endoscopy / methods. Frontal Lobe / surgery. Lateral Ventricles / surgery. Neurosurgical Procedures / methods

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  • [Copyright] Copyright © 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20934162.001).
  • [ISSN] 1878-8769
  • [Journal-full-title] World neurosurgery
  • [ISO-abbreviation] World Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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18. de Ribaupierre S, Dorfmüller G, Bulteau C, Fohlen M, Pinard JM, Chiron C, Delalande O: Subependymal giant-cell astrocytomas in pediatric tuberous sclerosis disease: when should we operate? Neurosurgery; 2007 Jan;60(1):83-89; discussion 89-90
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  • [Title] Subependymal giant-cell astrocytomas in pediatric tuberous sclerosis disease: when should we operate?
  • OBJECTIVE: A small percentage of tuberous sclerosis patients will develop a subependymal giant-cell astrocytoma.
  • METHODS: We retrospectively reviewed 19 patients treated surgically for intraventricular tumors in Foch Hospital and at the Fondation Adolphe de Rothschild in Paris, France, and we analyzed published pediatric reports from 1980 to 2006.
  • RESULTS: The results from our own population, as well as from other published pediatric series (15 series), indicate that subependymal giant-cell astrocytomas have a good prognosis when a macroscopically total resection has been performed.
  • In our series, residual lesions tended to enlarge, but residual tumors remaining stable have been reported.
  • Larger or symptomatic lesions tend to have a higher morbidity.
  • CONCLUSION: We think that any lesion fulfilling the criteria for a subependymal giant-cell astrocytoma as previously described in the literature (lesion around the foramen of Monro, greater than 5 mm, with incomplete calcifications) should be removed as soon as clear evidence of growth has been confirmed.
  • [MeSH-major] Astrocytoma / surgery. Brain Neoplasms / surgery. Tuberous Sclerosis / surgery


19. Takei H, Florez L, Bhattacharjee MB: Cytologic features of subependymal giant cell astrocytom: a review of 7 cases. Acta Cytol; 2008 Jul-Aug;52(4):445-50

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Cytologic features of subependymal giant cell astrocytom: a review of 7 cases.
  • OBJECTIVE: To describe the cytologic features of subependymal giant cell astrocytoma (SEGA) on smears and analyze cytomorphologic parameters that may help in reaching the diagnosis of SEGA.
  • STUDY DESIGN: Cytologic smears of 7 cases of SEGA were reviewed and graded semi-quantitatively for 11 cytologic features: clustering, cytoplasmic fibrillary processes (fibrillarity), cellularity, small prominent nudcleoli, binucleation or multinucleation, "strap cells", spindle-shaped cells, mitoses, intranuclear inclusions, nuclear atypia and perivascular palisading/pseudorosettes.
  • Predominant features included hypercellularity, cell clustering, and fibrillarity.
  • Although common in histologic sections, perivascular palisading/pseudorosettes and spindled astrocytic cells were rarely noted on smears.
  • CONCLUSION: The cytologic features of SEGA are highly characteristic and thus are of great use in supporting a diagnosis of SEGA and in excluding mimics, primarily gemistocytic astrocytoma and ependymoma.
  • [MeSH-major] Astrocytoma / pathology. Cerebral Ventricle Neoplasms / pathology. Lateral Ventricles / pathology
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Diagnosis, Differential. Female. Humans. Male. Retrospective Studies

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  • (PMID = 18702362.001).
  • [ISSN] 0001-5547
  • [Journal-full-title] Acta cytologica
  • [ISO-abbreviation] Acta Cytol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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20. Ahmad Z, Rauf F, Azad NS, Ahsan A: Subependymal giant cell astrocytoma. J Pak Med Assoc; 2006 Oct;56(10):463-5
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  • [Title] Subependymal giant cell astrocytoma.
  • Subependymal giant cell astrocytomas (SEGAs) are slowly growing tumours corresponding to WHO grade I.
  • [MeSH-major] Astrocytoma / pathology. Cerebral Ventricle Neoplasms / pathology

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  • (PMID = 17144395.001).
  • [ISSN] 0030-9982
  • [Journal-full-title] JPMA. The Journal of the Pakistan Medical Association
  • [ISO-abbreviation] J Pak Med Assoc
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Pakistan
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21. Ibrahim I, Young CA, Larner AJ: Fornix damage from solitary subependymal giant cell astrocytoma causing postoperative amnesic syndrome. Br J Hosp Med (Lond); 2009 Aug;70(8):478-9
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  • [Title] Fornix damage from solitary subependymal giant cell astrocytoma causing postoperative amnesic syndrome.
  • For example, anterograde amnesia associated with damage to the fornix (Sweet et al, 1959), a fibre bundle connecting the hippocampus to the mamillary bodies within the limbic system, is well described following removal of third ventricle colloid cysts (Hodges and Carpenter, 1991; McMackin et al, 1995; Aggleton et al, 2000), suggesting the importance of the fornix as one of the anatomical substrates of the distributed neural network underpinning memory functions (Mesulam, 1990).
  • This article reports the cognitive impairments associated with left fornix damage which became apparent following surgical removal of a solitary subependymal giant cell astrocytoma.
  • [MeSH-major] Amnesia / etiology. Astrocytoma / complications. Fornix, Brain. Postoperative Complications / etiology

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  • (PMID = 19684542.001).
  • [ISSN] 1750-8460
  • [Journal-full-title] British journal of hospital medicine (London, England : 2005)
  • [ISO-abbreviation] Br J Hosp Med (Lond)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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22. Bongiorni L, Arroyo HA, Lubienicki F: [Subependymal nodules-sudependymal giant cell astrocytoma complex in children with tuberous sclerosis]. Medicina (B Aires); 2009;69(1 Pt 1):8-14
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  • [Title] [Subependymal nodules-sudependymal giant cell astrocytoma complex in children with tuberous sclerosis].
  • [Transliterated title] Complejo nódulo subependimario-astrocitoma subependimario gigantocelular en niños con esclerosis tuberosa.
  • The object of this paper is to describe the imaging and clinical characteristics of subependymal nodule (SN) - subependymal giant cell astrocytoma (SGCA) complex in tuberous sclerosis and analyze its evolution in order to attempt early detection and the prevention of intracranial hypertension.
  • We evaluated 22 patients with the pathological diagnosis of SGCA.
  • The diagnosis was made at a median of 10.1 years old.
  • Six patients presented visual deficit and in these, the average diameter of the tumor was 31.5 mm, a high value when compared to 18.7 mm in the patients without visual deficit.
  • The imaging and clinical follow-up of any subependymal lesion close to the foramen of Monro will permit, at a presymptomatic stage, an anticipation of surgical treatment thus reducing intracranial hypertension incidence.
  • Prospective studies could determine whether the SN-SGCA complex corresponds to the same entity in distinct evolution stages or to two lesions with different growth potential.
  • [MeSH-major] Astrocytoma / pathology. Brain Neoplasms / pathology. Cerebral Ventricles / pathology. Tuberous Sclerosis / pathology
  • [MeSH-minor] Adolescent. Cerebral Ventricle Neoplasms / pathology. Cerebral Ventricle Neoplasms / radiography. Cerebral Ventricle Neoplasms / surgery. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Hydrocephalus / etiology. Infant. Intellectual Disability / etiology. Intracranial Hypertension / prevention & control. Male


23. Işik U, Dinçer A, Sav A, Ozek MM: Basal ganglia location of subependymal giant cell astrocytomas in two infants. Pediatr Neurol; 2010 Feb;42(2):157-9
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  • [Title] Basal ganglia location of subependymal giant cell astrocytomas in two infants.
  • Subependymal giant cell astrocytomas are benign tumors that constitute one of the primary features of tuberous sclerosis.
  • Two infants with tuberous sclerosis had very unusual subependymal giant cell astrocytomas, confirmed on biopsy in one of the infants.
  • In both cases, contrast-enhanced cranial magnetic resonance imaging suggested a calcified intra-axial mass with diffuse basal ganglia involvement extending into the lateral ventricle.
  • Biopsy of the basal ganglia lesion in that case suggested subependymal giant cell astrocytoma.
  • In infants, subependymal giant cell astrocytomas can present with unusual morphology and may feature diffuse basal ganglia involvement and severe calcification.
  • [MeSH-major] Astrocytoma / pathology. Basal Ganglia / pathology. Brain Neoplasms / pathology

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  • (PMID = 20117758.001).
  • [ISSN] 1873-5150
  • [Journal-full-title] Pediatric neurology
  • [ISO-abbreviation] Pediatr. Neurol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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24. Jozwiak J, Jozwiak S, Skopinski P: Immunohistochemical and microscopic studies on giant cells in tuberous sclerosis. Histol Histopathol; 2005 10;20(4):1321-6
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  • [Title] Immunohistochemical and microscopic studies on giant cells in tuberous sclerosis.
  • In three types of brain lesions: cortical tubers, subependymal nodules and subependymal giant-cell astrocytoma (SEGA) characteristic, so-called "giant cells" are found.
  • In the present review we summarise immunohistochemical findings of two types of studies performed on giant cells aiming at establishing the expression of hamartin and tuberin level and determining the presence of neuron- or astrocyte-specific markers.
  • We conclude that giant cells in cortical tubers and SEGAs are the same undifferentiated cells that, depending on individual determination, can show neural or glial features.
  • [MeSH-major] Giant Cells / pathology. Tuberous Sclerosis / metabolism. Tuberous Sclerosis / pathology

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  • (PMID = 16136513.001).
  • [ISSN] 0213-3911
  • [Journal-full-title] Histology and histopathology
  • [ISO-abbreviation] Histol. Histopathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Spain
  • [Number-of-references] 51
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25. Adamek D, Dec M, Sobol G, Urbanowicz B, Jaworski M: Giant cell ependymoma: a case report. Clin Neurol Neurosurg; 2008 Feb;110(2):176-81
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  • [Title] Giant cell ependymoma: a case report.
  • A peculiar variant of ependymoma known as "giant cell ependymoma" ("GCE") is especially rarely reported, it may pose some difficulties for the diagnosing neuropathologist.
  • Here we present a case of a giant cell ependymoma occuring in a 17-year-old patient with the history of 2-year recurrent headaches and a 1-month history of vision impairment.
  • CT scanning demonstrated a mass in the left occipital lobe, arising from the occipital horn of the lateral ventricle.
  • Especially striking was the presence of bizzare pleomorphic giant cells which predominated in the tumor tissue.
  • As a result the diagnosis of GCE was established.
  • This type of neoplasm necessitates, at least in theory, differentiation with anaplastic oligodendroglioma, clear cell ependymoma, pleomorphic xanthoastrocytoma, giant cell glioblastoma, and subependymal giant cell astrocytoma.
  • To date giant cell ependymomas (GCEs) were reported in seven cases in the literature.
  • In spite of apparently "worrisome" histology GCE seems to be a neoplasm with a relatively good prognosis.

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  • (PMID = 18006220.001).
  • [ISSN] 0303-8467
  • [Journal-full-title] Clinical neurology and neurosurgery
  • [ISO-abbreviation] Clin Neurol Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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26. Adriaensen ME, Schaefer-Prokop CM, Stijnen T, Duyndam DA, Zonnenberg BA, Prokop M: Prevalence of subependymal giant cell tumors in patients with tuberous sclerosis and a review of the literature. Eur J Neurol; 2009 Jun;16(6):691-6
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  • [Title] Prevalence of subependymal giant cell tumors in patients with tuberous sclerosis and a review of the literature.
  • BACKGROUND AND PURPOSE: To investigate the prevalence of subependymal giant cell ependymomas (SEGA) in patients with tuberous sclerosis complex (TSC).
  • The most recent scan was evaluated for SEGA and presence of hydrocephalus.
  • Additionally, a literature search was performed, and pooled estimates of SEGA prevalence in TSC were calculated.
  • RESULTS: Computed tomography showed radiological evidence of SEGA in 43 of the 214 TSC-patients (20%); 23 of 105 men (22%) and 20 of 109 women (18%; P = .52).
  • Average maximum tumor size was 11.4 mm (range, 4-29 mm).
  • Patients with SEGA (mean, 31 years; range, 16-58 years) were on average younger than patients without SEGA (mean, 37 years; range, 10-72 years; P = 0.007).
  • No association between tumor size and patient age was detected.
  • Nine patients had bilateral SEGA.
  • Meta-analysis of reported prevalence and our current study showed that studies using radiological evidence to diagnose SEGA gave a higher pooled estimate of the prevalence of SEGA in TSC (0.16; 95% CI: 0.12, 0.21) than studies using mainly histopathological evidence of SEGA (0.09; 95% CI: 0.07, 0.12).
  • CONCLUSIONS: In our cohort, CT demonstrated evidence of SEGA in 20% of TSC-patients.
  • Prevalence of SEGA in TSC is higher in studies using radiological evidence to diagnose SEGA than in studies using histopathological evidence.
  • [MeSH-major] Astrocytoma / epidemiology. Brain Neoplasms / epidemiology. Tuberous Sclerosis / epidemiology


27. Tyburczy ME, Kotulska K, Pokarowski P, Mieczkowski J, Kucharska J, Grajkowska W, Roszkowski M, Jozwiak S, Kaminska B: Novel proteins regulated by mTOR in subependymal giant cell astrocytomas of patients with tuberous sclerosis complex and new therapeutic implications. Am J Pathol; 2010 Apr;176(4):1878-90
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  • [Title] Novel proteins regulated by mTOR in subependymal giant cell astrocytomas of patients with tuberous sclerosis complex and new therapeutic implications.
  • Subependymal giant cell astrocytomas (SEGAs) are rare brain tumors associated with tuberous sclerosis complex (TSC), a disease caused by mutations in TSC1 or TSC2, resulting in enhancement of mammalian target of rapamycin (mTOR) activity, dysregulation of cell growth, and tumorigenesis.
  • Of the genes differentially expressed in TSC, 11 were validated by real-time PCR on independent tumor samples and 3 SEGA-derived cultures.
  • ANXA1, GPNMB, LTF, RND3, S100A11, SFRP4, and NPTX1 genes were likely to be mTOR effector genes in SEGA, as their expression was modulated by an mTOR inhibitor, rapamycin, in SEGA-derived cells.
  • Inhibition of mTOR signaling affected size of cultured SEGA cells but had no influence on their proliferation, morphology, or migration, whereas inhibition of both mTOR and extracellular signal-regulated kinase signaling pathways led to significant alterations of these processes.
  • For the first time, we identified genes related to the occurrence of SEGA and regulated by mTOR and demonstrated an effective modulation of SEGA growth by pharmacological inhibition of both mTOR and extracellular signal-regulated kinase signaling pathways, which could represent a novel therapeutic approach.
  • [MeSH-major] Astrocytoma / metabolism. Gene Expression Regulation, Neoplastic. Giant Cells / cytology. TOR Serine-Threonine Kinases / metabolism. Tuberous Sclerosis / metabolism

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  • (PMID = 20133820.001).
  • [ISSN] 1525-2191
  • [Journal-full-title] The American journal of pathology
  • [ISO-abbreviation] Am. J. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 63231-63-0 / RNA; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; G34N38R2N1 / Bromodeoxyuridine
  • [Other-IDs] NLM/ PMC2843477
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28. Levine NB, Collins J, Franz DN, Crone KR: Gradual formation of an operative corridor by balloon dilation for resection of subependymal giant cell astrocytomas in children with tuberous sclerosis: specialized minimal access technique of balloon dilation. Minim Invasive Neurosurg; 2006 Oct;49(5):317-20
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Gradual formation of an operative corridor by balloon dilation for resection of subependymal giant cell astrocytomas in children with tuberous sclerosis: specialized minimal access technique of balloon dilation.
  • BACKGROUND: Major sources of morbidity and mortality in patients with tuberous sclerosis who develop subependymal giant cell astrocytomas (SEGAs) relate to tumor growth and resultant hydrocephalus.
  • We describe a modification of a specialized minimal access resection technique in which an operative corridor is formed with balloon dilation over the course of a week prior to tumor resection.
  • METHODS: Three patients with tuberous sclerosis who had an enlarging SEGA and concomitant hydrocephalus underwent surgical resection with this modified technique.
  • A frontal craniotomy was performed and the optimal trajectory for tumor resection was confirmed by image guidance.
  • After initial insertion of the deflated balloon into the ventricle and removal of the peel-away sheath, inflation of the balloon with a 1-mL saline injection sealed the tract.
  • One week after the first operation, the balloon was deflated and removed, and the patient underwent tumor resection via the newly formed operative corridor.
  • CONCLUSIONS: Use of balloon dilation for the gradual formation of an operative corridor eliminated the need for additional retraction during SEGA resection, potentially decreasing injury to the surrounding neural tissue.
  • [MeSH-major] Astrocytoma / surgery. Brain Neoplasms / surgery. Catheterization / methods. Craniotomy / methods. Minimally Invasive Surgical Procedures / methods. Tuberous Sclerosis / surgery


29. Kawaguchi T, Kumabe T, Shimizu H, Watanabe M, Tominaga T: 201Tl-SPECT and 1H-MRS study of benign lateral ventricle tumors: differential diagnosis of subependymoma. Neurosurg Rev; 2005 Apr;28(2):96-103
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  • [Title] 201Tl-SPECT and 1H-MRS study of benign lateral ventricle tumors: differential diagnosis of subependymoma.
  • 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.
  • 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.
  • [MeSH-major] Astrocytoma / diagnosis. Cerebral Ventricle Neoplasms / diagnosis. Glioma, Subependymal / diagnosis. Lateral Ventricles. Neurocytoma / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Diagnosis, Differential. Female. Humans. Magnetic Resonance Spectroscopy. Male. Middle Aged. Retrospective Studies. Thallium Radioisotopes. Tomography, Emission-Computed, Single-Photon

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  • (PMID = 15580370.001).
  • [ISSN] 0344-5607
  • [Journal-full-title] Neurosurgical review
  • [ISO-abbreviation] Neurosurg Rev
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Thallium Radioisotopes
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30. Clarke MJ, Foy AB, Wetjen N, Raffel C: Imaging characteristics and growth of subependymal giant cell astrocytomas. Neurosurg Focus; 2006;20(1):E5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Imaging characteristics and growth of subependymal giant cell astrocytomas.
  • OBJECT: Subependymal giant cell astrocytomas (SEGAs) are a common manifestation of tuberous sclerosis (TS).
  • Differentiating SEGAs from subependymal nodules (SENs) before obstruction occurs may improve the morbidity associated with these tumors.
  • Resection was recommended for symptomatic and neuroimaging evidence of hydrocephalus (41%), tumor growth without evidence of hydrocephalus (33%), and for poorly controlled seizures (25%).
  • The mean diameter of the tumors at the time of resection was 1.9 cm (range 0.3-4 cm), and no tumor recurred.
  • Tumor growth and contrast enhancement are the most common signs of progression on neuroimages, and may be seen prior to the development of obstructive hydrocephalus.
  • CONCLUSIONS: Patients with SENs and SEGAs should undergo follow-up neuroimaging at yearly intervals, and if lesions show signs of progression (contrast enhancement or growth), these intervals should be shortened and consideration given to early resection.
  • [MeSH-major] Astrocytoma / pathology. Brain Neoplasms / pathology

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  • (PMID = 16459995.001).
  • [ISSN] 1092-0684
  • [Journal-full-title] Neurosurgical focus
  • [ISO-abbreviation] Neurosurg Focus
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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31. Jozwiak J, Kotulska K, Grajkowska W, Jozwiak S, Zalewski W, Oldak M, Lojek M, Rainko K, Maksym R, Lazarczyk M, Skopinski P, Wlodarski P: Upregulation of the WNT pathway in tuberous sclerosis-associated subependymal giant cell astrocytomas. Brain Dev; 2007 Jun;29(5):273-80
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  • [Title] Upregulation of the WNT pathway in tuberous sclerosis-associated subependymal giant cell astrocytomas.
  • Tuberous sclerosis (TS), autosomal dominant disorder manifested by the formation of usually benign tumors in the brain, heart, kidneys and skin, results from an inactivating mutation in one of two tumor suppressor genes TSC1 or TSC2.
  • Very recently it has been hypothesized that the pathway triggered by WNT, one of embryonic growth factors involved in cell differentiation and migration, could be disturbed in TS.
  • In order to test this hypothesis we evaluated samples of four subependymal giant cell astrocytomas (SEGAs), brain tumors developing in the progress of TS.
  • [MeSH-major] Astrocytoma / complications. Astrocytoma / physiopathology. Brain Neoplasms / complications. Brain Neoplasms / physiopathology. Signal Transduction / physiology. Tuberous Sclerosis / etiology. Tuberous Sclerosis / physiopathology. Up-Regulation / physiology. Wnt Proteins / physiology
  • [MeSH-minor] Blotting, Western. Cell Nucleus / genetics. Cyclin D1 / genetics. Cyclin D1 / physiology. Genes, myc / physiology. Humans. Immunohistochemistry. Nerve Tissue Proteins / metabolism. Phosphorylation. RNA, Messenger / biosynthesis. RNA, Messenger / genetics. Reverse Transcriptase Polymerase Chain Reaction. Transcription, Genetic. Translocation, Genetic / genetics. beta Catenin / biosynthesis. beta Catenin / genetics. beta Catenin / metabolism


32. Ellert-Miklaszewska A, Grajkowska W, Gabrusiewicz K, Kaminska B, Konarska L: Distinctive pattern of cannabinoid receptor type II (CB2) expression in adult and pediatric brain tumors. Brain Res; 2007 Mar 16;1137(1):161-9
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  • Most glioblastomas expressed very high levels of CB2 receptors and the expression correlated with tumor grade.
  • Interestingly, some benign pediatric astrocytic tumors, such as subependymal giant cell astrocytoma (SEGA), which may occasionally cause mortality owing to progressive growth, also displayed high CB2 immunoreactivity.
  • Our results suggest that the CB2 receptor expression depends primarily on the histopathological origin of the brain tumor cells and differentiation state, reflecting the tumor grade.
  • [MeSH-minor] Adolescent. Adult. Age Factors. Astrocytoma / metabolism. Child. Glioblastoma / metabolism. Histocompatibility Antigens / metabolism. Humans. Immunohistochemistry. Reverse Transcriptase Polymerase Chain Reaction / methods

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  • (PMID = 17239827.001).
  • [ISSN] 0006-8993
  • [Journal-full-title] Brain research
  • [ISO-abbreviation] Brain Res.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Histocompatibility Antigens; 0 / Receptor, Cannabinoid, CB2
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33. Ech-Cherif El Kettani N, Salaheddine T, El Quessar A, El Kharras A, El Hassani MR, Chakir N, Boukhrissi N, Benameur M, Jiddane M: [Neuro-imaging of tuberous sclerosis]. J Radiol; 2006 Feb;87(2 Pt 1):109-13
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  • [Transliterated title] Neuro-imagerie de la sclérose tubéreuse de Bourneville.
  • Tuberous sclerosis is a phakomatosis with central nervous system manifestations characterized by 4 lesions detectable on neuro-imaging: tubers, white matter abnormalities, subependymal nodules and subependymal astrocytomas.
  • The first three are benign lesions composed of by identical cytological lesions varying only in size and location.
  • At CT, enhancement of subependymal nodules is usually considered as evidence of transformation to subependymal giant cell astrocytoma.
  • [MeSH-major] Tuberous Sclerosis / diagnosis

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  • (PMID = 16484932.001).
  • [ISSN] 0221-0363
  • [Journal-full-title] Journal de radiologie
  • [ISO-abbreviation] J Radiol
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] France
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34. Scarabino T, Giannatempo GM, Nemore F, Popolizio T, Stranieri A: Supratentorial low-grade gliomas. Neuroradiology. J Neurosurg Sci; 2005 Sep;49(3):73-6
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  • A brain tumors can be reliably ruled out, if the standard magnetic resonance examination is performed properly and experts interpret the results as negative for tumor.
  • In this paper we will illustrate morphological aspects of low-grade supratentorial neoplasms, including tumors of neuroepithelial tissue, such as low-grade diffuse fibrillary astrocytomas, and circumscribed astrocytic lesions (pilocytic astrocytoma, pleomorphic xantoastrocytoma and subependymal giant cell astrocytoma).

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  • (PMID = 16288189.001).
  • [ISSN] 0390-5616
  • [Journal-full-title] Journal of neurosurgical sciences
  • [ISO-abbreviation] J Neurosurg Sci
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Italy
  • [Number-of-references] 14
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35. Tay A, Scheithauer BW, Cameron JD, Myhre MJ, Boerner MJ: Retinal ependymoma: an immunohistologic and ultrastructural study. Hum Pathol; 2009 Apr;40(4):578-83
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  • Most are syndrome associated and include pilocytic astrocytoma in neurofibromatosis type 1 and subependymal giant cell astrocytoma in tuberous sclerosis complex.
  • The tumor was sporadic in occurrence and unilateral, low grade, and of cellular type.
  • Its chronic course and large size prompted an initial pathologic diagnosis of "massive retinal gliosis."

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  • (PMID = 18835620.001).
  • [ISSN] 1532-8392
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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36. Arvanitis LD, Koukoulis GK, Kanavaros P: The expression of the O-linked N-acetylglucosamine containing epitope H in the gemistocytic, pilocytic and subependymal giant cell astrocytomas. Oncol Rep; 2009 Sep;22(3):521-4
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  • [Title] The expression of the O-linked N-acetylglucosamine containing epitope H in the gemistocytic, pilocytic and subependymal giant cell astrocytomas.
  • In this study the expression of the epitope H was investigated in thirty cases of gemistocytic (WHO grade II), pilocytic (WHO grade I), and subependymal giant cell (WHO grade I) astrocytomas using the mAbH with the indirect immunoperoxidase method.
  • The dense tumor areas composed of elongated pilocytic cells revealed high expression of the epitope H.
  • The loose cystic tumor areas composed of stellate cells revealed low expression of the epitope H.
  • The ten cases of subependynal giant cell astrocytomas occurring in tuberous sclerosis revealed an overall high expression pattern.
  • This study shows that there is high expression of the epitope H in gemistocytic, pilocytic and subependymal giant cell astrocytomas.
  • [MeSH-major] Acetylglucosamine / analysis. Astrocytoma / chemistry. Brain Neoplasms / chemistry. Epitopes

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  • (PMID = 19639198.001).
  • [ISSN] 1021-335X
  • [Journal-full-title] Oncology reports
  • [ISO-abbreviation] Oncol. Rep.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Epitopes; V956696549 / Acetylglucosamine
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37. Murai N, Kaneko T: [Case of jugular foramen schwannoma associated with tuberous sclerosis]. No Shinkei Geka; 2007 Oct;35(10):1007-11
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  • Her tuberous sclerosis was diagnosed at the age of 9, when she developed hydrocephalus caused by subependymal giant cell astrocytoma near the foramen of Monro, which was treated by surgical resection and VP shunt placement followed by radiation and chemotherapy.
  • Brain MR images revealed a 3 cm enhancing mass extending from the left jugular foramen to the cerebellopontine cistern, accompanied by perifocal edema of the brain stem and cerebellar hemisphere.
  • The tumor was partially removed via suboccipital craniotomy and histologically diagnosed as a schwannoma.
  • No tumor regrowth has been observed at 94 months.
  • [MeSH-minor] Adult. Astrocytoma / etiology. Astrocytoma / therapy. Cerebral Ventricle Neoplasms / etiology. Cerebral Ventricle Neoplasms / therapy. Cerebral Ventricles. Female. Humans. Hydrocephalus / etiology. Treatment Outcome

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  • (PMID = 17969337.001).
  • [ISSN] 0301-2603
  • [Journal-full-title] No shinkei geka. Neurological surgery
  • [ISO-abbreviation] No Shinkei Geka
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
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38. Dashti SR, Robinson S, Rodgers M, Cohen AR: Pineal region giant cell astrocytoma associated with tuberous sclerosis: case report. J Neurosurg; 2005 Apr;102(3 Suppl):322-5
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  • [Title] Pineal region giant cell astrocytoma associated with tuberous sclerosis: case report.
  • Tuberous sclerosis complex is a genetic disorder characterized by the development of hamartomas in multiple organs including the brain, skin, eye, kidney, and heart.
  • Cortical tubers and subependymal nodules are the characteristic intracranial lesions of tuberous sclerosis.
  • Subependymal giant cell astrocytomas, typically located adjacent to the foramen of Monro, can enlarge and cause symptomatic ventricular obstruction.
  • The authors describe the case of a 3-year-old boy with a history of tuberous sclerosis and retinal lesions who presented with an enlarging enhancing pineal region mass.
  • Via an infratentorial supracerebellar approach, the mass was removed using both the operative microscope and a rigid neuroendoscope.
  • Pathological examination showed a giant cell astrocytoma.
  • To the authors' knowledge, this is the first reported case of tuberous sclerosis associated with a giant cell astrocytoma of the pineal region.
  • [MeSH-major] Astrocytoma / complications. Astrocytoma / surgery. Pinealoma / complications. Pinealoma / surgery. Tuberous Sclerosis / complications
  • [MeSH-minor] Cell Transformation, Neoplastic / pathology. Child, Preschool. Eye Abnormalities / complications. Eye Abnormalities / diagnosis. Eye Abnormalities / genetics. Follow-Up Studies. Humans. Image Enhancement. Magnetic Resonance Imaging. Male. Microsurgery. Neuroendoscopes. Neurologic Examination. Pineal Gland / pathology. Pineal Gland / surgery. Postoperative Complications / diagnosis

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  • (PMID = 15881760.001).
  • [ISSN] 0022-3085
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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39. Kidd EA, Mansur DB, Leonard JR, Michalski JM, Simpson JR, Perry A: The efficacy of radiation therapy in the management of grade I astrocytomas. J Neurooncol; 2006 Jan;76(1):55-8
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  • INTRODUCTION: The purpose of this study was to analyze the outcome of patients with grade I astrocytomas treated with radiation therapy, specifically looking at the prognostic significance of age, timing of radiation therapy (immediately after surgery or delayed until progression) and tumor location.
  • Twenty patients with grade I pilocytic astrocytoma (n=19) or subependymal giant cell astrocytoma (n=1) were treated with radiation therapy with curative intent.
  • All tumor recurrences were local.
  • [MeSH-major] Astrocytoma / radiotherapy. Brain Neoplasms / radiotherapy. Neoplasm Recurrence, Local

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  • (PMID = 16132503.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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40. Boada M, Gómez E, Puig J, Pedraza S: [Intraventricular fibrous tumor: a case report]. Radiologia; 2009 Sep-Oct;51(5):512-5
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  • [Title] [Intraventricular fibrous tumor: a case report].
  • [Transliterated title] Tumor fibroso intraventricular: a propósito de un caso.
  • Solitary fibrous tumor was first reported in the pleura; however, it has since been reported in many other locations.
  • To our knowledge, only eight cases of intraventricular solitary fibrous tumor have been reported.
  • We describe a case of intraventricular solitary fibrous tumor.The imaging characteristics of intraventricular solitary fibrous tumors are nonspecific; the differential diagnosis should include other tumors that can affect the ventricular system such as meningioma, high grade glioma, metastasis, subependymoma, choroid plexus papilloma, ependymoma, subependymal giant cell astrocytoma, and neurocytoma.
  • At histological study, immunohistochemical techniques allow solitary fibrous tumor to be differentiated from fibrous meningioma and hemangiopericytoma.

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  • (PMID = 19646726.001).
  • [ISSN] 0033-8338
  • [Journal-full-title] Radiología
  • [ISO-abbreviation] Radiologia
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
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41. Ohgaki H, Kim YH, Steinbach JP: Nervous system tumors associated with familial tumor syndromes. Curr Opin Neurol; 2010 Dec;23(6):583-91
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  • [Title] Nervous system tumors associated with familial tumor syndromes.
  • Signaling pathways involved in hereditary syndromes predisposing to the development of nervous system tumors include RAS, WNT, RB1, TP53, and PTCH signaling pathways, which play key roles in gene regulation, apoptosis, and cell proliferation.
  • We also briefly review two important advances in this area: the treatment of medulloblastomas in patients with mutations in the PTCH1 gene, and the discovery of deregulated mammalian target of rapamycin as a major oncogenic driver molecule in patients with TSC mutations and subependymal giant cell astrocytoma.
  • SUMMARY: Progress in the understanding of hereditary nervous system tumors is increasingly important for diagnosis and treatment.
  • [MeSH-major] Genetic Diseases, Inborn / diagnosis. Genetic Diseases, Inborn / genetics. Genetic Predisposition to Disease / genetics. Nervous System Neoplasms / diagnosis. Nervous System Neoplasms / genetics
  • [MeSH-minor] Child. Humans. Medulloblastoma / diagnosis. Medulloblastoma / genetics. Medulloblastoma / metabolism. Signal Transduction / genetics. Syndrome. Tuberous Sclerosis / diagnosis. Tuberous Sclerosis / genetics. Tuberous Sclerosis / metabolism


42. Birca A, Mercier C, Major P: Rapamycin as an alternative to surgical treatment of subependymal giant cell astrocytomas in a patient with tuberous sclerosis complex. J Neurosurg Pediatr; 2010 Oct;6(4):381-4
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  • [Title] Rapamycin as an alternative to surgical treatment of subependymal giant cell astrocytomas in a patient with tuberous sclerosis complex.
  • Tuberous sclerosis complex (TSC) is associated with the potential development of benign hamartomas, including subependymal giant cell astrocytomas (SEGAs).
  • Three months after initiating rapamycin therapy, the SEGAs exhibited significant reduction in size (82.6% on the left and 46.7% on the right), and the lesions remained stable 5 months later.
  • [MeSH-major] Antibiotics, Antineoplastic / administration & dosage. Astrocytoma / drug therapy. Brain Neoplasms / drug therapy. Sirolimus / administration & dosage. Tuberous Sclerosis / complications


43. Pollock JM, Whitlow CT, Tan H, Kraft RA, Burdette JH, Maldjian JA: Pulsed arterial spin-labeled MR imaging evaluation of tuberous sclerosis. AJNR Am J Neuroradiol; 2009 Apr;30(4):815-20
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  • BACKGROUND AND PURPOSE: Tuberous sclerosis presents with characteristic cortical hamartomas and subependymal nodules associated with seizures.
  • One patient had a subependymal giant cell astrocytoma with a mean perfusion of 93.5 mL/100 g tissue/min.
  • Most lesions are hypoperfused; however, both normally perfused and hyperperfused lesions occur.
  • [MeSH-minor] Adolescent. Astrocytoma / etiology. Astrocytoma / pathology. Brain Diseases / complications. Brain Diseases / pathology. Brain Neoplasms / etiology. Brain Neoplasms / pathology. Child. Child, Preschool. Disease Progression. Hamartoma / complications. Hamartoma / pathology. Humans. Infant. Retrospective Studies. Young Adult

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  • (PMID = 19147711.001).
  • [ISSN] 1936-959X
  • [Journal-full-title] AJNR. American journal of neuroradiology
  • [ISO-abbreviation] AJNR Am J Neuroradiol
  • [Language] eng
  • [Grant] United States / NIBIB NIH HHS / EB / EB004673
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Spin Labels
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44. O'Callaghan FJ, Martyn CN, Renowden S, Noakes M, Presdee D, Osborne JP: Subependymal nodules, giant cell astrocytomas and the tuberous sclerosis complex: a population-based study. Arch Dis Child; 2008 Sep;93(9):751-4
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  • [Title] Subependymal nodules, giant cell astrocytomas and the tuberous sclerosis complex: a population-based study.
  • OBJECTIVES:. (1) In a population-based study of tuberous sclerosis (TSC), to identify the number of patients presenting with symptomatic giant cell astrocytomas (GCAs);.
  • Thirty-nine of these had subependymal nodules, of whom 24 (59%) had at least one (maximum 11) that showed enhancement with gadolinium.
  • In seven (17%), the lesion was >1 cm, and all of these lesions showed gadolinium enhancement.
  • [MeSH-major] Astrocytoma / epidemiology. Brain Neoplasms / epidemiology. Tuberous Sclerosis / epidemiology
  • [MeSH-minor] Adolescent. Adult. Child. Cross-Sectional Studies. Female. Humans. Intracranial Hypertension / etiology. Intracranial Hypertension / surgery. Magnetic Resonance Imaging. Male. Mental Disorders / etiology. Tomography, X-Ray Computed. Tumor Suppressor Proteins / metabolism

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  • [CommentIn] Arch Dis Child. 2009 Jan;94(1):75-6 [19103791.001]
  • (PMID = 18456692.001).
  • [ISSN] 1468-2044
  • [Journal-full-title] Archives of disease in childhood
  • [ISO-abbreviation] Arch. Dis. Child.
  • [Language] eng
  • [Grant] United Kingdom / Wellcome Trust / /
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Tumor Suppressor Proteins
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45. Jozwiak J, Jozwiak S, Wlodarski P: Possible mechanisms of disease development in tuberous sclerosis. Lancet Oncol; 2008 Jan;9(1):73-9
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  • Tumours in which loss of heterozygosity is rare, such as subependymal giant-cell astrocytoma, might all share a common feature that mimics loss of heterozygosity either by inactivation of the TSC complex or by direct activation of mammalian target of rapamycin (mTOR) or its downstream targets.
  • AKT activation is detected only in few samples, whereas ERK is hyperactive in all subependymal giant-cell astrocytomas.


46. Szeliga M, Matyja E, Obara M, Grajkowska W, Czernicki T, Albrecht J: Relative expression of mRNAS coding for glutaminase isoforms in CNS tissues and CNS tumors. Neurochem Res; 2008 May;33(5):808-13

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  • [Title] Relative expression of mRNAS coding for glutaminase isoforms in CNS tissues and CNS tumors.
  • The GAC mRNA/KGA mRNA expression ratio was as a rule higher in the neoplastic than in control tissues, irrespective of the cell type dominating in the tumor or tumor malignancy.
  • LGA mRNA expression was relatively very low in cultured astrocytes, and very low to absent in astrocytoma pilocyticum, ependymoma and subependymal giant cell astrocytoma (SEGA), tumors of astrocytic origin.
  • The results show that low expression of LGA mRNA is a feature common to normal astrocytes and astroglia-derived tumor cells or ependymomas and can be considered as a cell-type, rather than a malignancy marker.

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  • (PMID = 17940881.001).
  • [ISSN] 0364-3190
  • [Journal-full-title] Neurochemical research
  • [ISO-abbreviation] Neurochem. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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47. Pipp I, Wagner L, Rössler K, Budka H, Preusser M: Secretagogin expression in tumours of the human brain and its coverings. APMIS; 2007 Apr;115(4):319-26
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  • We found focal or widespread secretagogin expression in tumour cells in 1/18 oligoastrocytomas, 1/19 oligodendrogliomas, 2/20 anaplastic oligodendrogliomas, 2/9 ependymomas, 2/11 anaplastic ependymomas, 2/10 glioblastomas, 3/11 gangliogliomas and 1/2 anaplastic gangliogliomas, 10/10 central neurocytomas, 5/10 classic medulloblastomas, 4/5 desmoplastic medulloblastomas, 3/5 large cell/anaplastic medulloblastomas, 3/5 neuroblastomas, 3/10 meningiomas, 2/10 haemangioblastomas, and 13/19 pituitary adenomas.
  • We detected no secretagogin expression in fibrillary astrocytoma, pilocytic astrocytoma, DNT, pineocytoma, pineoblastoma, subependymal giant cell astrocytoma (SEGA), atypical teratoid/rhabdoid tumour (AT/RT), or primary central nervous system lymphoma (PCNSL).
  • [MeSH-major] Biomarkers, Tumor / analysis. Brain Neoplasms / diagnosis. Calcium-Binding Proteins / analysis

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  • (PMID = 17504298.001).
  • [ISSN] 0903-4641
  • [Journal-full-title] APMIS : acta pathologica, microbiologica, et immunologica Scandinavica
  • [ISO-abbreviation] APMIS
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Calcium-Binding Proteins; 0 / SCGN protein, human; 0 / Secretagogins
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48. Pinto Gama HP, da Rocha AJ, Braga FT, da Silva CJ, Maia AC Jr, de Campos Meirelles RG, Mendonça do Rego JI, Lederman HM: Comparative analysis of MR sequences to detect structural brain lesions in tuberous sclerosis. Pediatr Radiol; 2006 Feb;36(2):119-25
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Comparative analysis of MR sequences to detect structural brain lesions in tuberous sclerosis.
  • MR is the imaging method of choice to demonstrate structural brain lesions in TS.
  • MATERIALS AND METHODS: We reviewed MR scans of 18 TS patients for the presence of cortical tubers, white matter lesions (radial bands), subependymal nodules, and subependymal giant cell astrocytoma (SGCA) on the following sequences:.
  • The T1 SE/MTC sequence was far superior to other methods in detecting white matter lesions (P<0.01).
  • There was no significant difference between the T1 SE/MTC and T1 SE (before and after Gd injection) sequences in the detection of subependymal nodules; FLAIR sequence showed less sensitivity than the others in identifying the nodules.
  • CONCLUSION: We demonstrated the importance of appropriate MRI sequences for diagnosis of the most frequent brain lesions in TS.
  • Gd injection might be useful in detecting SGCA; however, the parameters of size and location are also important for a presumptive diagnosis of these tumors.
  • [MeSH-major] Brain / pathology. Cerebral Cortex / pathology. Magnetic Resonance Imaging. Tuberous Sclerosis / diagnosis

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  • (PMID = 16283285.001).
  • [ISSN] 0301-0449
  • [Journal-full-title] Pediatric radiology
  • [ISO-abbreviation] Pediatr Radiol
  • [Language] eng
  • [Publication-type] Comparative Study; Evaluation Studies; Journal Article
  • [Publication-country] Germany
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49. Ishida Y, Kori A, Ishihama A: Participation of regulator AscG of the beta-glucoside utilization operon in regulation of the propionate catabolism operon. J Bacteriol; 2009 Oct;191(19):6136-44
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  • [Title] Participation of regulator AscG of the beta-glucoside utilization operon in regulation of the propionate catabolism operon.
  • The ascFB genes for beta-D-galactoside transport and catabolism are repressed by the AscG regulator.
  • After genomic SELEX screening, AscG was found to recognize and bind the consensus palindromic sequence TGAAACC-GGTTTCA.
  • AscG binding was detected at two sites upstream of the ascFB promoter and at three sites upstream of the prpBC operon for propionate catabolism.
  • In an ascG-disrupted mutant, transcription of ascFB was enhanced, in agreement with the repressor model of AscG.
  • This repression was indicated to be due to interference of binding of cyclic AMP-CRP to the CRP box, which overlaps with the AscG-binding site 1, as well as binding of RNA polymerase to the promoter.
  • Under conditions of steady-state E. coli growth in a rich medium, the intracellular level of AscG stayed constant at a level supposedly leading to tight repression of the ascFB operon.
  • The level of prpR, encoding the activator of prpBCDE, was also increased in the absence of AscG, indicating the involvement of AscG in repression of prpR.


50. Fisher PG, Tihan T, Goldthwaite PT, Wharam MD, Carson BS, Weingart JD, Repka MX, Cohen KJ, Burger PC: Outcome analysis of childhood low-grade astrocytomas. Pediatr Blood Cancer; 2008 Aug;51(2):245-50
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  • All available pathology specimens were reviewed, masked to original diagnosis, patient data, and neuroimaging.
  • RESULTS: Two hundred seventy-eight children (160 males; mean age 9.1 years; tumor location: 77 cerebrum, 62 cerebellum, 51 hypothalamic, 30 thalamus, 9 ventricle, 40 brainstem, and 9 spine) were assessed.
  • Among 246 specimens reviewed, diagnoses were 135 pilocytic astrocytoma (PA), 27 diffuse astrocytoma (DA), 75 unclassifiable well-differentiated astrocytoma (NOS), and 9 subependymal giant cell astrocytoma.
  • Among patients with residual tumor after surgery, 5-year PFS was 48% with observation alone (n = 114), no different (P = 0.32) from that achieved with immediate irradiation (n = 86).
  • While tumor location and resection extent affect outcome, pathologic diagnosis when carefully interpreted significantly influences long-term survival.
  • [MeSH-major] Astrocytoma / mortality. Brain Neoplasms / mortality

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  • (PMID = 18386785.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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51. Tan YW, Yu HB, Leung KY, Sivaraman J, Mok YK: Structure of AscE and induced burial regions in AscE and AscG upon formation of the chaperone needle-subunit complex of type III secretion system in Aeromonas hydrophila. Protein Sci; 2008 Oct;17(10):1748-60
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  • [Title] Structure of AscE and induced burial regions in AscE and AscG upon formation of the chaperone needle-subunit complex of type III secretion system in Aeromonas hydrophila.
  • In the type III secretion system (T3SS) of Aeromonas hydrophila, the putative needle complex subunit AscF requires both putative chaperones AscE and AscG for formation of a ternary complex to avoid premature assembly.
  • Here we report the crystal structure of AscE at 2.7 A resolution and the mapping of buried regions of AscE, AscG, and AscF in the AscEG and AscEFG complexes using limited protease digestion.
  • The N-terminal 13 residues of AscE are buried only upon binding with AscG, but this region is found to be nonessential for the interaction.
  • AscE functions as a monomer and can be coexpressed with AscG or with both AscG and AscF to form soluble complexes.
  • The AscE binding region of AscG in the AscEG complex is identified to be within the N-terminal 61 residues of AscG.
  • The exposed C-terminal substrate-binding region of AscG in the AscEG complex is induced to be buried only upon binding to AscF.

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  • (PMID = 18662905.001).
  • [ISSN] 1469-896X
  • [Journal-full-title] Protein science : a publication of the Protein Society
  • [ISO-abbreviation] Protein Sci.
  • [Language] eng
  • [Databank-accession-numbers] PDB/ 2Q1K
  • [Grant] United States / NIBIB NIH HHS / EB / P30 EB009998
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Bacterial Proteins; 0 / Molecular Chaperones; 0 / Protein Subunits
  • [Other-IDs] NLM/ PMC2548367
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52. Chiarugi M, Martino MC, Pucciarelli M, Decanini L, Vignali C: Recurrent retroperitoneal hemorrhage in a patient with tuberous sclerosis complex: a case report. Cases J; 2008;1(1):424
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  • BACKGROUND: Tuberous sclerosis complex (TSC) is an autosomal dominant disorder.
  • It is characterized by seizures, mental retardation and hamartomatous lesions, including facial angiofibroma, subependymal giant cell astrocytoma, cardiac rhabdomyoma and renal angiomyolipoma (AML).

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  • (PMID = 19115994.001).
  • [ISSN] 1757-1626
  • [Journal-full-title] Cases journal
  • [ISO-abbreviation] Cases J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2636789
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53. Timotin L, Sarrot-Reynauld F, Lantuejoul S, Pasquier B, Massot C, Ashraf A, Borgel F: [Tuberous sclerosis without mental impairment, diagnosed in adulthood]. Rev Med Interne; 2005 Jun;26(6):511-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Transliterated title] Sclérose tubéreuse de Bourneville sans altération intellectuelle, diagnostiquée à l'âge adulte.
  • EXEGESIS: We report a case of TSC diagnosed in a 33-year-old man, without any known family history of phakomatosis, presenting with facial angiofibromas, hypomelanotic macules, a giant-cell astrocytoma and retinal phakomas without any mental impairment or epilepsy.
  • CONCLUSION: TSC may occur in patients who do not have any family history of phakomatosis because de novo mutations are frequent.
  • They should lead to brain imaging in search for astrocytoma, subependymal nodules and cortical tubers which number is directly correlated with the risk of seizures and the degree of mental impairment.

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  • (PMID = 15936480.001).
  • [ISSN] 0248-8663
  • [Journal-full-title] La Revue de medecine interne
  • [ISO-abbreviation] Rev Med Interne
  • [Language] FRE
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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54. Wu CJ, Wu JJ, Yan JJ, Lee HC, Lee NY, Chang CM, Shih HI, Wu HM, Wang LR, Ko WC: Clinical significance and distribution of putative virulence markers of 116 consecutive clinical Aeromonas isolates in southern Taiwan. J Infect; 2007 Feb;54(2):151-8
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  • All isolates were identified by biochemical phenotyping and their genomic sequences encoding eight putative virulence factors, including cytolytic enterotoxin (AHCYTOEN), aerolysin (aerA), hemolysin (hlyA), heat-labile enterotoxin (alt), heat-stable enterotoxin (ast), and components of type III secretion system (ascV, aexT and ascF-ascG) were analyzed using polymerase chain reaction and colony blot hybridization.
  • This study found no association between the presence of AHCYTOEN, aerA, hlyA, alt, ast, ascV, aexT or ascF-ascG genes in Aeromonas isolates and the development of extra-intestinal infections or bacteremia.

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  • (PMID = 16716402.001).
  • [ISSN] 1532-2742
  • [Journal-full-title] The Journal of infection
  • [ISO-abbreviation] J. Infect.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Bacterial Proteins; 0 / Virulence Factors
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55. Wen PY: American Society of Clinical Oncology 2010: report of selected studies from the CNS tumors section. Expert Rev Anticancer Ther; 2010 Sep;10(9):1367-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] American Society of Clinical Oncology 2010: report of selected studies from the CNS tumors section.
  • A number of important studies were presented at the CNS tumors section of the 2010 American Society of Oncology (ASCO) Annual Meeting.
  • There was particular interest in a Phase II study showing that the mTOR inhibitor everolimus had significant activity in tuberous patients with subependymal giant cell astrocytomas.
  • [MeSH-major] Astrocytoma / drug therapy. Central Nervous System Neoplasms / drug therapy. Immunosuppressive Agents / therapeutic use. Sirolimus / analogs & derivatives. Tuberous Sclerosis / drug therapy

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  • (PMID = 20836670.001).
  • [ISSN] 1744-8328
  • [Journal-full-title] Expert review of anticancer therapy
  • [ISO-abbreviation] Expert Rev Anticancer Ther
  • [Language] eng
  • [Publication-type] Clinical Conference; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Immunosuppressive Agents; 9HW64Q8G6G / Everolimus; W36ZG6FT64 / Sirolimus
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56. Henderson MA, Fakiris AJ, Timmerman RD, Worth RM, Lo SS, Witt TC: Gamma knife stereotactic radiosurgery for low-grade astrocytomas. Stereotact Funct Neurosurg; 2009;87(3):161-7
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  • Patients with low-grade astrocytoma (LGA; 8 pilocytic astrocytomas, 2 subependymal giant cell astrocytomas, 2 fibrillary astrocytomas) were selected for treatment with gamma knife stereotactic radiosurgery (GKSRS) based on having a demarcated appearance on CT or MRI and the possibility of dose sparing of adjacent eloquent structures.
  • A median dose of 13 Gy was prescribed to the 50% isodose line, which covered the gross tumor.
  • With a median follow-up of 48.2 months, 4-year tumor control and overall survival were 77 and 83%, respectively.
  • [MeSH-major] Astrocytoma / surgery. Brain Neoplasms / surgery. Radiosurgery / methods

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  • [Copyright] 2009 S. Karger AG, Basel.
  • (PMID = 19321969.001).
  • [ISSN] 1423-0372
  • [Journal-full-title] Stereotactic and functional neurosurgery
  • [ISO-abbreviation] Stereotact Funct Neurosurg
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Switzerland
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57. Balss J, Meyer J, Mueller W, Korshunov A, Hartmann C, von Deimling A: Analysis of the IDH1 codon 132 mutation in brain tumors. Acta Neuropathol; 2008 Dec;116(6):597-602
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  • We analyzed the genomic region spanning wild type R132 of IDH1 by direct sequencing in 685 brain tumors including 41 pilocytic astrocytomas, 12 subependymal giant cell astrocytomas, 7 pleomorphic xanthoastrocytomas, 93 diffuse astrocytomas, 120 adult glioblastomas, 14 pediatric glioblastomas, 105 oligodendrogliomas, 83 oligoastrocytomas, 31 ependymomas, 58 medulloblastomas, 9 supratentorial primitive neuroectodermal tumors, 17 schwannomas, 72 meningiomas and 23 pituitary adenomas.
  • The very high frequency of IDH1 mutations in WHO grade II astrocytic and oligodendroglial gliomas suggests a role in early tumor development.
  • [MeSH-minor] Astrocytoma / genetics. Astrocytoma / pathology. Base Sequence. DNA Mutational Analysis. Disease Progression. Gene Frequency. Glioblastoma / genetics. Glioblastoma / pathology. Glioma / etiology. Glioma / pathology. Humans. Oligodendroglioma / genetics. Oligodendroglioma / pathology. Polymerase Chain Reaction

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  • (PMID = 18985363.001).
  • [ISSN] 1432-0533
  • [Journal-full-title] Acta neuropathologica
  • [ISO-abbreviation] Acta Neuropathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] EC 1.1.1.41 / Isocitrate Dehydrogenase
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58. Walsh JK, Deacon S, Dijk DJ, Lundahl J: The selective extrasynaptic GABAA agonist, gaboxadol, improves traditional hypnotic efficacy measures and enhances slow wave activity in a model of transient insomnia. Sleep; 2007 May;30(5):593-602
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  • STUDY OBJECTIVES: Evaluate the hypnotic efficacy of gaboxadol, a selective extrasynaptic GABAA agonist (SEGA), in a phase advance model of transient insomnia.

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  • (PMID = 17552374.001).
  • [ISSN] 0161-8105
  • [Journal-full-title] Sleep
  • [ISO-abbreviation] Sleep
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / GABA Agonists; 0 / GABA-A Receptor Agonists; 0 / Hypnotics and Sedatives; 0 / Isoxazoles; 0 / Pyridines; 7K383OQI23 / zolpidem; K1M5RVL18S / gaboxadol
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59. Gallagher A, Grant EP, Madan N, Jarrett DY, Lyczkowski DA, Thiele EA: MRI findings reveal three different types of tubers in patients with tuberous sclerosis complex. J Neurol; 2010 Aug;257(8):1373-81
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  • Patients with Type C tuber dominance have more MRI abnormalities such as subependymal giant cell tumors, and were more likely to have an autism spectrum disorder, a history of infantile spasms, and a higher frequency of epileptic seizures, compared to patients who have a dominance in Type B tubers, and especially to those with a Type A dominance.

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  • (PMID = 20352250.001).
  • [ISSN] 1432-1459
  • [Journal-full-title] Journal of neurology
  • [ISO-abbreviation] J. Neurol.
  • [Language] ENG
  • [Grant] United States / NINDS NIH HHS / NS / P01 NS024279; None / None / / P01 NS024279-23; United States / NINDS NIH HHS / NS / P01 NS024279-23; Canada / Canadian Institutes of Health Research / /
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Other-IDs] NLM/ NIHMS279080; NLM/ PMC3075858
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60. Osztie E, Hanzély Z, Afra D: Lateral ventricle gliomas and central neurocytomas in adults diagnosis and perspectives. Eur J Radiol; 2009 Jan;69(1):67-73
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Lateral ventricle gliomas and central neurocytomas in adults diagnosis and perspectives.
  • Neuroimaging data of lateral ventricle gliomas and central neurocytomas diagnosed in one institution were reviewed and compared to the corresponding literature data.
  • Eight subependymal giant cell astrocytomas also displayed hypodense, rarely hyperdense or mixed imaging characteristics, and always showed significant degree of contrast enhancement.
  • Our data support those of previous studies in that MRI has been found to be superior to CT for a more precise imaging of lateral ventricle gliomas.
  • Survival data were available in 65 cases, which have confirmed a favourable outcome in most of the patients with subependymoma, subependymal giant cell astrocytoma, central neurocytomas or pilocytic astrocytoma.
  • [MeSH-major] Cerebral Ventricle Neoplasms / diagnosis. Glioma / diagnosis. Magnetic Resonance Imaging / methods. Neurocytoma / diagnosis. Tomography, X-Ray Computed / methods

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  • (PMID = 18023315.001).
  • [ISSN] 1872-7727
  • [Journal-full-title] European journal of radiology
  • [ISO-abbreviation] Eur J Radiol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Ireland
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61. Holveck A, Grand S, Boini S, Kirchin M, Le Bas JF, Dietemann JL, Bracard S, Kremer S: Dynamic susceptibility contrast-enhanced MRI evaluation of cerebral intraventricular tumors: preliminary results. J Neuroradiol; 2010 Dec;37(5):269-75
MedlinePlus Health Information. consumer health - MRI Scans.

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • INTRODUCTION: The aims of the present study were to determine the perfusion characteristics of several types of intraventricular tumors and to evaluate the usefulness of dynamic contrast-enhanced MRI in making the differential diagnosis.
  • METHODS: A total of 28 patients with intraventricular tumors (five meningiomas, five papillomas, three ependymomas, four subependymomas, seven central neurocytomas, two subependymal giant cell astrocytomas and two metastases) underwent conventional and dynamic susceptibility contrast-enhanced MRI.
  • Cerebral blood volume (CBV) maps were obtained and the relative CBV (rCBV) calculated for each tumor.
  • In cases of suspected meningioma, papilloma or neurocytoma, low rCBV values (<3) point to a diagnosis of neurocytoma rather than either of the other tumor types.
  • CONCLUSION: Susceptibility contrast-enhanced MRI can provide additional information on the vascularization of intraventricular cerebral tumors and may help in making the differential diagnosis.
  • [MeSH-major] Astrocytoma / pathology. Cerebral Ventricle Neoplasms / pathology. Ependymoma / pathology. Magnetic Resonance Imaging / methods. Meningioma / pathology. Neurocytoma / pathology. Papilloma / pathology

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  • [Copyright] Copyright © 2010 Elsevier Masson SAS. All rights reserved.
  • (PMID = 20435349.001).
  • [ISSN] 0150-9861
  • [Journal-full-title] Journal of neuroradiology. Journal de neuroradiologie
  • [ISO-abbreviation] J Neuroradiol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Contrast Media
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62. Wong KK, Tsang YT, Chang YM, Su J, Di Francesco AM, Meco D, Riccardi R, Perlaky L, Dauser RC, Adesina A, Bhattacharjee M, Chintagumpala M, Lau CC: Genome-wide allelic imbalance analysis of pediatric gliomas by single nucleotide polymorphic allele array. Cancer Res; 2006 Dec 1;66(23):11172-8
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  • Most of the low-grade gliomas had no detectable loss of heterozygosity (LOH) in any of the 11,562 SNP loci; exceptions were two gangliogliomas (3q and 9p), one astrocytoma (6q), and two subependymal giant cell astrocytomas (16p and 21q).
  • This may represent a new molecular mechanism underlying tumor progression.

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  • (PMID = 17145861.001).
  • [ISSN] 0008-5472
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / R21 CA120534
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] EC 2.7.10.1 / Receptor, Epidermal Growth Factor; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor alpha
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63. Franz DN, Leonard J, Tudor C, Chuck G, Care M, Sethuraman G, Dinopoulos A, Thomas G, Crone KR: Rapamycin causes regression of astrocytomas in tuberous sclerosis complex. Ann Neurol; 2006 Mar;59(3):490-8
The Lens. Cited by Patents in .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OBJECTIVE: Tuberous sclerosis complex (TSC) is a genetic disorder characterized by the formation of hamartomas in multiple organs.
  • Five to 15% of affected individuals display subependymal giant cell astrocytomas, which can lead to substantial neurological and postoperative morbidity due to the production of hydrocephalus, mass effect, and their typical location adjacent to the foramen of Monro.
  • METHODS: Five subjects with clinically definite TSC and either subependymal giant cell astrocytomas (n = 4) or a pilocytic astrocytoma (n = 1) were treated with oral rapamycin at standard immunosuppressive doses (serum levels 5-15 ng/ml) from 2.5 to 20 months.
  • All lesions demonstrated growth on serial neuroimaging studies.
  • RESULTS: All lesions exhibited regression and, in one case, necrosis.
  • Interruption of therapy resulted in regrowth of subependymal giant cell astrocytomas in one patient.
  • INTERPRETATION: Oral rapamycin therapy can induce regression of astrocytomas associated with TSC and may offer an alternative to operative therapy of these lesions.
  • [MeSH-major] Astrocytoma / drug therapy. Astrocytoma / etiology. Brain Neoplasms / drug therapy. Immunosuppressive Agents. Regression (Psychology). Sirolimus / therapeutic use. Tuberous Sclerosis / complications


64. Lam C, Bouffet E, Tabori U, Mabbott D, Taylor M, Bartels U: Rapamycin (sirolimus) in tuberous sclerosis associated pediatric central nervous system tumors. Pediatr Blood Cancer; 2010 Mar;54(3):476-9
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  • Tuberous sclerosis complex (TSC) is associated with hamartomatous growths including subependymal giant cell astrocytomas (SEGAs).
  • We summarize our institutional experience and literature review that highlight potential benefits and hazards of rapamycin therapy, for TSC patients with SEGA, and other syndromal brain tumors.

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  • [Copyright] Copyright 2009 Wiley-Liss, Inc.
  • (PMID = 19856393.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; W36ZG6FT64 / Sirolimus
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65. Jufe GS: [New hypnotics: perspectives from sleep physiology]. Vertex; 2007 Jul-Aug;18(74):294-9
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  • Insomnia is a disorder that affects a large part of the population.
  • Agents that are used to treat this sleep disorder have evolved: benzodiazepines replaced barbiturates, but there is still concern about their residual effects and about the development of dependence and the risk of withdrawal symptoms.
  • This article summarises these subjects as well as the pharmacology of investigational drugs, such as indiplon (another benzodiazepine receptor agonist), gaboxadol ( a selective extrasynaptic GABAA agonist -SEGA-), and some anticonvulsant drugs that could be useful as hypnotics: tiagabine ( a GABA transporter inhibitor), pregabaline and gabapentine (GABA analogs).

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  • (PMID = 18265473.001).
  • [ISSN] 0327-6139
  • [Journal-full-title] Vertex (Buenos Aires, Argentina)
  • [ISO-abbreviation] Vertex
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Argentina
  • [Chemical-registry-number] 0 / Hypnotics and Sedatives; 0 / Receptors, GABA; JL5DK93RCL / Melatonin
  • [Number-of-references] 15
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66. Ebert B, Wafford KA, Deacon S: Treating insomnia: Current and investigational pharmacological approaches. Pharmacol Ther; 2006 Dec;112(3):612-29
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Gaboxadol, an investigational treatment and a selective extrasynaptic GABA(A) receptor agonist (SEGA), targets GABA(A) receptors containing a delta subunit, which are located outside the synaptic junctions of thalamic and cortical neurons thought to play an important regulatory role in the onset, maintenance, and depth of the sleep process.

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  • (PMID = 16876255.001).
  • [ISSN] 0163-7258
  • [Journal-full-title] Pharmacology & therapeutics
  • [ISO-abbreviation] Pharmacol. Ther.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / GABA-A Receptor Agonists; 0 / Hypnotics and Sedatives; 0 / Receptors, Melatonin; 0 / Serotonin Antagonists; 56-12-2 / gamma-Aminobutyric Acid
  • [Number-of-references] 660
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67. An CL, Lim WJ, Hong SY, Shin EC, Kim MK, Lee JR, Park SR, Woo JG, Lim YP, Yun HD: Structural and biochemical analysis of the asc operon encoding 6-phospho-beta-glucosidase in Pectobacterium carotovorum subsp. carotovorum LY34. Res Microbiol; 2005 Mar;156(2):145-53

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Sequence analysis of the 5618-bp cloned DNA fragment (accession number AY622309) showed three open reading frames (ascG, ascF, and ascB) that are predicted to encode 375, 486, and 476 amino acid proteins, respectively.
  • The AscG ORF shared a high similarity with the Escherichia coli AscG repressor.

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  • (PMID = 15748978.001).
  • [ISSN] 0923-2508
  • [Journal-full-title] Research in microbiology
  • [ISO-abbreviation] Res. Microbiol.
  • [Language] eng
  • [Databank-accession-numbers] GENBANK/ AY622309
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Bacterial Proteins; EC 2.7.1.- / Phosphoenolpyruvate Sugar Phosphotransferase System; EC 3.2.1.- / Glucosidases; EC 3.2.1.86 / 6-phospho-beta-glucosidase
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68. Silver AC, Graf J: Prevalence of genes encoding the type three secretion system and the effectors AexT and AexU in the Aeromonas veronii group. DNA Cell Biol; 2009 Aug;28(8):383-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Two loci of the T3SS, ascV and ascF-ascG, and aexT and aexU were PCR amplified and sequenced from these isolates.

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  • [CommentIn] DNA Cell Biol. 2009 Aug;28(8):359-60 [19622050.001]
  • (PMID = 19534604.001).
  • [ISSN] 1557-7430
  • [Journal-full-title] DNA and cell biology
  • [ISO-abbreviation] DNA Cell Biol.
  • [Language] eng
  • [Databank-accession-numbers] GENBANK/ FJ753296/ FJ753297/ FJ753298/ FJ753299/ FJ753300/ FJ753301/ FJ753302/ FJ753303/ FJ753304/ FJ753305/ FJ753306/ FJ753307/ FJ753308/ FJ753309/ FJ753310/ FJ753311/ FJ753312/ FJ753313/ FJ753314/ FJ753315/ FJ753316/ FJ753317/ FJ753318/ FJ753319/ FJ753320/ FJ753321/ FJ753322/ FJ753323/ FJ753324/ FJ753325/ FJ753326/ FJ753327/ FJ753328/ FJ753329/ FJ753330
  • [Publication-type] Journal Article; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Bacterial Proteins; 0 / DNA, Bacterial; 0 / Virulence Factors
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69. McDonald LM, Sheppard WF, Staveley SM, Sohal B, Tattersall FD, Hutson PH: Gaboxadol, a selective extrasynaptic GABA(A) agonist, does not generalise to other sleep-enhancing drugs: a rat drug discrimination study. Neuropharmacology; 2007 Mar;52(3):844-53

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  • Gaboxadol is a selective extrasynaptic GABA(A) receptor agonist (SEGA) which enhances slow-wave sleep, and may act principally at extrasynaptic GABA(A)alpha4betadelta receptors.

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  • (PMID = 17196996.001).
  • [ISSN] 0028-3908
  • [Journal-full-title] Neuropharmacology
  • [ISO-abbreviation] Neuropharmacology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / GABA Agonists; 0 / Isoxazoles; K1M5RVL18S / gaboxadol
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70. Park SH, Min HS, Kim B, Myung J, Paek SH: Galectin-3: a useful biomarker for differential diagnosis of brain tumors. Neuropathology; 2008 Oct;28(5):497-506
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Galectin-3: a useful biomarker for differential diagnosis of brain tumors.
  • Definitely positive but heterogeneous expression was found in various tumors including subependymal giant cell astrocytomas (SEGA), classic glioblastoma multiforme, anaplastic oligoastrocytomas, CNS primitive neuroectodermal tumors (CNS PNETs), and hemangiopericytomas.
  • Eighty percent of small cell glioblstomas were completely negative, but 20% showed heterogeneous positivity for gal-3.
  • Focal positivity for gal-3 was also found in dysembryoplastic neuroepithelial tumors (DNTs) and gangliogliomas, in which the positive cells were the astrocytic component.
  • On the basis of our immunohistochemical data in conjunction with previous reports, we therefore conclude that gal-3 is differentially expressed in various brain tumors, and thereby, is a helpful biomarker in making differential diagnoses, especially in cases where a morphological diagnosis is controversial.
  • [MeSH-major] Biomarkers, Tumor / analysis. Brain Neoplasms / diagnosis. Brain Neoplasms / metabolism. Galectin 3 / biosynthesis
  • [MeSH-minor] Diagnosis, Differential. Humans. Immunohistochemistry

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  • (PMID = 18384511.001).
  • [ISSN] 1440-1789
  • [Journal-full-title] Neuropathology : official journal of the Japanese Society of Neuropathology
  • [ISO-abbreviation] Neuropathology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Galectin 3
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71. Walsh JK, Mayleben D, Guico-Pabia C, Vandormael K, Martinez R, Deacon S: Efficacy of the selective extrasynaptic GABA A agonist, gaboxadol, in a model of transient insomnia: a randomized, controlled clinical trial. Sleep Med; 2008 May;9(4):393-402
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  • OBJECTIVE: The hypnotic efficacy of gaboxadol, a selective extrasynaptic GABA A agonist (SEGA), was evaluated in a phase-advance model of transient insomnia.

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  • (PMID = 17765013.001).
  • [ISSN] 1389-9457
  • [Journal-full-title] Sleep medicine
  • [ISO-abbreviation] Sleep Med.
  • [Language] eng
  • [Databank-accession-numbers] ClinicalTrials.gov/ NCT00102167
  • [Publication-type] Comparative Study; Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / GABA Agonists; 0 / Isoxazoles; 0 / Receptors, GABA; K1M5RVL18S / gaboxadol
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72. Hajak G, Hedner J, Eglin M, Loft H, Stórustovu SI, Lütolf S, Lundahl J, Gaboxadol Study 99775 Group: A 2-week efficacy and safety study of gaboxadol and zolpidem using electronic diaries in primary insomnia outpatients. Sleep Med; 2009 Aug;10(7):705-12
The Lens. Cited by Patents in .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OBJECTIVES: To evaluate the efficacy and safety profile of gaboxadol, a selective extrasynaptic GABA(A) agonist (SEGA) previously in development for the treatment of insomnia.

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  • (PMID = 19346160.001).
  • [ISSN] 1878-5506
  • [Journal-full-title] Sleep medicine
  • [ISO-abbreviation] Sleep Med.
  • [Language] eng
  • [Publication-type] Journal Article; Randomized Controlled Trial; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / GABA Agonists; 0 / Isoxazoles; 0 / Pyridines; 7K383OQI23 / zolpidem; K1M5RVL18S / gaboxadol
  • [Investigator] Saletu-Zyhlarz G; Schmitz MM; Högl BE; de Volder I; Dumont F; Theys P; Herchuelz A; Matte R; Morehouse R; Lespérance P; Rajda M; Pawluk L; George CF; Morán M; Sonka K; Jennum P; Wildschiødtz G; Sørensen KV; Hion T; Partinen MM; Polo OJ; Mehtonen OP; Dauvilliers Y; Tiberge M; Leger D; Lainey E; Escourrou P; Schenk C; Kölmel HW; Wiegand M; Benes H; Hajak G; Hemmeter UM; Steinberg R; Cirignotta F; Gigli GL; Smits MG; de Weerd AW; Bjorvatn B; Lundemo G; Tvedten T; Eldøen G; Jernajczyk W; Myszka DM; Jakitowicz J; Lewandowski S; Borreguero DG; Estivill E; Sagalés T; Cuartero P; Pin G; Paniagua J; Cañellas F; Mejuto J; Hedner J; Ulfberg J; Hemlin M; Grote L; Despland PA; Holsboer E; Fisch HU; Nutt D; James IG; Soul P; Smith I; Ebrahim IO; Silvert BD
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73. Sparagana SP, Wilkes DC, Thompson CE, Bowers DC: Optic nerve tumor in tuberous sclerosis complex is not responsive to sirolimus. Pediatr Neurol; 2010 Jun;42(6):443-6
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  • [Title] Optic nerve tumor in tuberous sclerosis complex is not responsive to sirolimus.
  • A 12-year-old girl with clinically established tuberous sclerosis complex, and without signs of neurofibromatosis type 1, developed a right retro-ocular optic nerve tumor.
  • After rapid growth for 1 year after its discovery, the optic nerve tumor demonstrated modest progression.
  • The patient received the mammalian target of rapamycin inhibitor, sirolimus, for recurrent subependymal giant cell brain tumors.
  • Although her left ventricular subependymal giant cell tumor demonstrated a 49% reduction in volume, the optic nerve tumor did not respond, and even underwent slight (6%) growth during the 16-month treatment.
  • The quality of this child's vision has remained normal in both eyes, and she is otherwise asymptomatic with regard to the optic nerve tumor.
  • [MeSH-major] Astrocytoma / drug therapy. Brain Neoplasms / drug therapy. Neoplasm Recurrence, Local / drug therapy. Optic Nerve Neoplasms / drug therapy. Sirolimus / therapeutic use. Tuberous Sclerosis / complications

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20472200.001).
  • [ISSN] 1873-5150
  • [Journal-full-title] Pediatric neurology
  • [ISO-abbreviation] Pediatr. Neurol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; W36ZG6FT64 / Sirolimus
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74. Lioy VS, Pratto F, de la Hoz AB, Ayora S, Alonso JC: Plasmid pSM19035, a model to study stable maintenance in Firmicutes. Plasmid; 2010 Jul;64(1):1-17
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The segA locus plays a role both in maximizing plasmid random segregation, and in avoiding replication fork collapses in those plasmids with long inverted repeated regions.

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20403380.001).
  • [ISSN] 1095-9890
  • [Journal-full-title] Plasmid
  • [ISO-abbreviation] Plasmid
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 110
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75. Lambrecht V, Van Goethem JW, Ozsarlak O, Maes M, Parizel PM: Tuberous sclerosis and subependymal giant cell astrocytoma. JBR-BTR; 2005 May-Jun;88(3):144-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Tuberous sclerosis and subependymal giant cell astrocytoma.
  • [MeSH-major] Cerebral Ventricle Neoplasms / diagnosis. Glioma, Subependymal / diagnosis. Image Enhancement. Image Processing, Computer-Assisted. Magnetic Resonance Imaging. Tuberous Sclerosis / diagnosis
  • [MeSH-minor] Adenoma / diagnosis. Adenoma / genetics. Cerebral Ventricles / pathology. Child. Ependyma / pathology. Facial Neoplasms / diagnosis. Facial Neoplasms / genetics. Humans. Male. Neoplasms, Multiple Primary / diagnosis. Neoplasms, Multiple Primary / genetics. Sebaceous Gland Neoplasms / diagnosis. Sebaceous Gland Neoplasms / genetics

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  • (PMID = 16038234.001).
  • [ISSN] 0302-7430
  • [Journal-full-title] JBR-BTR : organe de la Société royale belge de radiologie (SRBR) = orgaan van de Koninklijke Belgische Vereniging voor Radiologie (KBVR)
  • [ISO-abbreviation] JBR-BTR
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Belgium
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76. Li WQ, Li YM, Lu YC, Yu HY: Bulky subependymal giant cell astrocytoma with profuse blood supply without tuberous sclerosis. Neurosciences (Riyadh); 2010 Oct;15(4):287-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Bulky subependymal giant cell astrocytoma with profuse blood supply without tuberous sclerosis.
  • [MeSH-minor] Adult. Astrocytoma / complications. Astrocytoma / diagnosis. Brain Neoplasms / complications. Brain Neoplasms / diagnosis. Humans. Magnetic Resonance Imaging. Male. Tuberous Sclerosis / physiopathology

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  • (PMID = 20956931.001).
  • [ISSN] 1319-6138
  • [Journal-full-title] Neurosciences (Riyadh, Saudi Arabia)
  • [ISO-abbreviation] Neurosciences (Riyadh)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Saudi Arabia
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77. Dickerman RD, Cohen A: Subependymal giant cell astrocytoma and concordant expression in a disease of variable penetrance. Neurosurg Rev; 2005 Oct;28(4):335-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Subependymal giant cell astrocytoma and concordant expression in a disease of variable penetrance.
  • [MeSH-major] Astrocytoma / genetics. Brain Neoplasms / genetics. Penetrance

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  • [CommentOn] Neurosurg Rev. 2004 Oct;27(4):274-80 [15309659.001]
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  • (PMID = 16041550.001).
  • [ISSN] 0344-5607
  • [Journal-full-title] Neurosurgical review
  • [ISO-abbreviation] Neurosurg Rev
  • [Language] eng
  • [Publication-type] Comment; Letter
  • [Publication-country] Germany
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78. Hussain N, Curran A, Pilling D, Malluci CL, Ladusans EJ, Alfirevic Z, Pizer B: Congenital subependymal giant cell astrocytoma diagnosed on fetal MRI. Arch Dis Child; 2006 Jun;91(6):520
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Congenital subependymal giant cell astrocytoma diagnosed on fetal MRI.
  • [MeSH-major] Astrocytoma / congenital. Brain Neoplasms / congenital. Magnetic Resonance Imaging. Prenatal Diagnosis / methods


79. Dolan E, O'Brien ET, Staessen JA: Letter regarding article by Sega et al, "Prognostic value of ambulatory and home blood pressures compared with office blood pressure in the general population". Circulation; 2005 Sep 27;112(13):e244-5; author reply e245-6
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  • [Title] Letter regarding article by Sega et al, "Prognostic value of ambulatory and home blood pressures compared with office blood pressure in the general population".

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  • [CommentOn] Circulation. 2005 Apr 12;111(14):1777-83 [15809377.001]
  • (PMID = 16189849.001).
  • [ISSN] 1524-4539
  • [Journal-full-title] Circulation
  • [ISO-abbreviation] Circulation
  • [Language] eng
  • [Publication-type] Comment; Letter
  • [Publication-country] United States
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80. Ohkubo T, Imai Y: Letter regarding article by Sega et al, "Prognostic value of ambulatory and home blood pressures compared with office blood pressure in the general population". Circulation; 2005 Sep 27;112(13):e245; author reply e245-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Letter regarding article by Sega et al, "Prognostic value of ambulatory and home blood pressures compared with office blood pressure in the general population".

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  • [CommentOn] Circulation. 2005 Apr 12;111(14):1777-83 [15809377.001]
  • (PMID = 16189850.001).
  • [ISSN] 1524-4539
  • [Journal-full-title] Circulation
  • [ISO-abbreviation] Circulation
  • [Language] eng
  • [Publication-type] Comment; Letter
  • [Publication-country] United States
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81. Hansen TW, Jeppesen J, Ibsen H: Letter regarding article by Sega et al, "Prognostic value of ambulatory and home blood pressures compared with office blood pressure in the general population". Circulation; 2005 Sep 27;112(13):e244; author reply e245-6

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Letter regarding article by Sega et al, "Prognostic value of ambulatory and home blood pressures compared with office blood pressure in the general population".

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  • [CommentOn] Circulation. 2005 Apr 12;111(14):1777-83 [15809377.001]
  • (PMID = 16186430.001).
  • [ISSN] 1524-4539
  • [Journal-full-title] Circulation
  • [ISO-abbreviation] Circulation
  • [Language] eng
  • [Publication-type] Comment; Letter
  • [Publication-country] United States
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