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1. Kuncova K, Janda A, Kasal P, Zamecnik J: Immunohistochemical prognostic markers in intracranial ependymomas: systematic review and meta-analysis. Pathol Oncol Res; 2009 Dec;15(4):605-14
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  • [Title] Immunohistochemical prognostic markers in intracranial ependymomas: systematic review and meta-analysis.
  • Distinction between grade II ependymomas and anaplastic ependymomas based on histopathological examination solely is problematic and, therefore, the management of intracranial ependymomas remains controversial.
  • The aim of this study was to conduct a systematic review (SR) and meta-analysis (MA) of data published on immunohistochemical prognostic markers (IPM) in intracranial ependymomas (IE), and to establish an evidence-based perspective on their clinical value.
  • Although the prognostic impact of MIB-1 immunoexpression in IE could be confirmed, there remains lack of further reliable IPM that could be used in routine diagnosis.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Brain Neoplasms / diagnosis. Ependymoma / diagnosis
  • [MeSH-minor] Humans. Ki-67 Antigen / metabolism. Prognosis. Tumor Suppressor Protein p53 / metabolism

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  • (PMID = 19301151.001).
  • [ISSN] 1532-2807
  • [Journal-full-title] Pathology oncology research : POR
  • [ISO-abbreviation] Pathol. Oncol. Res.
  • [Language] eng
  • [Publication-type] Journal Article; Meta-Analysis; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Ki-67 Antigen; 0 / Tumor Suppressor Protein p53
  • [Number-of-references] 49
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2. Jurkiewicz E, Pakuła-Kościesza I, Chełstowska S, Nowak K, Roszkowski M, Grajkowska W, Szary C: Infratentorial tumors in children - value of ADC in prediction of grade of neoplasms. Pol J Radiol; 2010 Oct;75(4):18-23
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  • [Title] Infratentorial tumors in children - value of ADC in prediction of grade of neoplasms.
  • All children were operated on and tumors were histopathologically proved as low-grade - 25 (24 pilocytic astrocytomas, 1 ependymoma) and high-grade lesions - 25 (19 medulloblastomas, 6 anaplastic ependymomas).
  • RESULTS: Statistically significant differences were found in the comparisons of mean ADC of pilocytic astrocytomas (1.54×10(-3)mm(2)/s ±0.2) with medulloblastomas (0.75×10(-3)mm(2)/s ±0.075) and pilocytic astrocytomas (1.54×10(-3)mm(2)/s ±0.2) with anaplastic ependymomas (0.99×10(-3)mm(2)/s ±0.25).
  • Statistical analysis including ependymomas should be discussed, because of small number of these tumors and a non-homogenous group of lesions.

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  • (PMID = 22802799.001).
  • [ISSN] 1899-0967
  • [Journal-full-title] Polish journal of radiology
  • [ISO-abbreviation] Pol J Radiol
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] Poland
  • [Other-IDs] NLM/ PMC3389889
  • [Keywords] NOTNLM ; children / diffusion-weighted imaging – DWI / infratentorial tumors / magnetic resonance
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3. Combs SE, Kelter V, Welzel T, Behnisch W, Kulozik AE, Bischof M, Hof H, Debus J, Schulz-Ertner D: Influence of radiotherapy treatment concept on the outcome of patients with localized ependymomas. Int J Radiat Oncol Biol Phys; 2008 Jul 15;71(4):972-8
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  • [Title] Influence of radiotherapy treatment concept on the outcome of patients with localized ependymomas.
  • PURPOSE: To assess the outcome of 57 patients with localized ependymomas treated with radiotherapy (RT).
  • METHODS AND MATERIALS: Fifty-seven patients with localized ependymomas were treated with RT.
  • Histology was myxopapillary ependymoma (n = 4), ependymoma (n = 23), and anaplastic ependymoma (n = 30).
  • Forty-one patients were treated with local RT, with a local dose of 45 Gy to the posterior fossa, including a boost to the tumor bed of 9 Gy.
  • In 19 patients, the tumor bed was irradiated with a median dose of 54 Gy.
  • RESULTS: Overall survival after primary diagnosis was 83% and 71% at 3 and 5 years.
  • Five-year overall survival was 80% in low-grade and 79% in high-grade tumors.
  • The radiation oncologist must keep in mind that patients with localized ependymomas benefit from local doses > or =45 Gy.
  • [MeSH-major] Brain Neoplasms / mortality. Brain Neoplasms / radionuclide imaging. Ependymoma / mortality. Ependymoma / radiotherapy. Radiotherapy / mortality. Risk Assessment / methods

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  • (PMID = 18337022.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] United States
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4. Qian X, Goumnerova LC, De Girolami U, Cibas ES: Cerebrospinal fluid cytology in patients with ependymoma: a bi-institutional retrospective study. Cancer; 2008 Oct 25;114(5):307-14
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  • [Title] Cerebrospinal fluid cytology in patients with ependymoma: a bi-institutional retrospective study.
  • BACKGROUND: Ependymoma cells are known to occasionally exfoliate into cerebrospinal fluid (CSF).
  • However, the frequency of CSF involvement in patients with ependymoma is unclear, and to the authors' knowledge the cytomorphologic features of the tumor cells have not been described in detail to date.
  • In this study, the CSF findings in patients with ependymal neoplasms are summarized and the cytomorphologic features of ependymoma, including its variants, are illustrated.
  • METHODS: A search of the pathology databases of 2 medical centers was performed to identify all patients with a histologic diagnosis of ependymoma in whom CSF samples were examined.
  • RESULTS: In all, 177 patients with a diagnosis of ependymoma were identified.
  • The detection rate of tumor cells in CSF was 6.7% in 15 adults and 21.2% in 33 children, with an overall rate of 16.7%.
  • Of the 8 patients with positive and/or suspicious diagnoses, 5 ependymomas exhibited anaplastic features and 1 tumor was a myxopapillary ependymoma.
  • The positive samples were usually hypercellular, with cohesive epithelioid cells; long cytoplasmic processes resembling bipolar tanycytes were observed in the tanycytic variant of ependymoma.
  • CONCLUSIONS: Exfoliated cells from ependymomas are recognizable in CSF samples, especially in patients with myxopapillary tumors and tumors with anaplastic features.
  • [MeSH-major] Brain Neoplasms / cerebrospinal fluid. Brain Neoplasms / pathology. Ependymoma / cerebrospinal fluid. Ependymoma / pathology


5. Morgan RJ, Synold T, Mamelak A, Lim D, Al-Kadhimi Z, Twardowski P, Leong L, Chow W, Margolin K, Shibata S, Somlo G, Yen Y, Frankel P, Doroshow JH: Plasma and cerebrospinal fluid pharmacokinetics of topotecan in a phase I trial of topotecan, tamoxifen, and carboplatin, in the treatment of recurrent or refractory brain or spinal cord tumors. Cancer Chemother Pharmacol; 2010 Oct;66(5):927-33
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  • The tumors included glioblastoma (6), anaplastic astrocytoma (2), metastatic non-small cell (3), small cell lung (2), and one each with medulloblastoma, ependymoma, and metastatic breast or colon carcinoma.
  • 4/8 pts with high-grade gliomas had stable disease (median: 3 cycles (range 2-5)).

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  • (PMID = 20107803.001).
  • [ISSN] 1432-0843
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P30 CA033572; United States / NCI NIH HHS / CA / P30 CA033572-26; United States / NCI NIH HHS / CA / CA 33572
  • [Publication-type] Clinical Trial, Phase I; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 094ZI81Y45 / Tamoxifen; 7M7YKX2N15 / Topotecan; BG3F62OND5 / Carboplatin
  • [Other-IDs] NLM/ NIHMS335377; NLM/ PMC3265324
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6. Kumar R, Wani AA, Reddy J, Pal L, Pradhan S: Development of anaplastic ependymoma in Rasmussen's encephalitis: review of the literature and case report. Childs Nerv Syst; 2006 Apr;22(4):416-9
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  • [Title] Development of anaplastic ependymoma in Rasmussen's encephalitis: review of the literature and case report.
  • The occurrence of anaplastic ependymoma in a diagnosed case of RE has not been reported in the literature.
  • CASE REPORT: We report an eight and a half year-old boy, a diagnosed case of RE, suffering from intractable right-sided focal seizures, who developed features of raised intracranial pressure during follow-up.
  • Histopathology of the mass revealed anaplastic ependymoma.
  • [MeSH-major] Brain Neoplasms / pathology. Encephalitis / pathology. Ependymoma / pathology


7. Shih CS, Hale GA, Gronewold L, Tong X, Laningham FH, Gilger EA, Srivastava DK, Kun LE, Gajjar A, Fouladi M: High-dose chemotherapy with autologous stem cell rescue for children with recurrent malignant brain tumors. Cancer; 2008 Mar 15;112(6):1345-53
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  • [Title] High-dose chemotherapy with autologous stem cell rescue for children with recurrent malignant brain tumors.
  • BACKGROUND: High-dose chemotherapy (HDCT) with autologous stem cell rescue (ASCR) has been reported to be effective in treating children with recurrent central nervous system (CNS) malignancies.
  • METHODS: To evaluate the efficacy and toxicities of HDCT and ASCR, the medical records of 27 children with recurrent CNS malignancies who received such therapy at St. Jude Children's Research Hospital between 1989 and 2004 were reviewed.
  • RESULTS: The median age at diagnosis was 4.5 years (range, 0.4-16.6 years) and that at ASCR was 6.7 years (range, 1.1-18.5 years).
  • Diagnoses included medulloblastoma (13 patients), primitive neuroectodermal tumor (3 patients), pineoblastoma (2 patients), atypical teratoid rhabdoid tumor (2 patients), ependymoma (3 patients), anaplastic astrocytoma (2 patients), and glioblastoma multiforme (2 patients).
  • The 5-year PFS rate for patients aged<3 years at diagnosis (57.1%) was significantly better than older patients (5.0%) (P=.019).
  • Among the 6 long-term survivors (5 with M0 disease and 1 with M3 disease at diagnosis), 5 received both radiotherapy and HDCT as part of their salvage regimen; 4 were aged<3 years at diagnosis and had received chemotherapy only as part of frontline therapy.
  • Two patients died of transplant-related toxicities; 44% experienced grade 3 or 4 transplant-related toxicities (toxicities were graded according to the National Cancer Institute Common Toxicity Criteria).
  • CONCLUSIONS: HDCT with ASCR is not an effective salvage strategy for older children with recurrent CNS malignancies.
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Ependymoma / diagnosis. Ependymoma / therapy. Female. Follow-Up Studies. Glioblastoma / diagnosis. Humans. Infant. Male. Medulloblastoma / pathology. Medulloblastoma / therapy. Neuroectodermal Tumors, Primitive / diagnosis. Neuroectodermal Tumors, Primitive / therapy. Pinealoma / pathology. Pinealoma / therapy. Retrospective Studies. Rhabdoid Tumor / pathology. Rhabdoid Tumor / therapy. Salvage Therapy. Survival Rate. Transplantation, Autologous. Treatment Outcome


8. Borrelli A, Mattiazzi L, Capucchio MT, Biolatti C, Cagnasso A, Gianella P, D'Angelo A: Cachexia secondary to intracranial anaplastic (malignant) ependymoma in a boxer dog. J Small Anim Pract; 2009 Oct;50(10):554-7
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  • [Title] Cachexia secondary to intracranial anaplastic (malignant) ependymoma in a boxer dog.
  • On physical examination, cachexia was the only reported abnormality.
  • Based on the morphological features of the tumour, marked parenchymal invasion, extensive necrosis and cellular atypia, the mass was classified as an anaplastic ependymoma.
  • [MeSH-major] Brain Neoplasms / veterinary. Cachexia / veterinary. Dog Diseases / diagnosis. Ependymoma / veterinary
  • [MeSH-minor] Animals. Diagnosis, Differential. Dogs. Fatal Outcome. Female

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  • (PMID = 19796316.001).
  • [ISSN] 1748-5827
  • [Journal-full-title] The Journal of small animal practice
  • [ISO-abbreviation] J Small Anim Pract
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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9. Shibahara I, Kanamori M, Kumabe T, Endo H, Sonoda Y, Ogawa Y, Watanabe M, Tominaga T: Hemorrhagic onset of pilocytic astrocytoma and pilomyxoid astrocytoma. Brain Tumor Pathol; 2009;26(1):1-5
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  • Hemorrhagic onset was observed in 4 of 35 (11.4%) patients with pilocytic astrocytoma and pilomyxoid astrocytoma, with higher incidence than in glioblastoma (3.9%), anaplastic oligodendroglioma (7.7%), and anaplastic ependymoma (7.1%).
  • The hemorrhagic onset occurred in 2 patients with sporadic pilocytic astrocytoma, 1 with pilocytic astrocytoma associated with neurofibromatosis type 1, and 1 with pilomyxoid astrocytoma.
  • There was no correlation between hemorrhagic onset and clinical features, including age, sex, tumor location, proliferative activity, or microvascular proliferation.
  • Hemorrhagic onset of pilocytic astrocytoma and pilomyxoid astrocytoma is not as uncommon as was previously thought, so pilocytic astrocytoma or pilomyxoid astrocytoma should be considered in the differential diagnosis of patients with brain tumors manifesting as hemorrhagic onset.


10. Apel-Sarid L, Cochrane DD, Steinbok P, Byrne AT, Dunham C: Microfibrillar collagen hemostat-induced necrotizing granulomatous inflammation developing after craniotomy: a pediatric case series. J Neurosurg Pediatr; 2010 Oct;6(4):385-92
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  • Based primarily on MR imaging, postoperative reactions have most commonly elicited clinical differential diagnoses of tumor recurrence or abscess.
  • RESULTS: Case 1 is that of a 10-year-old girl whose diagnosis was a right temporal lobe ganglioglioma, classified as WHO Grade I.
  • Case 2 is that of a 9-year-old boy whose diagnosis was a left parietal lobe anaplastic ependymoma, classified as WHO Grade III.
  • Finally, Case 3 is that of a 15-year-old girl whose diagnosis was focal cortical dysplasia Type IIA affecting the left occipital lobe.
  • The postsurgical reactions incited by MCH mimicked the radiological appearance of either an abscess (Cases 2 and 3) or recurrent tumor (Case 1).
  • Based on the authors' observations, the possibility of an idiopathic inflammatory reaction to MCH should be considered when either seizures, a typical radiological appearance (that is, consistent with tumor recurrence or abscess formation), or both arise shortly after initial surgery.
  • A conservative treatment approach to this type of inflammatory lesion appears to be the most appropriate management strategy.
  • [MeSH-minor] Adolescent. Biopsy. Child. Ependymoma / pathology. Ependymoma / surgery. Female. Humans. Intracranial Hemorrhages / prevention & control. Magnetic Resonance Imaging. Male. Malformations of Cortical Development / pathology. Malformations of Cortical Development / surgery. Necrosis. Postoperative Complications / etiology. Postoperative Complications / pathology

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  • (PMID = 20887115.001).
  • [ISSN] 1933-0715
  • [Journal-full-title] Journal of neurosurgery. Pediatrics
  • [ISO-abbreviation] J Neurosurg Pediatr
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 9007-34-5 / Collagen
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11. Erdoğan G, Ozel E, Peştereli HE, Salar Z, Tirak B, Karaveli S: Ovarian ependymoma. APMIS; 2005 Apr;113(4):301-3
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  • [Title] Ovarian ependymoma.
  • The tumor contained solid areas of a yellowish white color.
  • Microscopic examination revealed highly cellular solid areas with many typical ependymal perivascular pseudorosettes.
  • Glial fibrillary acidic protein (GFAP) immunopositivity was observed in the cytoplasm of the tumor cells.
  • Based on the histopathologic and immunohistochemical features, the tumor was diagnosed as an anaplastic ependymoma.
  • This is to the best of our knowledge only the second case of anaplastic ependymoma in the medical literature.
  • [MeSH-major] Ependymoma / pathology. Ovarian Neoplasms / pathology

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  • (PMID = 15865613.001).
  • [ISSN] 0903-4641
  • [Journal-full-title] APMIS : acta pathologica, microbiologica, et immunologica Scandinavica
  • [ISO-abbreviation] APMIS
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Glial Fibrillary Acidic Protein
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12. Jaremko JL, Jans LB, Coleman LT, Ditchfield MR: Value and limitations of diffusion-weighted imaging in grading and diagnosis of pediatric posterior fossa tumors. AJNR Am J Neuroradiol; 2010 Oct;31(9):1613-6
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  • [Title] Value and limitations of diffusion-weighted imaging in grading and diagnosis of pediatric posterior fossa tumors.
  • Ependymoma (n = 5) could not be reliably differentiated from medulloblastoma or JPA.
  • A trend toward increased diffusion restriction in higher grade tumors (1/14 grade I, 7%; 9/12 grade IV, 75%) had too much overlap to predict the grade of individual cases.
  • The overlap in ADC between tumor types appeared partly due to technical factors (in small, heterogeneous, calcific, or hemorrhagic tumors) but also likely reflected true histologic variability, given that our 3 overlap cases included a desmoplastic medulloblastoma, an anaplastic ependymoma, and a JPA with restricted diffusion in its nodule.
  • Simple structural features (macrocystic tumor, location off midline) aided in distinguishing JPA from the other tumors in these cases.

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  • [CommentIn] AJNR Am J Neuroradiol. 2010 Oct;31(9):1617-8 [20581070.001]
  • [ErratumIn] AJNR Am J Neuroradiol. 2010 Nov;31(10):E90
  • (PMID = 20538820.001).
  • [ISSN] 1936-959X
  • [Journal-full-title] AJNR. American journal of neuroradiology
  • [ISO-abbreviation] AJNR Am J Neuroradiol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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13. Rieber J, Remke M, Hartmann C, Korshunov A, Burkhardt B, Sturm D, Mechtersheimer G, Wittmann A, Greil J, Blattmann C, Witt O, Behnisch W, Halatsch ME, Orakcioglu B, von Deimling A, Lichter P, Kulozik A, Pfister S: Novel oncogene amplifications in tumors from a family with Li-Fraumeni syndrome. Genes Chromosomes Cancer; 2009 Jul;48(7):558-68
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  • Li-Fraumeni syndrome (LFS) represents an inherited tumor syndrome that is typically caused by germline mutations of the tumor suppressor gene TP53.
  • The index patient developed an anaplastic medulloblastoma with unusual genomic profile exhibiting six distinct high-level genomic amplifications, two of them targeting the MYCN and GLI2 genes, respectively.
  • In an extrarenal rhabdoid tumor from the same patient, we found a novel high-level amplification of the MYC oncogene.
  • The father of this patient was diagnosed with myxopapillary ependymoma (WHO degrees I), whereas a brother died from an early relapse of a choroid plexus carcinoma.
  • [MeSH-major] Gene Amplification. Germ-Line Mutation. Li-Fraumeni Syndrome / genetics. Tumor Suppressor Protein p53 / genetics
  • [MeSH-minor] Child. Choroid Plexus Neoplasms / genetics. Choroid Plexus Neoplasms / metabolism. Comparative Genomic Hybridization. Female. Gene Dosage. Humans. Immunohistochemistry. In Situ Hybridization, Fluorescence. Loss of Heterozygosity. Male. Medulloblastoma / genetics. Medulloblastoma / metabolism. Oligonucleotide Array Sequence Analysis. Pedigree. Rhabdoid Tumor / genetics. Rhabdoid Tumor / metabolism

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  • (PMID = 19378321.001).
  • [ISSN] 1098-2264
  • [Journal-full-title] Genes, chromosomes & cancer
  • [ISO-abbreviation] Genes Chromosomes Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / TP53 protein, human; 0 / Tumor Suppressor Protein p53
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14. Rousselot C, Francois P, Jan M, Bergemer AM: [Report of seven cases of clear-cell meningioma and a literature review]. Ann Pathol; 2010 Apr;30(2):73-82
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  • Three patients belonged to the same family, probably affected by neurofibromatosis type 2.
  • CONCLUSION: Our study supports the fact that MCC course is less favourable than meningioma WHO grade I, even in the absence of anaplastic area, high mitotic activity, or necrosis.
  • [MeSH-major] Biomarkers, Tumor / analysis. Meningeal Neoplasms / pathology. Meningioma / pathology. Neoplasm Proteins / analysis
  • [MeSH-minor] Adult. Aged. Astrocytoma / diagnosis. Child, Preschool. Diagnostic Errors. Ependymoma / diagnosis. Female. Humans. Keratins / analysis. Ki-67 Antigen / analysis. Male. Middle Aged. Mucin-1 / analysis. Neurofibromatosis 2 / diagnosis. Neurofibromatosis 2 / genetics. Neurofibromatosis 2 / pathology. Receptors, Progesterone / analysis. Retrospective Studies. S100 Proteins / analysis. Young Adult

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  • [Copyright] Copyright 2010 Elsevier Masson SAS. All rights reserved.
  • (PMID = 20451062.001).
  • [ISSN] 0242-6498
  • [Journal-full-title] Annales de pathologie
  • [ISO-abbreviation] Ann Pathol
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Ki-67 Antigen; 0 / Mucin-1; 0 / Neoplasm Proteins; 0 / Receptors, Progesterone; 0 / S100 Proteins; 68238-35-7 / Keratins
  • [Number-of-references] 33
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15. Beschorner R, Wehrmann M, Ernemann U, Bonin M, Horber V, Oehl-Jaschkowitz B, Meyermann R, Dufke A: Extradural ependymal tumor with myxopapillary and ependymoblastic differentiation in a case of Schinzel-Giedion syndrome. Acta Neuropathol; 2007 Mar;113(3):339-46
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  • [Title] Extradural ependymal tumor with myxopapillary and ependymoblastic differentiation in a case of Schinzel-Giedion syndrome.
  • Primary extradural ependymomas are rare neoplasms usually of the myxopapillary type.
  • Reports on malignant primary extradural ependymal tumors are exceptionally rare.
  • We here report on a 3-year-old boy with Schinzel-Giedion syndrome (SGS), who presented with lumbar spina bifida occulta and a progressive extraspinal lesion in the subcutaneous sacrococcygeal region.
  • Microscopic examinations revealed an uncommon ependymal tumor with well-differentiated regions reflecting myxopapillary ependymoma and highly anaplastic regions with numerous mitoses, necroses, ependymal rosettes and ependymoblastic rosettes.
  • Final neuropathologic diagnosis was an extraspinal anaplastic ependymal tumor with myxopapillary and ependymoblastic differentiation, corresponding to WHO grade IV.
  • So far, 42 cases have been reported, among them 7 were diagnosed to have malignant neoplasms, including three malignant sacrococcygeal teratomas, two sacrococcygeal primitive neuroectodermal tumors (PNET), one hepatoblastoma and one malignant kidney tumor.
  • The present case is the first report on an ependymal tumor with a mixture of myxopapillary, anaplastic and ependymoblastic features and the first report on an ependymal tumor arising on the genetic background of SGS.
  • [MeSH-major] Central Nervous System Neoplasms / complications. Ependymoma / complications. Genetic Diseases, Inborn / complications. Sacrococcygeal Region / pathology

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  • (PMID = 17165030.001).
  • [ISSN] 0001-6322
  • [Journal-full-title] Acta neuropathologica
  • [ISO-abbreviation] Acta Neuropathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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16. Monoranu CM, Huang B, Zangen IL, Rutkowski S, Vince GH, Gerber NU, Puppe B, Roggendorf W: Correlation between 6q25.3 deletion status and survival in pediatric intracranial ependymomas. Cancer Genet Cytogenet; 2008 Apr 1;182(1):18-26
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  • [Title] Correlation between 6q25.3 deletion status and survival in pediatric intracranial ependymomas.
  • We previously used microsatellite analysis of ependymomas to identify frequent deletions in regions 6q15 approximately q16, 6q21 approximately q22.1, and 6q24.3 approximately q25.3.
  • To refine our preliminary analysis of potential prognostic regions, we used a panel of 25 microsatellite markers located between 6q15 and 6qter in 49 pairs of matched normal and tumor specimens from 28 children and 21 adults with ependymoma.
  • Pediatric anaplastic intracranial (supra- and infratentorial) ependymomas harboring the 6q25.3 deletion (n = 9) showed significantly longer overall survival than did patients of the same group without the aberration (n = 6), independent of the extent of resection (P = 0.013).
  • [MeSH-major] Brain Neoplasms / genetics. Chromosome Deletion. Chromosomes, Human, Pair 6. Ependymoma / genetics

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  • (PMID = 18328946.001).
  • [ISSN] 0165-4608
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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17. Lopez-Gines C, Gil-Benso R, Faus C, Monleon D, Mata M, Morales JM, Cigudosa JC, Gonzalez-Darder J, Celda B, Cerda-Nicolas M: Metastasizing anaplastic ependymoma in an adult. Chromosomal imbalances, metabolic and gene expression profiles. Histopathology; 2009 Mar;54(4):500-4
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  • [Title] Metastasizing anaplastic ependymoma in an adult. Chromosomal imbalances, metabolic and gene expression profiles.
  • [MeSH-major] Brain Neoplasms / pathology. Ependymoma / pathology
  • [MeSH-minor] Adult. Anaplasia. Biomarkers, Tumor / metabolism. Chromosome Aberrations. Cyclin-Dependent Kinase Inhibitor p16 / genetics. Gene Expression Profiling. Genes, p53. Humans. In Situ Hybridization, Fluorescence. Magnetic Resonance Imaging. Male. Mutation. Oligonucleotide Array Sequence Analysis

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  • (PMID = 19309408.001).
  • [ISSN] 1365-2559
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Publication-type] Case Reports; Letter; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Cyclin-Dependent Kinase Inhibitor p16
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18. Azarpira N, Rakei M, Mokhtari M: Cytologic findings in malignant ependymoma: a case report. Acta Cytol; 2010 Sep-Oct;54(5 Suppl):1023-6
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  • [Title] Cytologic findings in malignant ependymoma: a case report.
  • BACKGROUND: Intraoperative imprint cytology has proved to be a valuable tool in the diagnosis of central nervous system (CNS) tumors.
  • Ependymomas are uncommon glial neoplasms of the CNS, arising from ependymal lining of the ventricular system and central canal of the spinal cord.
  • Anaplastic ependymoma is a rare tumor that causes diagnostic difficulties in imprint cytology because of variable cytomorphologic findings.
  • Computed tomography of the head showed hydrocephalus with a large parietal lobe tumor with midline structural shift.
  • The tumor showed pseudorosettes with glial fibrillary acidic protein and epithelial membrane antigen expression.
  • The diagnosis was made with histologic and immunologic confirmation.
  • CONCLUSION: Although imprint cytology is a useful method for making a rapid diagnosis, but immunohistochemical markers play a major role in the final diagnosis.
  • [MeSH-major] Central Nervous System Neoplasms / pathology. Ependymoma / pathology

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  • (PMID = 21053591.001).
  • [ISSN] 0001-5547
  • [Journal-full-title] Acta cytologica
  • [ISO-abbreviation] Acta Cytol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Glial Fibrillary Acidic Protein
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19. Ahmed N, Bhurgri Y, Sadiq S, Shakoor KA: Pediatric brain tumours at a tertiary care hospital in Karachi. Asian Pac J Cancer Prev; 2007 Jul-Sep;8(3):399-404
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  • The morphological distribution of cases was astrocytoma (28 cases, 34.6%), primitive neuroectodermal tumor or PNET (40 cases; 49.4%), ependymoma (8 cases, 10%), mixed glioma (4 cases; 5%) and a case of oligodendroglioma.
  • The morphological categorization of supratentorial tumors was astrocytoma (17 cases; 63%), ependymoma (5 cases; 18.5%), mixed glioma (2 cases; 7.4%).
  • The 17 supratentorial astrocytoma were sub-categorized as follows - pilocytic astrocytoma (5 cases; 29.4%), grade II astrocytoma (6 cases; 35.3%); grade III astrocytoma (2 cases; 11.8%), anaplastic astrocytoma (1 case; 5.9%) and glioblastoma multiforme (3 cases; 17.7%).
  • The morphological categorization of infratentorial tumors was astrocytoma (11 cases; 20.4%), medulloblastoma (38 cases; 70.4%), ependymoma (3 cases; 5.6%) and mixed glioma - astroependymoma (2 cases, 3.7%).
  • The morphological sub-categorization of infratentorial astrocytoma was pilocytic astrocytoma (7 cases, 63.6%), with gemistocytic astrocytoma, grade II, grade III and anaplastic astrocytoma comprising 1 (9.1%) case each.

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  • (PMID = 18159977.001).
  • [ISSN] 1513-7368
  • [Journal-full-title] Asian Pacific journal of cancer prevention : APJCP
  • [ISO-abbreviation] Asian Pac. J. Cancer Prev.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Thailand
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20. Juric-Sekhar G, Zarkovic K, Waeg G, Cipak A, Zarkovic N: Distribution of 4-hydroxynonenal-protein conjugates as a marker of lipid peroxidation and parameter of malignancy in astrocytic and ependymal tumors of the brain. Tumori; 2009 Nov-Dec;95(6):762-8
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  • [Title] Distribution of 4-hydroxynonenal-protein conjugates as a marker of lipid peroxidation and parameter of malignancy in astrocytic and ependymal tumors of the brain.
  • The aim of this study was to evaluate the expression of HNE in 120 astrocytic and 40 ependymal tumors in relation to tumor type, grade of malignancy, angiogenesis, and presence of necrosis and apoptosis.
  • The incidence of HNE-immunopositive tumor cells increased with increasing grades of malignancy.
  • Significantly higher HNE expression was found in tumor cells of glioblastomas multiforme than in cells of pilocytic astrocytomas (P < 0.005), and in anaplastic ependymomas than in benign ependymomas (P < 0.01).
  • HNE-immunopositive tumor cells were distributed more diffusely than in perivascular locations (P < 0.05).
  • HNE was expressed in the endothelium of almost all tumor vessels, but its expression in the walls of the vessels was significantly higher in diffuse and anaplastic astrocytomas than in pilocytic astrocytomas and glioblastomas multiforme (P < 0.05).
  • The number of microvessels containing HNE in their endothelium and walls was significantly associated with the grade of malignancy in both astrocytic (P < 0.001) and ependymal tumors (P < 0.05), although microvessels in pilocytic astrocytomas were significantly more numerous (P < 0.05) than in diffuse astrocytomas.
  • CONCLUSIONS: LPO seems to be a common pathological process in astrocytic and ependymal glial tumors, proportional to the level of malignancy and neovascularization.
  • [MeSH-major] Aldehydes / analysis. Astrocytoma / chemistry. Biomarkers, Tumor / analysis. Brain Neoplasms / chemistry. Brain Neoplasms / pathology. Ependymoma / chemistry. Lipid Peroxidation. Neoplasm Proteins / analysis

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  • (PMID = 20210242.001).
  • [ISSN] 0300-8916
  • [Journal-full-title] Tumori
  • [ISO-abbreviation] Tumori
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Aldehydes; 0 / Biomarkers, Tumor; 0 / Cross-Linking Reagents; 0 / Neoplasm Proteins; K1CVM13F96 / 4-hydroxy-2-nonenal
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21. Paramanathan N, Ooi KG, Reeves D, Wilcsek GA: Synchronous radiation-induced orbital meningioma and multiple cavernomas. Clin Exp Ophthalmol; 2010 May;38(4):414-7
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  • We present the first reported case of synchronous radiation-induced orbital meningioma and cavernomas of the cerebellum and bilateral basal ganglia, presenting 16 years after ionizing radiation therapy for parietal anaplastic ependymoma, at the age of five.

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  • (PMID = 20491803.001).
  • [ISSN] 1442-9071
  • [Journal-full-title] Clinical & experimental ophthalmology
  • [ISO-abbreviation] Clin. Experiment. Ophthalmol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] AU0V1LM3JT / Gadolinium
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22. Hashiba T, Izumoto S, Kagawa N, Suzuki T, Hashimoto N, Maruno M, Yoshimine T: Expression of WT1 protein and correlation with cellular proliferation in glial tumors. Neurol Med Chir (Tokyo); 2007 Apr;47(4):165-70; discussion 170
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  • The expression of Wilms' tumor gene WT1 protein was investigated immunohistochemically in 73 glial tumors, including 60 astrocytic tumors, eight oligodendroglial tumors, and five ependymal tumors.
  • Almost all glioblastomas, anaplastic astrocytomas, anaplastic ependymomas, and anaplastic oligodendrogliomas expressed high levels of WT1 protein.
  • Histological examination found that WT1 protein was strongly expressed in the anaplastic portions and areas with perivascular proliferation and high cellularity, implying that WT1 gene might be important in glial tumor cell proliferation.
  • This study indicates that many malignant glial tumors are good candidates for cancer immunotherapy targeting WT1 protein and that WT1 protein expression could be used as a proliferation marker in glial tumors.

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  • (PMID = 17457020.001).
  • [ISSN] 0470-8105
  • [Journal-full-title] Neurologia medico-chirurgica
  • [ISO-abbreviation] Neurol. Med. Chir. (Tokyo)
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Ki-67 Antigen; 0 / WT1 Proteins
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23. De Sio L, Milano GM, Castellano A, Jenkner A, Fidani P, Dominici C, Donfrancesco A: Temozolomide in resistant or relapsed pediatric solid tumors. Pediatr Blood Cancer; 2006 Jul;47(1):30-6
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  • Tumor types were: neuroblastoma (NB; n = 17), medulloblastoma (MB; 8), brain stem glioma (BSG; 8), extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor (EOES; 4), Ewing's sarcoma (ES; 4), anaplastic astrocytoma (AA; 3), rhabdomyosarcoma (RMS; 2), ependymoma (EP; 2), cerebral primitive neuroectodermal tumor (cPNET; 2), hepatocarcinoma (HC; 1), and osteosarcoma (OS; 1).
  • Haematological toxicity grade 3-4 (mainly thrombocytopenia) was observed in 21.4% of administered courses, nausea was reported in 3.1% and respiratory distress in 0.7%.
  • CONCLUSION: Oral TMZ was well tolerated in children with resistant or relapsed solid tumors and showed activity in NB and CNS tumours refractory to standard chemotherapy.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Dacarbazine / analogs & derivatives. Drug Resistance, Neoplasm. Neoplasm Recurrence, Local / drug therapy. Neoplasms / drug therapy

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  • [Copyright] Copyright 2006 Wiley-Liss, Inc.
  • [ErratumIn] Pediatr Blood Cancer. 2006 Oct 15;47(5):647-8
  • (PMID = 16047361.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide
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24. Kurimoto M, Nagai S, Hamada H, Tsuboi Y, Hayashi N, Kubota T, Endo S: Malignant transformation of supratentorial clear cell ependymoma. Neuropathology; 2009 Jun;29(3):299-302
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant transformation of supratentorial clear cell ependymoma.
  • Recurrence of clear cell ependymoma is not a rare condition, but malignant transformation of clear cell ependymoma has not yet been well presented.
  • The authors report a 44-year-old man who presented with progressive right hemiparesis.
  • A brain tumor in the left frontal premotor area was removed and an initial pathological diagnosis of oligodendroglioma was made.
  • The tumor recurred 4 months later, and reoperation of the tumor and adjuvant local radiotherapy were performed.
  • The first and second surgical specimens did not contain any ependymal rosettes or pseudorosettes, and thus a diagnosis of oligodendroglioma was made.
  • At this time, the tumor had an ultrastructural appearance compatible with ependymoma.
  • Thereafter, the recurrent tumors showed anaplastic features such as nuclear pleomorphisms and necrosis with pseudopallisading.
  • The autopsy specimens resembled a feature of glioblastoma but the tumor was sharply demarcated from the surrounding parenchyma.
  • [MeSH-major] Brain Neoplasms / pathology. Ependymoma / pathology. Neoplasm Recurrence, Local / pathology
  • [MeSH-minor] Adult. Brain / pathology. Brain / ultrastructure. Diagnosis, Differential. Fatal Outcome. Humans. Magnetic Resonance Imaging. Male. Tomography, X-Ray Computed

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  • (PMID = 18647267.001).
  • [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
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25. Kumar P, Rastogi N, Jain M, Chhabra P: Extraneural metastases in anaplastic ependymoma. J Cancer Res Ther; 2007 Apr-Jun;3(2):102-4
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  • [Title] Extraneural metastases in anaplastic ependymoma.
  • Ependymoma are rare glial neoplasm, it rarely metastasize outside the central nervous system.
  • We present a case of anaplastic ependymoma with extraneural metastases with review of literature.
  • A ten-year-old male child presented with anaplastic ependymoma of choroid plexus and treated with craniospinal radiotherapy in 1998.
  • [MeSH-major] Choroid Plexus Neoplasms / pathology. Ependymoma / secondary. Scalp / pathology. Skin Neoplasms / secondary

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  • (PMID = 17998733.001).
  • [ISSN] 1998-4138
  • [Journal-full-title] Journal of cancer research and therapeutics
  • [ISO-abbreviation] J Cancer Res Ther
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
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26. Grundy RG, Wilne SH, Robinson KJ, Ironside JW, Cox T, Chong WK, Michalski A, Campbell RH, Bailey CC, Thorp N, Pizer B, Punt J, Walker DA, Ellison DW, Machin D, Children's Cancer and Leukaemia Group (formerly UKCCSG) Brain Tumour Committee: Primary postoperative chemotherapy without radiotherapy for treatment of brain tumours other than ependymoma in children under 3 years: results of the first UKCCSG/SIOP CNS 9204 trial. Eur J Cancer; 2010 Jan;46(1):120-33
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  • [Title] Primary postoperative chemotherapy without radiotherapy for treatment of brain tumours other than ependymoma in children under 3 years: results of the first UKCCSG/SIOP CNS 9204 trial.
  • We aimed to assess the role of a primary chemotherapy strategy in avoiding or delaying radiotherapy in children younger than 3 years with malignant brain tumours other than ependymoma, the results of which have already been published.
  • METHODS: Ninety-seven children were enrolled between March 1993 and July 2003 and, following diagnostic review, comprised: medulloblastoma (n=31), astrocytoma (26), choroid plexus carcinoma [CPC] (15), CNS PNET (11), atypical teratoid/rhabdoid tumours [AT/RT] (6) and ineligible (6).
  • FINDINGS: Over all diagnostic groups the cumulative progression rate was 80.9% at 5 years while the corresponding need-for-radiotherapy rate for progression was 54.6%, but both rates varied by tumour type.
  • Patients with medulloblastoma presented as a high-risk group, 83.9% having residual disease and/or metastases at diagnosis.
  • All children with large cell or anaplastic variants of medulloblastoma died within 2 years of diagnosis.
  • The 5-year EFS for non-brainstem high-grade gliomas [HGGs] was 13.0% (CI: 2.2-33.4) and the OS was 30.9% (CI: 11.5-52.8).
  • This treatment strategy was less effective for AT/RT with 3-year OS of 16.7% (CI: 0.8-51.7) and CNS PNET with 1-year OS of 9.1% (CI: 0.5-33.3).
  • INTERPRETATION: The outcome for very young children with brain tumours is dictated by degree of surgical resection and histological tumour type and underlying biology as an indicator of treatment sensitivity.
  • Desmoplastic/nodular sub-type of medulloblastoma has a better prognosis than classic histology, despite traditional adverse clinical features of metastatic disease and incomplete surgical resection.
  • This study highlights the differing therapeutic challenges presented by the malignant brain tumours of early childhood, the importance of surgical approaches and the need to explore individualised brain sparing approaches to the range of malignant brain tumours that present in early childhood.

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  • (PMID = 19818598.001).
  • [ISSN] 1879-0852
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Grant] United Kingdom / Cancer Research UK / /
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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27. Gilbert MR, Ruda R, Soffietti R: Ependymomas in adults. Curr Neurol Neurosci Rep; 2010 May;10(3):240-7
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  • [Title] Ependymomas in adults.
  • Ependymomas are rare primary central nervous system tumors in adults.
  • They occur most commonly in the spinal cord, where histopathologic evaluation is critical to differentiate the grade I myxopapillary ependymoma from the grade II ependymoma or grade III anaplastic ependymoma.
  • Brain ependymomas are either grade II or III.
  • For myxopapillary ependymoma, complete removal while maintaining capsule integrity may be curative.
  • Some grade II ependymomas may be observed carefully after imaging confirms complete resection, but grade III tumors require adjuvant radiation treatment.
  • Radiation commonly is given to the region of tumor, except in cases in which there is imaging or cerebrospinal fluid evidence of tumor dissemination.
  • [MeSH-major] Central Nervous System Neoplasms. Ependymoma

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  • (PMID = 20425040.001).
  • [ISSN] 1534-6293
  • [Journal-full-title] Current neurology and neuroscience reports
  • [ISO-abbreviation] Curr Neurol Neurosci Rep
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 62
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28. Benesch M, Weber-Mzell D, Gerber NU, von Hoff K, Deinlein F, Krauss J, Warmuth-Metz M, Kortmann RD, Pietsch T, Driever PH, Quehenberger F, Urban C, Rutkowski S: Ependymoma of the spinal cord in children and adolescents: a retrospective series from the HIT database. J Neurosurg Pediatr; 2010 Aug;6(2):137-44
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Ependymoma of the spinal cord in children and adolescents: a retrospective series from the HIT database.
  • OBJECT: Reports on spinal cord ependymoma in children are rare.
  • The aim of this study was to evaluate the clinical spectrum, treatment, and outcome of children with primary ependymoma of the spinal cord who were registered in the database of the pediatric German brain tumor studies Hirntumor (HIT) '91 and HIT 2000.
  • METHODS: Between 1991 and 2007, 29 patients (12 male and 17 female, median age at diagnosis 13.6 years) with primary spinal cord ependymoma (myxopapillary ependymoma WHO Grade I, II, and III tumors in 6, 17, and 6 patients, respectively) were identified.
  • Four patients had neurofibromatosis Type 2.
  • One patient with anaplastic ependymoma (WHO Grade III) died 65 months after diagnosis of disease progression.
  • Primary adjuvant treatment (radiotherapy, chemotherapy, or both) was used in 8 (50%) of 16 patients following GTR and in 9 (82%) of 11 patients who underwent less than a GTR.
  • Progression-free survival at 5 years is 84.4% (95% CI 50%-96%) for patients following GTR compared with 57.1% (95% CI 25%-69%) for patients who achieved a less than GTR (p = 0.088, log-rank test).
  • A high relapse incidence (4 of 6) was observed among patients with myxopapillary ependymoma.
  • CONCLUSIONS: Gross-total resection is the mainstay of treatment for patients with primary spinal cord ependymoma and may be achieved in about 50% of the patients using modern surgical techniques.
  • Primary adjuvant treatment was commonly used in children with spinal cord ependymoma irrespective of the extent of resection or tumor grade.
  • [MeSH-major] Ependymoma / surgery. Spinal Cord Neoplasms / surgery
  • [MeSH-minor] Adolescent. Austria. Biopsy. Chemotherapy, Adjuvant. Child. Combined Modality Therapy. Disability Evaluation. Disease Progression. Female. Follow-Up Studies. Germany. Humans. Male. Neurofibromatosis 2 / diagnosis. Neurofibromatosis 2 / drug therapy. Neurofibromatosis 2 / pathology. Neurofibromatosis 2 / radiotherapy. Neurofibromatosis 2 / surgery. Postoperative Complications / diagnosis. Postoperative Complications / mortality. Prospective Studies. Radiotherapy, Adjuvant. Registries. Survival Rate

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  • (PMID = 20672934.001).
  • [ISSN] 1933-0715
  • [Journal-full-title] Journal of neurosurgery. Pediatrics
  • [ISO-abbreviation] J Neurosurg Pediatr
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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29. 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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  • [Title] Lateral ventricle gliomas and central neurocytomas in adults diagnosis and perspectives.
  • In our series ventricular ependymomas (eight cases) were mostly hyperdense with pronounced contrast uptake.
  • Ependymomas and anaplastic astrocytomas and glioblastomas followed the characteristics of the similar extraventricular ones.
  • In our series low-grade astrocytomas, WHO I-II [Louis DN, Ohgaki H, Wiestler OD, Canevee WK.
  • WHO classification of tumours of the central nervous system.
  • [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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30. Mogler C, Kohlhof P, Penzel R, Grenacher L, Haag GM, Schirmacher P, Mueller W: A primary malignant ependymoma of the abdominal cavity: a case report and review of the literature. Virchows Arch; 2009 Apr;454(4):475-8
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  • [Title] A primary malignant ependymoma of the abdominal cavity: a case report and review of the literature.
  • Ependymomas generally arise in the central nervous system (CNS).
  • Rare primary extraneural ependymomas have been observed.
  • Here, we describe the first case of an overt malignant primary extraneural ependymoma in a young female patient.
  • Careful reevaluation together with extensive review of the literature and comparison of related cases established the diagnosis after treatment failure and tumor progression.
  • The tumor was large and firm with some small cysts and showed pseudorosettes with strong glial fibrillary acidic protein (GFAP) expression.
  • In conclusion, primary extraneural ependymomas have to be included into the differential diagnosis of abdominal tumors with pseudorosette-formation, even in unusual sites, and GFAP-immunohistochemistry (IHC) supports the diagnosis.
  • [MeSH-major] Abdominal Cavity / pathology. Diagnostic Errors. Ependymoma / diagnosis

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  • (PMID = 19238432.001).
  • [ISSN] 1432-2307
  • [Journal-full-title] Virchows Archiv : an international journal of pathology
  • [ISO-abbreviation] Virchows Arch.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Glial Fibrillary Acidic Protein
  • [Number-of-references] 15
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31. Valera ET, Machado HR, Santos AC, de Oliveira RS, Araújo D, Neder L, Tone LG: The use of neoadjuvant chemotherapy to achieve complete surgical resection in recurring supratentorial anaplastic ependymoma. Childs Nerv Syst; 2005 Mar;21(3):230-3
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  • [Title] The use of neoadjuvant chemotherapy to achieve complete surgical resection in recurring supratentorial anaplastic ependymoma.
  • INTRODUCTION: Ependymoma is a central nervous system neoplasm that is often managed with surgery and radiotherapy.
  • The benefits of chemotherapy in treating this tumor remain poorly defined.
  • CASE REPORT: The use of a platinum-based chemotherapy as a neoadjuvant treatment to permit complete surgical resection of a supratentorial anaplastic ependymoma recurring for the third time is described.
  • DISCUSSION: This paper also discusses the possible use of chemotherapy as a strategy that may allow tumor shrinkage and ease tumor resection in giant recurring ependymomas.
  • [MeSH-major] Combined Modality Therapy. Ependymoma / therapy. Neoadjuvant Therapy. Supratentorial Neoplasms / therapy

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  • (PMID = 15338180.001).
  • [ISSN] 0256-7040
  • [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
  • [Publication-country] Germany
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32. Sardi I, Sanzo M, Giordano F, Sandri A, Mussa F, Donati PA, Genitori L: Intracavitary chemotherapy (Gliadel) and oral low-dose etoposide for recurrent anaplastic ependymoma. Oncol Rep; 2008 May;19(5):1219-23
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Intracavitary chemotherapy (Gliadel) and oral low-dose etoposide for recurrent anaplastic ependymoma.
  • Anaplastic ependymoma is associated with a higher incidence of tumor recurrence and its prognosis still remains unsatisfactory.
  • Consolidated therapy for ependymoma includes surgery followed by focal radiotherapy when resection is incomplete.
  • In the case of relapse treatment, options are limited especially for patients who have already received radiotherapy.
  • We sought to establish the feasibility of administering low-dose oral etoposide (50 mg/m(2)/day for 21 days) in combination with the implantation of intracavitary carmustine (BCNU) wafers (Gliadel) at the gross total resection for achieving synergistic treatment in three children affected by recurrent anaplastic ependymoma.
  • This multimodal approach was not effective for the treatment of refractory anaplastic ependymoma and further studies are required in order to define the role of the combination of multidrug systemic chemotherapy with BCNU wafer implantation in children with high-risk brain tumors.
  • [MeSH-major] Biocompatible Materials. Brain / pathology. Brain Neoplasms / drug therapy. Decanoic Acids / therapeutic use. Ependymoma / drug therapy. Etoposide / administration & dosage. Polyesters / therapeutic use

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  • (PMID = 18425379.001).
  • [ISSN] 1021-335X
  • [Journal-full-title] Oncology reports
  • [ISO-abbreviation] Oncol. Rep.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; 0 / Biocompatible Materials; 0 / Decanoic Acids; 0 / Polyesters; 6PLQ3CP4P3 / Etoposide; 90409-78-2 / decanedioic acid-4,4'-(1,3-propanediylbis(oxy))bis(benzoic acid) copolymer; U68WG3173Y / Carmustine
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33. Robles SG, Saldaña C, Boto GR, Martinez A, Zamarron AP, Jorquera M, Mata P: Intradural extramedullary spinal ependymoma: a benign pathology? Spine (Phila Pa 1976); 2005 May 1;30(9):E251-4
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  • [Title] Intradural extramedullary spinal ependymoma: a benign pathology?
  • STUDY DESIGN: Spinal ependymoma is a benign central nervous system tumor described as an intramedullary lesion more frequently located at the conus medullaris.
  • It has been described exceptionally in the literature as an intradural extramedullary tumor.
  • OBJECTIVE: Presentation of an extremelly rare location and evolution of extremedullary ependymoma and discussion of its probable origin, differential diagnosis, treatment options, and follow-up.
  • SUMMARY OF BACKGROUND DATA: This case demonstrates an unusual location of a benign ependymal tumor in the extramedullary space with a total resection, which recurred in a lower level with a malignant transformation with the same extramedullary location.
  • METHODS: The authors present the case of a 47-year-old woman with a subacute spinal cord dysfunction and an intradural extramedullary D2-D3 tumor mimicking meningioma or neurinoma.
  • RESULTS: After complete resection, anatomic-pathologic studies confirmed that the lesion was a benign classic ependymoma.
  • Good neurologic outcome was achieved, and no residual tumor was present at magnetic resonance imaging (MRI) control performed at 3 and 9 months later.
  • One year after surgery, a new intradural extramedullary tumor was found at the D4 level without recurrence at D2.
  • The patient was operated on again, but at this time the histologic study showed an anaplastic ependymoma with a proliferation index of 25% measured by Ki-67.
  • CONCLUSION: All of the previously reported cases of spinal intradural extramedullary ependymomas carried out a benign course.
  • The case we are reporting is the first one in which malignant transformation occurred.
  • This tumor should be taken into account in the differential diagnosis of intradural extramedullary lesions.
  • Moreover, close follow-up is recommended for this unusual location of ependymomas.
  • [MeSH-major] Cell Transformation, Neoplastic / pathology. Dura Mater / pathology. Ependymoma / pathology. Spinal Neoplasms / pathology
  • [MeSH-minor] Combined Modality Therapy. Diagnosis, Differential. Female. Humans. Meningioma / diagnosis. Middle Aged. Neoplasms, Second Primary / pathology. Neoplasms, Second Primary / radiotherapy. Neoplasms, Second Primary / surgery. Neurilemmoma / diagnosis

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  • (PMID = 15864145.001).
  • [ISSN] 1528-1159
  • [Journal-full-title] Spine
  • [ISO-abbreviation] Spine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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34. Kano T, Ikota H, Wada H, Iwasa S, Kurosaki S: A case of an anaplastic ependymoma with gliosarcomatous components. Brain Tumor Pathol; 2009;26(1):11-7
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  • [Title] A case of an anaplastic ependymoma with gliosarcomatous components.
  • The tumor was removed, and pathological studies revealed a cerebellar astrocytoma corresponding to World Health Organization grade II.
  • When she was 35 years old, or 6 years after the surgery, magnetic resonance imaging revealed a recurrence of the tumor in the right cerebellum, and subtotal removal of the recurrent tumor was performed.
  • Pathological studies revealed a mixed glioblastoma multiforme and anaplastic ependymoma.
  • Thereafter, at 39 years of age, or 4 years after radiation therapy, magnetic resonance imaging again revealed a recurrence of the tumor, which was heterogeneously enhanced with gadoliniumdiethylenetriamine pentaacetic acid in the right cerebellum.
  • Subtotal removal of the tumor was performed; pathological studies revealed an anaplastic ependymoma with sarcomatous components.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Ependymoma / pathology. Gliosarcoma / pathology
  • [MeSH-minor] Adult. Astrocytoma / pathology. Astrocytoma / surgery. Female. Glial Fibrillary Acidic Protein / metabolism. Headache / etiology. Humans. Immunohistochemistry. Magnetic Resonance Imaging. Neoplasm Recurrence, Local. Paraffin Embedding. Tomography, X-Ray Computed

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  • (PMID = 19408092.001).
  • [ISSN] 1861-387X
  • [Journal-full-title] Brain tumor pathology
  • [ISO-abbreviation] Brain Tumor Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Glial Fibrillary Acidic Protein
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35. Oshiro S, Tsugu H, Komatsu F, Ohnishi H, Ueno Y, Sakamoto S, Fukushima T, Soma G: Evaluation of intratumoral administration of tumor necrosis factor-alpha in patients with malignant glioma. Anticancer Res; 2006 Nov-Dec;26(6A):4027-32
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  • [Title] Evaluation of intratumoral administration of tumor necrosis factor-alpha in patients with malignant glioma.
  • BACKGROUND: This study assessed safety and efficacy for intratumoral administration of tumor necrosis factor-a (TNF-SAM2) into the post-operative tumor cavity through an Ommaya reservoir for patients with malignant glioma.
  • MATERIALS AND METHODS: Seven patients with malignant glioma, comprising 3 cases with glioblastoma multiforme (GBM), 3 cases with anaplastic astrocytoma (AA) and 1 case with malignant ependymoma (ME) were included in the study.
  • TNF-SAM2 was administrated into the post-operative tumor cavity through a reservoir at a concentration of 1x10(4) U/body when recurrence was detected, or as initial induction therapy concomitant with radiotherapy.
  • RESULTS: Partial response to this regional immunotherapy was seen in 4 out of 7 patients, and 1 patient with GBM has remained clinically stable for >184 weeks without tumor progression.
  • With AA, 2 cases appeared to display slowed advance and longer times to tumor recurrence or regrowth.
  • CONCLUSION: Local immunotherapy with TNF-SAM2 may safely contribute to therapeutic efficacy in some patients with malignant glioma.
  • [MeSH-major] Glioma / therapy. Tumor Necrosis Factor-alpha / administration & dosage

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  • (PMID = 17195453.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Clinical Trial; Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / TNF-SAM2; 0 / Tumor Necrosis Factor-alpha
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36. Park EK, Lee YH, Kim DS, Choi JU, Kim TS, Shim KW: 17-year-old girl with headache and complex partial seizure. Brain Pathol; 2010 Nov;20(6):1111-4
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  • Supratentorial cortical ependymoma is a rare clinical entity where ependymoma occurring in the cortex without any connection to the ventricular system since ependymoma usually arises from the lining of the ventricular system or central canal of spinal cord.
  • There have been 14 such cases reported in the literature.We report the first case of a supratentorial extraaxial cortical anaplastic ependymoma with minimal cortical attachment in a 17-years-old girl, presented with headache and complex partial seizure.

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  • (PMID = 20925697.001).
  • [ISSN] 1750-3639
  • [Journal-full-title] Brain pathology (Zurich, Switzerland)
  • [ISO-abbreviation] Brain Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / Glial Fibrillary Acidic Protein; 0 / Ki-67 Antigen
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37. Fujisawa H, Misaki K, Takabatake Y, Hasegawa M, Yamashita J: Cyclin D1 is overexpressed in atypical teratoid/rhabdoid tumor with hSNF5/INI1 gene inactivation. J Neurooncol; 2005 Jun;73(2):117-24
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  • [Title] Cyclin D1 is overexpressed in atypical teratoid/rhabdoid tumor with hSNF5/INI1 gene inactivation.
  • OBJECT: Although atypical teratoid/rhabdoid tumor (AT/RT) is known to generate through inactivation of the hSNF5/INI1 gene on chromosome 22q, the downstream molecular mechanism remains unclear.
  • METHODS: We analyzed 16 tumors under three years of age: seven medulloblastomas, three anaplastic ependymomas (E IIIs), two each of supratentorial primitive neuroectodermal tumors (sPNETs) and choroid plexus carcinomas (CPCs), and one each of neuroblastoma and pineoblastoma.
  • Because of the presence of rhabdoid cells and the polyimmunophenotypic features, the diagnosis was revised to AT/RT in five (31%) tumors, namely, two E IIIs and one each of medulloblastoma, CPC and pineoblastoma.
  • CONCLUSION: AT/RT can be misdiagnosed as a variety of tumors, including ependymoma that potentially harbors LOH 22q.
  • [MeSH-major] Central Nervous System Neoplasms / genetics. Chromosomes, Human, Pair 22 / genetics. Cyclin D1 / metabolism. DNA-Binding Proteins / genetics. Rhabdoid Tumor / genetics. Teratoma / genetics. Transcription Factors / genetics

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  • (PMID = 15981100.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / SMARCB1 Protein; 0 / SMARCB1 protein, human; 0 / Transcription Factors; 136601-57-5 / Cyclin D1
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38. Bouvier C, De Paula AM, Fernandez C, Quilichini B, Scavarda D, Gentet JC, Figarella-Branger D: Atypical teratoid/rhabdoid tumour: 7-year event-free survival with gross total resection and radiotherapy in a 7-year-old boy. Childs Nerv Syst; 2008 Jan;24(1):143-7
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  • CASE STUDY: We report the case of a 7-year-old boy who presented in 1998 a tumour of the left frontal lobe.
  • Initially diagnosed as anaplastic ependymoma, the boy was treated by gross total resection followed by radiotherapy at the operated site.
  • Molecular techniques performed on frozen specimen of the orbital tumour confirmed the diagnosis of atypical teratoid/rhabdoid tumour (ATRT).
  • DISCUSSION: We discuss the pathological criteria for diagnosis of ATRT and the usefulness of early radiotherapy in the light of the recent literature.
  • [MeSH-major] Brain Neoplasms / diagnosis. Teratoma / diagnosis
  • [MeSH-minor] Actins / analysis. Child. Combined Modality Therapy. Diagnosis, Differential. Frontal Lobe / chemistry. Frontal Lobe / radiation effects. Frontal Lobe / surgery. Humans. Immunohistochemistry. Male. Mucin-1 / analysis. Muscle, Smooth / chemistry. Rhabdoid Tumor / diagnosis. Rhabdoid Tumor / radiotherapy. Rhabdoid Tumor / surgery. Treatment Outcome. Vimentin / analysis

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  • [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
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39. Roma AA, Prayson RA: Expression of cyclo-oxygenase-2 in ependymal tumors. Neuropathology; 2006 Oct;26(5):422-8
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  • [Title] Expression of cyclo-oxygenase-2 in ependymal tumors.
  • Little is known about its expression in ependymal neoplasms.
  • The objective of the present study was to assess COX-2 immunostaining of ependymal tumors.
  • Retrospective COX-2 immunohistochemical analysis was conducted on 117 ependymal tumors.
  • The study group (56 men and 44 women, mean age, 30.8 years) was comprised of 48 low-grade ependymomas (WHO grade II), 12 anaplastic ependymomas (WHO grade III), 27 myxopapillary ependymomas (WHO grade I) and 13 subependymomas (WHO grade I).
  • At last known follow-up (range, 12-226 months; mean, 74 months), 52 patients were alive with no evidence of tumor, 16 patients were alive with residual tumor, nine patients died with tumor, one patient died with no tumor and three died with tumor status unknown.
  • Thirty-six (36%) patients had tumors, which demonstrated positive COX-2 staining, including 16/27 (59%) myxopapillary ependymomas, 3/13 (23%) subependymomas, 14/48 (29%) ependymomas and 3/12 (25%) anaplastic ependymomas.
  • Statistically significant COX-2 positive immunostaining was observed in myxopapillary ependymomas versus WHO grade II (P = 0.03) and grade III (P = 0.02) tumors.
  • Increased COX-2 expression in myxopapillary ependymoma as compared to the WHO grade II and II ependymoma was observed.
  • The reason for this apparent increased immunoexpression in these low-grade tumors is uncertain.
  • COX-2 inhibitors may play a role in treatment of the subset of ependymal tumors that demonstrate increased expression.
  • COX-2 staining did not reliably predict tumor behavior.
  • [MeSH-major] Biomarkers, Tumor / analysis. Brain Neoplasms / metabolism. Cyclooxygenase 2 / biosynthesis. Ependymoma / metabolism

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  • (PMID = 17080719.001).
  • [ISSN] 0919-6544
  • [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 / Biomarkers, Tumor; EC 1.14.99.1 / Cyclooxygenase 2
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40. Cao WD, Zhang X, Zhang JN, Yang ZJ, Zhen HN, Cheng G, Li B, Gao D: Immunocytochemical detection of 14-3-3 in primary nervous system tumors. J Neurooncol; 2006 Apr;77(2):125-30
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  • However, 14-3-3 immunoreactivity was seen in the majority of astrocytomas [grade I (9/11), II (16/21), III (13/17), IV (17/21)].
  • But the intensity and degree of 14-3-3 immunoreactivity in diffuse astrocytomas, anaplastic astrocytoma, and glioblastoma multiformes showed trends with tumor grade, with glioblastomas having the highest positivity (P = 0.048).
  • The 14-3-3 immunoreactivity was also seen in the majority of other gliomas [oligodendroglioma (2/3), anaplastic oligodendroglioma (4/4), ependymoma (1/2), anaplastic ependymoma (2/2), choroid plexus papilloma (3/3), pineocytoma (2/2), medulloblastoma (5/8)].
  • The up-regulated expression of 14-3-3 may be a common mechanism for evading apoptosis in most primary human nervous system tumors, and targeting 14-3-3 may be a novel promising strategy for the treatment of these tumors, especially for malignant tumors.
  • [MeSH-major] 14-3-3 Proteins / biosynthesis. Biomarkers, Tumor / analysis. Brain Neoplasms / metabolism

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  • (PMID = 16292484.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / 14-3-3 Proteins; 0 / Biomarkers, Tumor; 0 / YWHAB protein, human
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41. Nakagawa Y, Kageji T, Mizobuchi Y, Kumada H, Nakagawa Y: Clinical results of BNCT for malignant brain tumors in children. Appl Radiat Isot; 2009 Jul;67(7-8 Suppl):S27-30
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  • [Title] Clinical results of BNCT for malignant brain tumors in children.
  • It is very difficult to treat the patients with malignant brain tumor in children, especially under 3 years, because the conventional irradiation cannot be applied due to the damage of normal brain tissue.
  • However, boron neutron capture therapy (BNCT) has tumor selectivity such that it can make damage only in tumor cells.
  • We evaluated the clinical results and courses in patients with malignant glioma under 15 years.
  • There were 3 glioblastomas (GBM), 6 anaplastic astrocytomas(AAS), 7 primitive neuroectodermal tumors (PNET), 6 pontine gliomas and 1 anaplastic ependymoma.
  • All GBM and PNET patients died due to CSF and/or CNS dissemination without local tumor regrowth.
  • All pontine glioma patients died due to regrowth of the tumor.
  • Four of 6 anaplastic astrocytoma and 1 anaplastic ependymoma patients alive without tumor recurrence.
  • BNCT can be applied to malignant brain tumors in children, especially under 3 years instead of conventional radiation.
  • [MeSH-minor] Adolescent. Astrocytoma / pathology. Astrocytoma / radiotherapy. Child. Child, Preschool. Ependymoma / pathology. Ependymoma / radiotherapy. Fatal Outcome. Female. Glioblastoma / pathology. Glioblastoma / radiotherapy. Humans. Infant. Magnetic Resonance Angiography. Magnetic Resonance Imaging. Male. Neoplasm Invasiveness / pathology. Neuroectodermal Tumors, Primitive / pathology. Neuroectodermal Tumors, Primitive / radiotherapy

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  • (PMID = 19406652.001).
  • [ISSN] 1872-9800
  • [Journal-full-title] Applied radiation and isotopes : including data, instrumentation and methods for use in agriculture, industry and medicine
  • [ISO-abbreviation] Appl Radiat Isot
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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42. Schuurmans M, Vanneste JA, Verstegen MJ, van Furth WR: Spinal extramedullary anaplastic ependymoma with spinal and intracranial metastases. J Neurooncol; 2006 Aug;79(1):57-9
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  • [Title] Spinal extramedullary anaplastic ependymoma with spinal and intracranial metastases.
  • We describe a 29-year-old woman who presented with progressive neck pain, sensory deficit and weakness in both arms.
  • Magnetic resonance imaging (MRI) of the cervical spine revealed an extramedullary tumor with severe spinal cord compression.
  • During surgery an intradural extramedullary tumor was found.
  • Further imaging showed a second lumbar spinal tumor.
  • Microscopy of both tumors showed that both tumors were anaplastic ependymomas, which almost never present as extramedullary tumors.
  • [MeSH-major] Brain Neoplasms / secondary. Ependymoma / pathology. Spinal Cord Neoplasms / pathology
  • [MeSH-minor] Adult. Cervical Vertebrae. Combined Modality Therapy. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Ki-67 Antigen / metabolism. Lumbar Vertebrae. Magnetic Resonance Imaging. S100 Proteins / metabolism

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  • (PMID = 16614942.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Ki-67 Antigen; 0 / S100 Proteins
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43. Kieran MW, Packer RJ, Onar A, Blaney SM, Phillips P, Pollack IF, Geyer JR, Gururangan S, Banerjee A, Goldman S, Turner CD, Belasco JB, Broniscer A, Zhu Y, Frank E, Kirschmeier P, Statkevich P, Yver A, Boyett JM, Kun LE: Phase I and pharmacokinetic study of the oral farnesyltransferase inhibitor lonafarnib administered twice daily to pediatric patients with advanced central nervous system tumors using a modified continuous reassessment method: a Pediatric Brain Tumor Consortium Study. J Clin Oncol; 2007 Jul 20;25(21):3137-43
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  • [Title] Phase I and pharmacokinetic study of the oral farnesyltransferase inhibitor lonafarnib administered twice daily to pediatric patients with advanced central nervous system tumors using a modified continuous reassessment method: a Pediatric Brain Tumor Consortium Study.
  • PURPOSE: A dose-escalation phase I and pharmacokinetic study of the farnesyltransferase inhibitor lonafarnib (SCH66336) was conducted in children with recurrent or progressive CNS tumors.
  • Both radiographic response (one anaplastic astrocytoma) and stable disease (one medulloblastoma, two high-grade and four low-grade gliomas, one ependymoma, and one sarcoma) were noted, and seven patients remained on treatment for 1 year or longer.
  • [MeSH-major] Central Nervous System Neoplasms / drug therapy. Central Nervous System Neoplasms / mortality. Enzyme Inhibitors / pharmacokinetics. Farnesyltranstransferase / antagonists & inhibitors. Neoplasm Invasiveness / pathology. Piperidines / pharmacokinetics. Pyridines / pharmacokinetics
  • [MeSH-minor] Administration, Oral. Adolescent. Adult. Child. Child, Preschool. Dose-Response Relationship, Drug. Drug Administration Schedule. Female. Follow-Up Studies. Humans. Infant. Infant, Newborn. Male. Maximum Tolerated Dose. Neoplasm Staging. Risk Assessment. Survival Analysis. Treatment Outcome

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  • (PMID = 17634493.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / U01 CA81457
  • [Publication-type] Clinical Trial, Phase I; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Enzyme Inhibitors; 0 / Piperidines; 0 / Pyridines; 193275-84-2 / lonafarnib; EC 2.5.1.29 / Farnesyltranstransferase
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44. Duda-Szymańska J, Papierz W: Morphological analysis of vascular density in ependymomas. Folia Neuropathol; 2007;45(3):115-9
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  • [Title] Morphological analysis of vascular density in ependymomas.
  • Ependymomas generally show slow growth rate and are associated with a long clinical history.
  • Density of microvessels was shown to serve in various malignant neoplasms as a prognostic factor that correlates with increased risk of metastasis and overall free survival.
  • The aim of this study was to determinate whether that observation can be applied to ependymomas.
  • The materials included 51 ependymomas G2 and G3 according to the WHO classification.
  • The density of blood vessels in anaplastic (WHO G3) ependymomas was shown to be significantly higher than that in WHO G2 type of the tumour, while there was no statistical difference between subtypes of WHO G2 ependymomas.
  • The results suggest a connection between density of vasculature and the degree of histological malignancy in gliomas of ependymal derivation.

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  • (PMID = 17849361.001).
  • [ISSN] 1641-4640
  • [Journal-full-title] Folia neuropathologica
  • [ISO-abbreviation] Folia Neuropathol
  • [Language] ENG
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Poland
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45. Gaspar N, Grill J, Geoerger B, Lellouch-Tubiana A, Michalowski MB, Vassal G: p53 Pathway dysfunction in primary childhood ependymomas. Pediatr Blood Cancer; 2006 May 1;46(5):604-13
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  • [Title] p53 Pathway dysfunction in primary childhood ependymomas.
  • BACKGROUND: Childhood ependymoma remains a major therapeutic challenge despite surgery, chemotherapy, and irradiation.
  • We hypothesized that p53 function might be abrogated in ependymomas and implicated in their resistance to anti-cancer therapy.
  • PROCEDURE: Primary ependymomas at diagnosis or relapse from 24 children were analyzed for p53 pathway, using a functional assay in yeast, RT-PCR, Western blot analysis, and/or immunohistochemistry for TP53 mutation, p14(ARF) deletion and promoter hypermethylation, MDM2 and PAX5 expression, respectively. p53-mediated response to radiation-induced DNA damage was evaluated using Western blot and flow cytometry analysis in two ependymoma xenograft models, IGREP37 and IGREP83, derived from primary anaplastic childhood ependymomas.
  • RESULTS: No TP53, MDM2, p14(ARF), PAX5 gene abnormalities were detected in the primary ependymomas tumors and xenografts tested.
  • In contrast, irradiation yielded significant tumor growth delays and tumor regressions in the p53 functional IGREP83 xenografts.
  • CONCLUSION: Alterations in p53-mediated growth arrest in ependymomas might be implicated in the radio-resistance of these tumors and demand further evaluation.
  • [MeSH-major] Ependymoma / metabolism. Gene Expression Regulation, Neoplastic. Tumor Suppressor Protein p53 / metabolism
  • [MeSH-minor] Animals. B-Cell-Specific Activator Protein / genetics. B-Cell-Specific Activator Protein / metabolism. Blotting, Western. Child. Child, Preschool. DNA Methylation. Female. Humans. Infant. Male. Mice. Promoter Regions, Genetic. Proto-Oncogene Proteins c-mdm2 / genetics. Proto-Oncogene Proteins c-mdm2 / metabolism. Reverse Transcriptase Polymerase Chain Reaction. Signal Transduction. Transplantation, Heterologous. Tumor Cells, Cultured. Tumor Suppressor Protein p14ARF / genetics. Tumor Suppressor Protein p14ARF / metabolism

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  • (PMID = 16086408.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / B-Cell-Specific Activator Protein; 0 / PAX5 protein, human; 0 / Pax5 protein, mouse; 0 / Tumor Suppressor Protein p14ARF; 0 / Tumor Suppressor Protein p53; EC 6.3.2.19 / MDM2 protein, human; EC 6.3.2.19 / Mdm2 protein, mouse; EC 6.3.2.19 / Proto-Oncogene Proteins c-mdm2
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46. Guzman G, Oh S, Shukla D, Engelhard HH, Valyi-Nagy T: Expression of entry receptor nectin-1 of herpes simplex virus 1 and/or herpes simplex virus 2 in normal and neoplastic human nervous system tissues. Acta Virol; 2006;50(1):59-66
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  • In normal human NS, nectin-1 was detected in the soma and processes of central and peripheral neurons, in ependymal cells, choroid plexus epithelial cells, vascular endothelial cells and meningothelial cells.
  • Oligodendroglioma, ependymoma, pilocytic astrocytoma, pleomorphic xanthoastrocytoma, diffuse astrocytoma, anaplastic astrocytoma, glioblastoma multiforme and meningothelial meningioma showed weak focal nectin-1-positivity.


47. Shintaku M, Nagata N, Itoh H: Tanycytic ependymoma of the spinal cord with anaplastic cytological features. Brain Tumor Pathol; 2009;26(1):7-10
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  • [Title] Tanycytic ependymoma of the spinal cord with anaplastic cytological features.
  • In a 43-year-old man, an intramedullary spinal cord tumor spreading from the level of the T2 to T5 vertebrae was subtotally resected.
  • The tumor predominantly consisted of a fascicular proliferation of spindle cells having bland nuclei and bipolar, long cytoplasmic processes, and a few perivascular pseudo-rosettes were found.
  • Although there were no true ependymal rosettes, intracytoplasmic dot-like immunoreactivity for epithelial membrane antigen (EMA) was found in a few cells.
  • In some areas, a dense and diffuse proliferation of anaplastic, short-spindled cells having hyperchromatic nuclei and scant cytoplasm was noted, and the Ki-67 labeling index was remarkably higher (18.2%) in these areas.
  • This is the first documentation of tanycytic ependymoma in which tumor cells showed anaplastic cytological features.
  • [MeSH-major] Ependymoma / pathology. Spinal Cord / pathology. Spinal Cord Neoplasms / pathology


48. Daou MC, Smith TW, Litofsky NS, Hsieh CC, Ross AH: Doublecortin is preferentially expressed in invasive human brain tumors. Acta Neuropathol; 2005 Nov;110(5):472-80
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  • DCX was highly expressed in both high-grade invasive tumors (glioblastoma, n=11; anaplastic astrocytoma/oligoastrocytoma, n=7; and medulloblastoma/PNET, n=6) and low-grade invasive tumors (oligodendroglioma, n=3; and astrocytoma/oligoastrocytoma, n=5).
  • However, DCX was less intensely expressed in the circumscribed group of tumors (pilocytic astrocytoma, n=6; ependymoma/subependymoma, n=7; dysembryoplastic neuroepithelial tumor, n=4; ganglioglioma, n=2; meningioma, n=9; and schwannoma, n=9).
  • By the Cochran-Mantel-Haenszel statistical test, the circumscribed group was significantly different from both the high-grade invasive group (P<0.0001) and the low-grade invasive group (P<0.0001).
  • In addition, DCX immunostaining was stronger at the margin of the tumor than at the center.

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  • (PMID = 16195916.001).
  • [ISSN] 0001-6322
  • [Journal-full-title] Acta neuropathologica
  • [ISO-abbreviation] Acta Neuropathol.
  • [Language] ENG
  • [Grant] United States / NINDS NIH HHS / NS / R01 NS021716; United States / NCI NIH HHS / CA / R21 CA107372; United States / NCI NIH HHS / CA / CA-10737; United States / NINDS NIH HHS / NS / NS-21716
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Microtubule-Associated Proteins; 0 / Neuropeptides; 0 / RNA, Messenger; 0 / doublecortin protein
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49. Metellus P, Figarella-Branger D, Guyotat J, Barrie M, Giorgi R, Jouvet A, Chinot O, Club de Neuro-Oncologie de la Société Française de Neurochirurgie and the Association des Neuro-Oncologues d'Expression Française: Supratentorial ependymomas: prognostic factors and outcome analysis in a retrospective series of 46 adult patients. Cancer; 2008 Jul 1;113(1):175-85
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  • [Title] Supratentorial ependymomas: prognostic factors and outcome analysis in a retrospective series of 46 adult patients.
  • BACKGROUND: Ependymomas account for 2% of all intracranial tumors in adults.
  • Supratentorial ependymomas are less common than their infratentorial counterparts.
  • The authors report a series of adult patients with supratentorial ependymomas to characterize the roles of surgery and histology in tumor control.
  • METHODS: The authors retrospectively studied a homogenous population of 46 adult patients who had supratentorial ependymomas from 24 French neurosurgical centers between 1990 and 2004.
  • On both univariate and multivariate analysis, age <55 years, greater extent of surgery, and lower histologic grade were associated with longer overall and progression-free survival.
  • CONCLUSIONS: In association with age and extent of surgery, histologic grade was identified as a major prognostic factor in adult supratentorial ependymomas.
  • The application of a simple and reproducible grading scheme using objective anaplastic criteria appeared to be both useful practically and clinically applicable.
  • The role of adjuvant radiotherapy for patients with incompletely resected, low-grade ependymomas needs to be investigated further.
  • [MeSH-major] Ependymoma / pathology. Ependymoma / surgery. Supratentorial Neoplasms / pathology. Supratentorial Neoplasms / surgery

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  • [Copyright] (Copyright) 2008 American Cancer Society.
  • (PMID = 18470910.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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50. Vajtai I, Kuhlen D, Kappeler A, Mariani L, Zimmermann A, Paulus W: Rapid spontaneous malignant progression of supratentorial tanycytic ependymoma with sarcomatous features - "Ependymosarcoma". Pathol Res Pract; 2010 Jul 15;206(7):493-8
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  • [Title] Rapid spontaneous malignant progression of supratentorial tanycytic ependymoma with sarcomatous features - "Ependymosarcoma".
  • By analogy to gliosarcoma, the term "ependymosarcoma" has recently been coined to thematize the rare phenomenon of a malignant mesenchymal component arising within an ependymoma.
  • We report on an example of this paradigm, involving tanycytic ependymoma as the host tumor in a 40-year-old female who underwent two tumor extirpation procedures at one-year interval.
  • Microscopically, the tumor consisted of solid, wavy fascicles of elongated cells that were occasionally interrupted by vague perivascular pseudorosettes.
  • A histological diagnosis of tanycytic ependymoma (WHO grade II) was rendered, and no adjuvant therapy given.
  • Histology showed a biphasic glial-sarcomatous architecture with remnants of the original ependymoma now displaying hypercellularity and atypical - yet not frankly anaplastic - features.
  • While the ependymal component was GFAP and S100 protein positive, and featured punctate staining for EMA, none of these markers was expressed in the adjacent sarcoma.
  • To the best of our knowledge, this is the first documentation of tanycytic ependymoma undergoing malignant transformation, one driven by a highly anaplastic mesenchymal component, corresponding to "ependymosarcoma".
  • [MeSH-major] Cell Transformation, Neoplastic / pathology. Ependymoma / pathology. Gliosarcoma / pathology. Neoplasm Recurrence, Local / pathology. Supratentorial Neoplasms / pathology

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  • [Copyright] Copyright 2009 Elsevier GmbH. All rights reserved.
  • (PMID = 19853384.001).
  • [ISSN] 1618-0631
  • [Journal-full-title] Pathology, research and practice
  • [ISO-abbreviation] Pathol. Res. Pract.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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51. Green RM, Cloughesy TF, Stupp R, DeAngelis LM, Woyshner EA, Ney DE, Lassman AB: Bevacizumab for recurrent ependymoma. Neurology; 2009 Nov 17;73(20):1677-80
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  • [Title] Bevacizumab for recurrent ependymoma.
  • BACKGROUND: Ependymoma is a rare type of glioma, representing 5% of all CNS malignancies.
  • At recurrence, ependymoma is notoriously refractory to therapy and the prognosis is poor.
  • METHODS: In this Institutional Review Board-approved study, we retrospectively analyzed the records of 8 adult patients treated for recurrent ependymoma and anaplastic ependymoma with bevacizumab containing chemotherapy regimens.

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  • (PMID = 19917990.001).
  • [ISSN] 1526-632X
  • [Journal-full-title] Neurology
  • [ISO-abbreviation] Neurology
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / UO1 CA-105663-01
  • [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 / Angiogenesis Inhibitors; 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 2S9ZZM9Q9V / Bevacizumab
  • [Other-IDs] NLM/ PMC2788805
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52. Kumar R, Singhal N, Jaiswal SK, Mahapatra AK: Recurrence in supratentorial anaplastic ependymoma. Pediatr Neurosurg; 2007;43(5):364-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Recurrence in supratentorial anaplastic ependymoma.
  • AIM: To study the outcome and recurrence in supratentorial anaplastic ependymoma.
  • METHODS: Sixteen cases of supratentorial anaplastic ependymoma were reviewed.
  • RESULTS: Gross total excision of tumor was achieved in 14 cases, as judged on the basis of intraoperative impression and confirmed with postoperative contrast MR or CT scan.
  • Two operated and irradiated cases of differentiated ependymomas (grade II) developed anaplastic recurrence at follow-up of 5 years and 9 years, respectively, suggesting a malignant transformation of tumor at follow-up.
  • CONCLUSION: It is obvious that anaplastic ependymomas of the supratentorial compartment are aggressive tumors with high rates of recurrence even after gross total excision and irradiation.
  • Gross total excision and postoperative irradiation are not effective in preventing early recurrence in anaplastic ependymomas, and other factors affecting the outcome need to be analyzed.
  • [MeSH-major] Ependymoma / pathology. Neoplasm Recurrence, Local / pathology. Supratentorial Neoplasms / pathology

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  • [Copyright] (c) 2007 S. Karger AG, Basel.
  • (PMID = 17786000.001).
  • [ISSN] 1423-0305
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
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53. Plotkin M, Amthauer H, Eisenacher J, Wurm R, Michel R, Wust P, Stockhammer F, Röttgen R, Gutberlet M, Ruf J, Felix R: Value of 123I-IMT SPECT for diagnosis of recurrent non-astrocytic intracranial tumours. Neuroradiology; 2005 Jan;47(1):18-26
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  • [Title] Value of 123I-IMT SPECT for diagnosis of recurrent non-astrocytic intracranial tumours.
  • The value of single-photon emission tomography (SPECT) using iodine-123-alpha-methyl-tyrosine (IMT) for the diagnosis of recurrent or residual gliomas is well established.
  • The study included 22 patients with suspected recurrent intracranial tumours of non-astrocytic origin (12 brain metastases, one supratentorial primitive neuroendocrine tumour (PNET), one rhabdoid tumour, two clivus chordomas, three ependymomas, two pituitary tumours, one anaplastic meningioma) who had previously been treated by surgery and/or radio/chemotherapy.
  • [MeSH-major] Brain Neoplasms / diagnostic imaging. Iodine Radioisotopes. Methyltyrosines. Neoplasm Recurrence, Local / diagnostic imaging. Radiopharmaceuticals. Tomography, Emission-Computed, Single-Photon / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Chordoma / diagnostic imaging. Ependymoma / diagnostic imaging. False Negative Reactions. False Positive Reactions. Female. Follow-Up Studies. Glioma / diagnostic imaging. Humans. Magnetic Resonance Imaging. Male. Meningioma / diagnostic imaging. Middle Aged. Neuroendocrine Tumors / diagnostic imaging. Pituitary Neoplasms / diagnostic imaging. Retrospective Studies. Rhabdoid Tumor / diagnostic imaging. Sensitivity and Specificity. Supratentorial Neoplasms / diagnostic imaging

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  • (PMID = 15630586.001).
  • [ISSN] 0028-3940
  • [Journal-full-title] Neuroradiology
  • [ISO-abbreviation] Neuroradiology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Iodine Radioisotopes; 0 / Methyltyrosines; 0 / Radiopharmaceuticals; A77N8J5H5T / 3-iodo-alpha-methyltyrosine
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54. Stark AM, Modlich S, Claviez A, van Baalen A, Hugo HH, Mehdorn HM: Congenital diffuse anaplastic astrocytoma with ependymal and leptomeningeal spread: case report. J Neurooncol; 2007 Sep;84(3):325-8
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  • [Title] Congenital diffuse anaplastic astrocytoma with ependymal and leptomeningeal spread: case report.
  • [MeSH-major] Astrocytoma / congenital. Astrocytoma / secondary. Brain Neoplasms / congenital. Brain Neoplasms / pathology. Ependymoma / secondary. Meningeal Neoplasms / secondary


55. Larysz D, Blamek S, Larysz P, Pietras K, Mandera M: Posterior fossa brain tissue injury: developmental, neuropsychological, and neurological consequences of brain tumors in children. Acta Neurochir Suppl; 2010;106:271-4
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  • The aim of the study was the functional neurodevelopmental assessment of children with posterior fossa tumors, specifically examining whether tumor location in particular cerebellar structures determines particular neuropsychological deficits.
  • There were 21 total and 8 subtotal resections of tumor, and marsupialization was performed in cases of arachnoid cysts.
  • Histopathological diagnoses of tumors were as follows: 4 medulloblastomas, 8 pilocytic astrocytomas, 6 fibrillary astrocytomas, 1 anaplastic astrocytoma, 2 oligodendrogliomas, 4 anaplastic ependymomas, 1 choroid plexus papilloma, and 5 arachnoid cysts.

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  • (PMID = 19812963.001).
  • [ISSN] 0065-1419
  • [Journal-full-title] Acta neurochirurgica. Supplement
  • [ISO-abbreviation] Acta Neurochir. Suppl.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Austria
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56. Liu JG, Liu YH, Cai J, Liu XS, Song WZ, Huang Y, Mao Q: [Expression of epidermal growth factor receptor and PTEN in malignancy brain tumors]. Sichuan Da Xue Xue Bao Yi Xue Ban; 2006 Nov;37(6):868-71
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  • METHODS: Using immunohistochemistry to detect the expression of EGFR and PTEN and adopting confocal technology to verify their location in the specimens of 25 respectively glioblastoma multiformes, medulloblastomas, anaplastic oligodendrogliomas, and anaplastic ependymomas.
  • They were 36% and 8% in medulloblastomas, and 28% and 8% in anaplastic oligodendrogliomas, and 24% and 4% in anaplastic ependymomas.
  • PTEN mutation and EGFR overexpression are rare in medulloblastoma, anaplastic oligodendroglioma, and anaplastic ependymoma, so the EGFR or PTEN targeted antitumor approaches may be useful in glioblastoma multiformes but the other 3 tumors.


57. Haberler C, Laggner U, Slavc I, Czech T, Ambros IM, Ambros PF, Budka H, Hainfellner JA: Immunohistochemical analysis of INI1 protein in malignant pediatric CNS tumors: Lack of INI1 in atypical teratoid/rhabdoid tumors and in a fraction of primitive neuroectodermal tumors without rhabdoid phenotype. Am J Surg Pathol; 2006 Nov;30(11):1462-8
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  • [Title] Immunohistochemical analysis of INI1 protein in malignant pediatric CNS tumors: Lack of INI1 in atypical teratoid/rhabdoid tumors and in a fraction of primitive neuroectodermal tumors without rhabdoid phenotype.
  • Immunohistochemical lack of nuclear INI1 protein expression has been recently described as characteristic finding in atypical teratoid/rhabdoid tumors (AT/RTs), and has been suggested as useful marker to distinguish AT/RTs from other malignant pediatric central nervous system (CNS) tumors.
  • In this study, we examined a large series of malignant pediatric CNS tumors to determine the immunohistochemical expression of INI1 protein in different malignant pediatric tumor entities.
  • Archival paraffin-embedded biopsy specimens of 289 malignant pediatric CNS tumors including medulloblastomas, supratentorial primitive neuroectodermal tumors, glioblastomas, anaplastic astrocytomas, anaplastic ependymomas, choroid plexus carcinomas, germ cell tumors, and AT/RTs were analyzed immunohistochemically for expression of nuclear INI1 protein.
  • In summary, immunohistochemical expression of INI1 protein is lacking in tumors displaying characteristic morphologic features of AT/RT.
  • We conclude that INI1 protein analysis should be routinely performed in all malignant pediatric embryonal CNS tumors to detect cases with lack of INI1 protein, because patients with these tumors are likely to benefit from intensified treatment.
  • [MeSH-major] Biomarkers, Tumor / analysis. Brain Neoplasms / metabolism. Chromosomal Proteins, Non-Histone / metabolism. DNA-Binding Proteins / metabolism. Neuroectodermal Tumors, Primitive / metabolism. Rhabdoid Tumor / metabolism. Teratoma / metabolism. Transcription Factors / metabolism

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  • (PMID = 17063089.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / SMARCB1 protein, human; 0 / Transcription Factors
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58. Bartolek F, Zganjer M, Pajić A, Cizmić A, Kljenak A, Cigit I, Car A, Stepan J, Bartolek D, Boras A: A 10-year experience in the treatment of intraabdominal cerebrospinal fluid pseudocysts. Coll Antropol; 2010 Dec;34(4):1397-400
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  • Recurrence of the abdominal cyst was observed in one girl who was in terminal stadium of anaplastic ependymoma.

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  • (PMID = 21874727.001).
  • [ISSN] 0350-6134
  • [Journal-full-title] Collegium antropologicum
  • [ISO-abbreviation] Coll Antropol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Croatia
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59. Onguru O, Kurt B, Gunhan O, Soylemezoglu F: Cyclooxygenase-2 (cox-2) expression and angiogenesis in intracranial ependymomas. Clin Neuropathol; 2006 Sep-Oct;25(5):216-20
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  • [Title] Cyclooxygenase-2 (cox-2) expression and angiogenesis in intracranial ependymomas.
  • Little is known about the role of Cox-2 in angiogenesis and proliferation of ependymomas.
  • We studied Cox-2 expression, Ki-67 labeling index (Ki-67 LI) and microvessel density (MVD) in 30 intracranial ependymomas and analyzed the relationship among these parameters to evaluate their importance in the tumor biology of ependymomas.
  • Statistically significant difference was present for Ki-67 LI between ependymomas (grade II, WHO) and anaplastic ependymomas (grade III, WHO) (p < 0.001) (mean Ki-67 LI for ependymoma, 2.8%, for anaplastic ependymomas, 15.6%).
  • Anaplastic ependymomas did not demonstrate a greater vascularization than ependymomas, and the MVD values were 84.5 +/- 39.7 for ependymomas, and 90.6 +/- 61.4 for anaplastic ependymomas.
  • Although Cox-2 expression was slightly higher in anaplastic ependymomas, it was not statistically significant.
  • CONCLUSION: Similar to morphologic and prognostic heterogeneity in ependymomas, Cox-2 expression, MVD and Ki-67 LI also show a great variability.
  • Other factors may be more important for the proliferation and angiogenesis of ependymomas.
  • [MeSH-major] Brain Neoplasms / blood supply. Brain Neoplasms / enzymology. Cyclooxygenase 2 / biosynthesis. Ependymoma / blood supply. Ependymoma / enzymology. Neovascularization, Pathologic
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor / analysis. Cell Proliferation. Child. Child, Preschool. Female. Humans. Immunohistochemistry. Infant. Ki-67 Antigen / metabolism. Male

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  • (PMID = 17007443.001).
  • [ISSN] 0722-5091
  • [Journal-full-title] Clinical neuropathology
  • [ISO-abbreviation] Clin. Neuropathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Ki-67 Antigen; EC 1.14.99.1 / Cyclooxygenase 2
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60. Massimino M, Giangaspero F, Garrè ML, Genitori L, Perilongo G, Collini P, Riva D, Valentini L, Scarzello G, Poggi G, Spreafico F, Peretta P, Mascarin M, Modena P, Sozzi G, Bedini N, Biassoni V, Urgesi A, Balestrini MR, Finocchiaro G, Sandri A, Gandola L, AIEOP Neuro-Oncology Group: Salvage treatment for childhood ependymoma after surgery only: Pitfalls of omitting "at once" adjuvant treatment. Int J Radiat Oncol Biol Phys; 2006 Aug 1;65(5):1440-5
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  • [Title] Salvage treatment for childhood ependymoma after surgery only: Pitfalls of omitting "at once" adjuvant treatment.
  • PURPOSE: To discuss the results obtained by giving adjuvant treatment for childhood ependymoma (EPD) at relapse after complete surgery only.
  • METHODS AND MATERIALS: Between 1993 and 2002, 63 children older than 3 years old entered the first Italian Association for Pediatric Hematology and Oncology protocol for EPD (group A), and another 14 patients were referred after relapsing after more tumor excisions only (group B).
  • Diagnoses were classic EPD in 9 patients, anaplastic in 5.
  • All received radiotherapy (RT) to tumor bed and 5 also had pre-RT chemotherapy.
  • CONCLUSIONS: Relapsers after surgery only, especially if with PF-EPD, do worse than those treated after first diagnosis; subsequent surgery for tumor relapse has severe neurologic sequelae.
  • [MeSH-major] Brain Neoplasms / radiotherapy. Ependymoma / radiotherapy. Salvage Therapy / methods
  • [MeSH-minor] Adolescent. Chemotherapy, Adjuvant. Child. Child, Preschool. Female. Humans. Neoplasm, Residual. Radiotherapy, Adjuvant. Treatment Outcome

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  • (PMID = 16863927.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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61. Poulsen HS: [Gliomas in adults: primary non-surgical treatment]. Ugeskr Laeger; 2006 Nov 20;168(47):4082-5
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  • Radiation therapy has a definite place in the treatment of low-grade gliomas, but the timing is a matter of dispute.
  • Anaplastic astrocytomas should be treated with postoperative radiation therapy with or without adjuvant chemotherapy.
  • Anaplastic oligodendroglioma should be treated with radiation therapy only.
  • [MeSH-minor] Adult. Antineoplastic Agents / therapeutic use. Astrocytoma / drug therapy. Astrocytoma / radiotherapy. Chemotherapy, Adjuvant. Ependymoma / drug therapy. Ependymoma / radiotherapy. Glioblastoma / drug therapy. Glioblastoma / radiotherapy. Humans. Oligodendroglioma / drug therapy. Oligodendroglioma / radiotherapy. Radiotherapy Dosage. Radiotherapy, Adjuvant

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  • (PMID = 17134603.001).
  • [ISSN] 1603-6824
  • [Journal-full-title] Ugeskrift for laeger
  • [ISO-abbreviation] Ugeskr. Laeg.
  • [Language] dan
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 10
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62. Buccoliero AM, Castiglione F, Rossi Degl'Innocenti D, Sardi I, Genitori L, Taddei GL: Merlin expression in pediatric anaplastic ependymomas real time PCR study. Fetal Pediatr Pathol; 2010;29(4):245-54
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  • [Title] Merlin expression in pediatric anaplastic ependymomas real time PCR study.
  • The most common genetic abnormalities of ependymomas involve the chromosome 22 where there is the oncosuppressor gene neurofibromin 2 (NF2).
  • In contrast, NF2 alterations do not seem related to tumor grade.
  • We studied the NF2 expression through a real-time polymerase chain reaction in 25 pediatric anaplastic ependymomas.
  • We compared the NF2 expression in neoplastic and non-neoplastic tissues, in supratentorial and infratentorial ependymomas and in primitive and non-primitive tumors (recurrences and metastases).
  • Our results suggest that NF2 alterations are not typical of intracranial anaplastic ependymomas.
  • [MeSH-major] Brain Neoplasms / genetics. Ependymoma / genetics. Neurofibromin 2 / genetics. Reverse Transcriptase Polymerase Chain Reaction / methods
  • [MeSH-minor] Adolescent. Child. Child, Preschool. DNA, Neoplasm / analysis. Female. Gene Expression. Humans. Infant. Infratentorial Neoplasms / genetics. Infratentorial Neoplasms / pathology. Infratentorial Neoplasms / surgery. Male. Neoplasm Metastasis. Neoplasm Recurrence, Local / genetics. Neoplasm Recurrence, Local / pathology. RNA, Messenger / metabolism. Supratentorial Neoplasms / genetics. Supratentorial Neoplasms / pathology. Supratentorial Neoplasms / surgery

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  • (PMID = 20594149.001).
  • [ISSN] 1551-3823
  • [Journal-full-title] Fetal and pediatric pathology
  • [ISO-abbreviation] Fetal Pediatr Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Neurofibromin 2; 0 / RNA, Messenger
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63. Kanamori M, Kumabe T, Saito R, Yamashita Y, Sonoda Y, Tominaga T: [The safety of combination chemotherapy with ifosfamide, cisplatin, and etoposide (ICE): single-institution retrospective review of 108 cases]. No Shinkei Geka; 2010 Nov;38(11):997-1005
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  • The histological diagnosis was newly diagnosed or recurrent germ cell tumor in 45 cases, medulloblastoma in 19, primitive neuroectodermal tumor (PNET) in 7, anaplastic ependymoma in 6, recurrent glioblastoma in 13, and others in 18 cases.
  • 3.0 (CTCAE v3.0) grade 4 neutropenia, anemia, and thrombocytopenia occurred in 81.4%, 14.8%, and 35.2% of patients, respectively.
  • The proportion of ICE cycles associated with CTCAE v3.0 grade 4 neutropenia, transfusion of platelets, and elevated AST/ALT significantly decreased after enforcement of dose reduction criteria.
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents / administration & dosage. Antineoplastic Agents / adverse effects. Antineoplastic Agents, Alkylating / administration & dosage. Antineoplastic Agents, Alkylating / adverse effects. Antineoplastic Agents, Phytogenic / administration & dosage. Antineoplastic Agents, Phytogenic / adverse effects. Child. Child, Preschool. Cisplatin / administration & dosage. Cisplatin / adverse effects. Ependymoma / drug therapy. Etoposide / administration & dosage. Etoposide / adverse effects. Female. Glioblastoma / drug therapy. Hematologic Diseases / chemically induced. Humans. Ifosfamide / administration & dosage. Ifosfamide / adverse effects. Infant. Male. Medulloblastoma / drug therapy. Middle Aged. Neoplasms, Germ Cell and Embryonal / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy. Retrospective Studies

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  • Hazardous Substances Data Bank. CIS-DIAMINEDICHLOROPLATINUM .
  • Hazardous Substances Data Bank. ETOPOSIDE .
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  • (PMID = 21081811.001).
  • [ISSN] 0301-2603
  • [Journal-full-title] No shinkei geka. Neurological surgery
  • [ISO-abbreviation] No Shinkei Geka
  • [Language] jpn
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Antineoplastic Agents, Alkylating; 0 / Antineoplastic Agents, Phytogenic; 6PLQ3CP4P3 / Etoposide; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide
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64. Sugita Y, Ono T, Ohshima K, Niino D, Ito M, Toda K, Baba H: Brain surface spindle cell glioma in a patient with medically intractable partial epilepsy: a variant of monomorphous angiocentric glioma? Neuropathology; 2008 Oct;28(5):516-20
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  • At surgery, a yellowish tumor was localized in the superficial cortex.
  • Histologically, the tumor was predominantly composed of elongated astrocytic cells forming rings around blood vessels.
  • Tumor cells circumferential to vessels predominanted in low cellurarity areas, whereas radial alignment with perivascular pseudorosettes was observed in more cellular regions.
  • These perivascular pseudorosettes closely resembled those of ependymoma.
  • The tumor cells showed variable cytoplasmic immunoreactivity with GFAP.
  • However, in our case the tumor had a small foci of polymorphous appearance and a comparatively high MIB-1 labeling index (8%).
  • However, no de novo anaplastic monomorphous angiocentric glioma similar to our case has yet been reported in the literature.
  • It remains to be determined whether the behavior of monomorphous angiocentric glioma is an example of benign biological characteristics or whether it more closely resembles a low-grade malignant tumor.
  • [MeSH-minor] Diagnosis, Differential. Humans. Immunohistochemistry. Magnetic Resonance Imaging. Male. Malformations of Cortical Development / pathology. Neurosurgical Procedures

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  • (PMID = 18179412.001).
  • [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
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65. 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.
  • [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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66. Wu WX, Yu SZ, Sun CY, Wang Q, Jin SM, An TL: [Detection of chromosomal imbalance in ependymoma by comparative genomic hybridization]. Zhonghua Bing Li Xue Za Zhi; 2009 Mar;38(3):148-52
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  • [Title] [Detection of chromosomal imbalance in ependymoma by comparative genomic hybridization].
  • OBJECTIVE: To investigate genomic DNA imbalances in ependymomas (EDMs) and their correlations with the tumor histological types, grades, locations, patients' gender and age.
  • Both regional gains and losses were mostly seen in myxopapillary EDMs (MPE, WHO grade I), more commonly seen in cellular EDMs (CE, WHO grade II) and tanycytic EDMs (TE, WHO grade II) than in anaplastic EDMs (AE, WHO grade III).
  • CONCLUSIONS: The frequencies of chromosomal imbalances in EDMs decrease as the tumor grade increases.
  • Characteristic chromosomal imbalances in each subtype may play an important role in determination of histological phenotypes and tumor grades.
  • [MeSH-major] Brain Neoplasms / genetics. Chromosome Aberrations. Ependymoma / genetics. Spinal Cord Neoplasms / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Comparative Genomic Hybridization. DNA, Neoplasm / genetics. Female. Humans. Male. Middle Aged. Young Adult

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  • (PMID = 19575847.001).
  • [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; Research Support, Non-U.S. Gov't
  • [Publication-country] China
  • [Chemical-registry-number] 0 / DNA, Neoplasm
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67. Barut F, Kandemir NO, Ozdamar SO, Gul S, Bektas S, Gun BD, Bahadir B: Gliosarcoma with chondroblastic osteosarcomatous differentation: report of two case with clinicopathologic and immunohistochemical features. Turk Neurosurg; 2009 Oct;19(4):417-22
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  • Gliosarcoma is a rare tumor of the central nervous system characterized by a biphasic histological pattern.
  • CASE 1: A 52- year-old male patient underwent parietal craniotomy due to anaplastic ependymoma.
  • After the first operation, additional resections were performed for tumor because of recurrences at the fourth, seventh and tenth months.
  • The patient died after the last tumor resection.
  • Histopathologic examination of the postmortem biopsy revealed neoplasm displaying a biphasic morphologic pattern including both gliomatous and sarcomatous components.
  • We report two cases with an extremely rare histopathological diagnosis of "gliosarcoma with features of chondroblastic osteosarcoma".
  • [MeSH-minor] Aged. Biopsy. Cell Differentiation. Ependymoma / pathology. Ependymoma / surgery. Fatal Outcome. Humans. Immunohistochemistry. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Recurrence, Local / pathology


68. Rudà R, Gilbert M, Soffietti R: Ependymomas of the adult: molecular biology and treatment. Curr Opin Neurol; 2008 Dec;21(6):754-61
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  • [Title] Ependymomas of the adult: molecular biology and treatment.
  • PURPOSE OF REVIEW: To review state of art and relevant advances in the molecular biology and management of ependymomas of the adult.
  • RECENT FINDINGS: Ependymomas of the adult are uncommon neoplasms of the central nervous system, and may occur either in the brain or the spinal cord.
  • Compared with intracranial ependymomas, spinal ependymomas are less frequent and exhibit a better prognosis.
  • Studies performed on genetic changes in ependymoma provide some insight into the pathogenesis and prognostic markers and yield new therapeutic targets, particularly focused on signal transduction modulators.
  • Involved field radiotherapy is recommended for anaplastic or incompletely resected grade II tumors.
  • SUMMARY: Owing to the rarity of the disease, the literature regarding ependymomas in adults is scarce and limited to retrospective series.
  • [MeSH-major] Ependymoma / genetics. Ependymoma / therapy. Molecular Biology / methods

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  • (PMID = 18989122.001).
  • [ISSN] 1350-7540
  • [Journal-full-title] Current opinion in neurology
  • [ISO-abbreviation] Curr. Opin. Neurol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 106
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69. Kano H, Yang HC, Kondziolka D, Niranjan A, Arai Y, Flickinger JC, Lunsford LD: Stereotactic radiosurgery for pediatric recurrent intracranial ependymomas. J Neurosurg Pediatr; 2010 Nov;6(5):417-23
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  • [Title] Stereotactic radiosurgery for pediatric recurrent intracranial ependymomas.
  • OBJECT: To evaluate the role of stereotactic radiosurgery (SRS) in patients with recurrent or residual intracranial ependymomas after resection and fractionated radiation therapy (RT), the authors assessed overall survival, distant tumor relapse, progression-free survival (PFS), and complications.
  • METHODS: The authors retrospectively reviewed the records of 21 children with ependymomas who underwent SRS for 32 tumors.
  • All patients underwent resection of an ependymoma followed by cranial or neuraxis (if spinal metastases was confirmed) RT.
  • Twelve patients had low-grade ependymomas (17 tumors), and 9 patients had anaplastic ependymomas (15 tumors).
  • The median radiosurgical target volume was 2.2 cm(3) (range 0.1-21.4 cm(3)), and the median dose to the tumor margin was 15 Gy (range 9-22 Gy).
  • Factors associated with a longer PFS included patients without spinal metastases (p = 0.033) and tumor volumes < 2.2 cm(3) (median tumor volume 2.2 cm(3), p = 0.029).
  • The distant tumor relapse rate despite RT and SRS was 33.6%, 41.0%, and 80.3% at 1, 2, and 3 years, respectively.
  • Factors associated with a higher rate of distant tumor relapse included patients who had spinal metastases before RT (p = 0.037), a fourth ventricle tumor location (p = 0.002), and an RT to SRS interval < 18 months (p = 0.015).
  • CONCLUSIONS: Stereotactic radiosurgery offers an additional option beyond repeat surgery or RT in pediatric patients with residual or recurrent ependymomas after initial management.
  • [MeSH-major] Brain Neoplasms / surgery. Ependymoma / surgery. Neoplasm Recurrence, Local / surgery. Neoplasm, Residual / surgery. Radiosurgery


70. Iddrissu M, Dakurah T, Wepeba G: Anaplastic ependymoma of the fourth ventricle causing obstrictive hydrocephalus. Ghana Med J; 2005 Mar;39(1):33-6
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  • [Title] Anaplastic ependymoma of the fourth ventricle causing obstrictive hydrocephalus.
  • The case of fourth ventricular anaplastic epednymoma in a four-year-old child is reported in which the initial presentation was deterioration of the level of consciousness secondary to acute obstructive hydrocephalus.
  • The most important prognostic factors are tumour grade and the presence of residual tumour on post operative imaging studies.
  • A median survival of 31 months is noted in children with infratentorial ependymomas and one year survival is quoted as 81%.

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  • (PMID = 17299539.001).
  • [ISSN] 0016-9560
  • [Journal-full-title] Ghana medical journal
  • [ISO-abbreviation] Ghana Med J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Ghana
  • [Other-IDs] NLM/ PMC1790804
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71. Metellus P, Barrie M, Figarella-Branger D, Chinot O, Giorgi R, Gouvernet J, Jouvet A, Guyotat J: Multicentric French study on adult intracranial ependymomas: prognostic factors analysis and therapeutic considerations from a cohort of 152 patients. Brain; 2007 May;130(Pt 5):1338-49
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  • [Title] Multicentric French study on adult intracranial ependymomas: prognostic factors analysis and therapeutic considerations from a cohort of 152 patients.
  • Ependymomas account for 2% of all intracranial tumours in adults.
  • The authors report a retrospective study of a homogenous population of 152 adult patients harbouring intracranial ependymomas from 24 French Neurosurgical Centres between 1990 and 2004.
  • On multivariate analysis, overall survival rates were associated with histological grade (P < 0.001), extent of surgery (P = 0.006), patient age (P = 0.004) and patient Karnofski performance status (P = 0.03).
  • The multivariate analysis also revealed that the risk of recurrence was associated with high histological grade (P < 0.001), incomplete resection (P < 0.001) and Karnofski performance status < or = 80 (P = 0.04).
  • The impact of radiotherapy was found to be beneficial for incompletely resected low-grade ependymomas and to a lesser extent for completely removed high-grade tumours.
  • In association with Karnofski performance status and extent of surgery, histological grade is a major prognostic factor in adult intracranial ependymomas.
  • The application of a simple and reproducible grading scheme using objective anaplastic criteria seems useful practically and clinically applicable.
  • The role of adjuvant radiotherapy for patients with incompletely resected low-grade ependymomas seems to be beneficial but remains to be addressed for high-grade tumours.
  • [MeSH-major] Brain Neoplasms / mortality. Ependymoma / mortality

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  • (PMID = 17449478.001).
  • [ISSN] 1460-2156
  • [Journal-full-title] Brain : a journal of neurology
  • [ISO-abbreviation] Brain
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] England
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72. Benesch M, Siegler N, Hoff Kv, Lassay L, Kropshofer G, Müller H, Sommer C, Rutkowski S, Fleischhack G, Urban C: Safety and toxicity of intrathecal liposomal cytarabine (Depocyte) in children and adolescents with recurrent or refractory brain tumors: a multi-institutional retrospective study. Anticancer Drugs; 2009 Oct;20(9):794-9
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  • Nineteen heavily pretreated patients (males, n = 14; females, n = 5; median age at diagnosis 8.5 years; range, 1.4-22 years) were given intrathecal liposomal cytarabine on a compassionate use basis for recurrent refractory medulloblastoma (n = 12), mixed germ cell tumor (n = 2), central nervous system primitive neuroectodermal tumors of the pons (n = 1), anaplastic ependymoma (n = 1), anaplastic oligodendroglioma (n = 1), atypical teratoid rhabdoid tumor (n = 1), or rhabdoid papillary meningioma (n = 1).
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Compassionate Use Trials. Delayed-Action Preparations. Drug Resistance, Neoplasm. Female. Humans. Infant. Injections, Spinal. Liposomes / administration & dosage. Male. Retrospective Studies. Salvage Therapy. Young Adult

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  • (PMID = 19617818.001).
  • [ISSN] 1473-5741
  • [Journal-full-title] Anti-cancer drugs
  • [ISO-abbreviation] Anticancer Drugs
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; 0 / Delayed-Action Preparations; 0 / Liposomes; 04079A1RDZ / Cytarabine
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73. Alexiou GA, Panagopoulos D, Moschovi M, Stefanaki K, Sfakianos G, Prodromou N: Supratentorial extraventricular anaplastic ependymoma in a 10-year-old girl. Pediatr Neurosurg; 2010;46(6):480-1
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  • [Title] Supratentorial extraventricular anaplastic ependymoma in a 10-year-old girl.
  • [MeSH-major] Ependymoma / pathology. Ependymoma / radiography. Supratentorial Neoplasms / pathology. Supratentorial Neoplasms / radiography

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  • (PMID = 21555909.001).
  • [ISSN] 1423-0305
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
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74. Nakamura Y, Kanemura Y, Yamada T, Sugita Y, Higaki K, Yamamoto M, Takahashi M, Yamasaki M: D2-40 antibody immunoreactivity in developing human brain, brain tumors and cultured neural cells. Mod Pathol; 2006 Jul;19(7):974-85
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  • Some brain tumors such as anaplastic ependymoma, some medulloblastomas, glioblastoma, pineal germinoma, craniopharyngioma, choroid plexus papilloma, choroid plexus carcinoma, and meningioma showed positive immunoreactivity with D2-40.
  • Therefore, D2-40 antibody is considered a useful marker for research on developing brain and diagnosis of brain tumors, differentiation between choroid plexus carcinoma and metastatic carcinoma.
  • [MeSH-major] Antibodies, Monoclonal. Antigens, Neoplasm / analysis. Brain Neoplasms / immunology. Cerebellum / immunology. Telencephalon / immunology

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  • (PMID = 16648867.001).
  • [ISSN] 0893-3952
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Murine-Derived; 0 / Antigens, Neoplasm; 0 / monoclonal antibody D2-40; 0 / oncofetal antigens
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75. Kano H, Niranjan A, Kondziolka D, Flickinger JC, Lunsford LD: Outcome predictors for intracranial ependymoma radiosurgery. Neurosurgery; 2009 Feb;64(2):279-87; discussion 287-8
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  • [Title] Outcome predictors for intracranial ependymoma radiosurgery.
  • OBJECTIVE: To develop outcome predictors after stereotactic radiosurgery (SRS) in patients with intracranial ependymomas who had received previous fractionated radiation therapy, we compared tumor control, survival, and complications with tumor grade, volume, age of patients, and imaging characteristics.
  • METHODS: We retrospectively reviewed records of 39 consecutive ependymoma patients who underwent SRS for 56 tumors.
  • All patients had previous surgical resection of their ependymomas followed by radiotherapy, and 14 patients underwent previous chemotherapy.
  • Twenty-five patients had low-grade ependymomas (34 tumors), and 14 patients had anaplastic ependymomas (22 tumors).
  • The progression-free survival rates after SRS at 1, 3, and 5 years were 81.6, 45.8, and 45.8%, respectively, for all grades of ependymomas.
  • Lower histological tumor grade was not significantly associated with better progression-free survival (P = 0.725).
  • Factors associated with an improved progression-free survival included smaller tumor volume and homogeneous tumor contrast enhancement in low-grade ependymomas.
  • CONCLUSION: SRS provides another management option for patients with residual or recurrent ependymomas that have failed surgery and radiation therapy.
  • [MeSH-major] Brain Neoplasms / epidemiology. Brain Neoplasms / surgery. Ependymoma / epidemiology. Ependymoma / surgery. Neoplasm Recurrence, Local / epidemiology. Neoplasm Recurrence, Local / prevention & control. Outcome Assessment (Health Care) / methods. Radiosurgery / statistics & numerical data


76. Giller CA, Berger BD, Pistenmaa DA, Sklar F, Weprin B, Shapiro K, Winick N, Mulne AF, Delp JL, Gilio JP, Gall KP, Dicke KA, Swift D, Sacco D, Harris-Henderson K, Bowers D: Robotically guided radiosurgery for children. Pediatr Blood Cancer; 2005 Sep;45(3):304-10
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  • Three had pilocytic astrocytomas, two had anaplastic astrocytomas, three had ependymomas (two anaplastic), four had medulloblastomas, three had atypical teratoid/rhabdoid tumors, three had craniopharyngiomas, and three had other pathologies.
  • RESULTS: Local control was achieved in the patients with pilocytic and anaplastic astrocytoma, three of the patients with medulloblastoma, and the three with craniopharyngioma, but not for those with ependymoma.
  • Two of the patients with rhabdoid tumors are alive 16 and 35 months after this diagnosis.
  • CONCLUSION: Cyberknife radiosurgery can be used to achieve local control for some children with CNS tumors without the need for rigid head fixation.

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  • [Copyright] (c) 2004 Wiley-Liss, Inc.
  • (PMID = 15558704.001).
  • [ISSN] 1545-5009
  • [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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77. López-Aguilar E, Sepúlveda-Vildósola AC, Betanzos-Cabrera Y, Gascón-Lastiri G, Ortiz-Suárez L, Rivera-Márquez H, Cerecedo-Díaz F, Wanzke-Del Angel V, De la Cruz-Yáñez H, Ramírez-Reyes G, Arenas-Aranda D, Siordia-Reyes G: [Prognostic and survival factors among pediatric patients with ependymomas]. Gac Med Mex; 2009 Jan-Feb;145(1):7-13
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  • [Title] [Prognostic and survival factors among pediatric patients with ependymomas].
  • BACKGROUND: Ependymomas constitute the third most common intracranial tumors in children.
  • OBJECTIVE: Determine global survival of patients with ependymomas according to different prognostic factors.
  • METHODS: We reviewed the medical charts of every pediatric patient with ependymoma from 1996 to 2005.
  • The presence of chromosomal imbalances, particularly in chromosome 21, significantly affected survival Being under 5 years of age, anaplastic histology, chemotherapy other than ICE (ifosfamida-carboplatin-etoposide) and partial resection increased the risk of death.
  • [MeSH-major] Brain Neoplasms / mortality. Ependymoma / mortality

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  • (PMID = 19256405.001).
  • [ISSN] 0016-3813
  • [Journal-full-title] Gaceta médica de México
  • [ISO-abbreviation] Gac Med Mex
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Mexico
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78. Timmermann B, Kortmann RD, Kühl J, Rutkowski S, Dieckmann K, Meisner C, Bamberg M: Role of radiotherapy in anaplastic ependymoma in children under age of 3 years: results of the prospective German brain tumor trials HIT-SKK 87 and 92. Radiother Oncol; 2005 Dec;77(3):278-85
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  • [Title] Role of radiotherapy in anaplastic ependymoma in children under age of 3 years: results of the prospective German brain tumor trials HIT-SKK 87 and 92.
  • BACKGROUND AND PURPOSE: To evaluate the outcome of very young children with anaplastic ependymoma after delayed or omitted radiotherapy (RT).
  • MATERIALS AND METHODS: Children under age of 3 years with anaplastic ependymoma were enrolled in the HIT-SKK 87 trial from 1987.
  • RESULTS: Thirty-four children with anaplastic ependymoma were eligible (age 1.0-33.0 months).
  • Predominant site of failure is the primary tumor site.
  • [MeSH-major] Brain Neoplasms / radiotherapy. Ependymoma / radiotherapy


79. Denaro L, Gardiman M, Calderone M, Rossetto M, Ciccarino P, Giangaspero F, Perilongo G, d'Avella D: Intraventricular astroblastoma. Case report. J Neurosurg Pediatr; 2008 Feb;1(2):152-5
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  • Astroblastoma is a rare primary brain neoplasm that accounts for 0.45-2.8% of brain gliomas.
  • Macroscopic radical resection of the tumor was performed via the superior temporal sulcus.
  • In patients harboring anaplastic astroblastoma, gross-total resection and adjuvant therapy after the initial surgery seems to be the best choice.
  • It is important to distinguish astroblastoma from ependymoma in clinical practice because of the differences in therapeutic approaches.
  • [MeSH-major] Cerebral Ventricle Neoplasms / diagnosis. Neoplasms, Neuroepithelial / diagnosis

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  • (PMID = 18352788.001).
  • [ISSN] 1933-0707
  • [Journal-full-title] Journal of neurosurgery. Pediatrics
  • [ISO-abbreviation] J Neurosurg Pediatr
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 26
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80. Oshiro S, Tsugu H, Komatsu F, Ohmura T, Ohta M, Sakamoto S, Fukushima T, Inoue T: Efficacy of temozolomide treatment in patients with high-grade glioma. Anticancer Res; 2009 Mar;29(3):911-7
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  • [Title] Efficacy of temozolomide treatment in patients with high-grade glioma.
  • BACKGROUND: Numerous studies have reported the clinical efficacy of temozolomide (TMZ) treatment for high-grade glioma, but information on Japanese populations has been limited.
  • PATIENTS AND METHODS: The subjects comprised ten patients with high-grade glioma [glioblastoma multiforme (GBM), n=3, gliosarcoma (GS), n=1, anaplastic oligodendroglioma (AO), n=3, anaplastic mixed oligoastrocytoma (AOA), n=1, and anaplastic ependymoma (AE), n=2].
  • As second- or third-line chemotherapy, patients received TMZ for recurrence or tumor progression.
  • As combination therapy, the local administration of tumor necrosis factor-alpha and the addition of carboplatin and etoposide were included for three patients during the course of oral TMZ treatment.
  • One of the patients receiving combination therapy has continued to show shrinkage of the relapsed tumor.
  • Despite prior radio- and chemotherapy, most patients experienced only grade 1-2 hematotoxicity that was well-controlled by conservative therapy.
  • CONCLUSION: TMZ chemotherapy is effective for the treatment of high-grade glioma in some patients without serious toxicity.
  • [MeSH-minor] Adult. Aged. Carboplatin / administration & dosage. Chemotherapy, Adjuvant. Combined Modality Therapy. Dacarbazine / administration & dosage. Dacarbazine / analogs & derivatives. Etoposide / administration & dosage. Female. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Prognosis. Treatment Outcome. Tumor Necrosis Factor-alpha / therapeutic use

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  • (PMID = 19414327.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Tumor Necrosis Factor-alpha; 6PLQ3CP4P3 / Etoposide; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide; BG3F62OND5 / Carboplatin
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81. Ghosal N, Murthy G, Dadlani R, Hegde AS, Singh D: Recurrent posterior fossa anaplastic ependymoma with prominent chondroid metaplasia: a case report and review of literature. Indian J Pathol Microbiol; 2010 Oct-Dec;53(4):787-9
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  • [Title] Recurrent posterior fossa anaplastic ependymoma with prominent chondroid metaplasia: a case report and review of literature.
  • We report an unusual case of a recurrent fourth ventricular anaplastic ependymoma with prominent chondroid metaplasia in a 16-year-old male.
  • On initial presentation, the patient had a WHO Grade II tumor.
  • However, at recurrence 1 year later, the tumor progressed to WHO Grade III tumor with more cellularity, necrosis and brisk mitotic activity.
  • [MeSH-major] Brain Neoplasms / diagnosis. Brain Neoplasms / pathology. Cranial Fossa, Posterior / pathology. Ependymoma / diagnosis. Ependymoma / pathology. Metaplasia / pathology


82. Schneider D, Monoranu CM, Huang B, Rutkowski S, Gerber NU, Krauss J, Puppe B, Roggendorf W: Pediatric supratentorial ependymomas show more frequent deletions on chromosome 9 than infratentorial ependymomas: a microsatellite analysis. Cancer Genet Cytogenet; 2009 Jun;191(2):90-6
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  • [Title] Pediatric supratentorial ependymomas show more frequent deletions on chromosome 9 than infratentorial ependymomas: a microsatellite analysis.
  • In our previous screening study in ependymomas, we used microsatellite analysis to identify frequent aberrations on this chromosome.
  • A total of 48 pairs of matched normal and tumor specimens from patients with ependymoma, including 28 children (mean age, 4.4 years) and 20 adults (mean age, 44.9 years), were genotyped.
  • Pediatric tumors, which were predominantly anaplastic, showed fewer aberrations (57.1%) than adult tumors (70%), and two common regions of deletions were identified (9p21.1 approximately p22.3 and 9q31.3 approximately q33.2).
  • Adults with ependymomas harboring aberrations on chromosome 9 (n=14) showed significantly longer overall survival than patients of the same group without this aberration (n=6; P=0.034), irrespective of the extent of resection in multivariate analysis.
  • Aberrations of chromosome 9, and particularly of DCR1, may play a role in the prognostic evaluation for ependymomas in adults in the future.
  • [MeSH-major] Allelic Imbalance / genetics. Brain Neoplasms / genetics. Chromosome Deletion. Chromosomes, Human, Pair 9. Ependymoma / genetics. Microsatellite Repeats / genetics
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Chromosome Aberrations. DNA, Neoplasm / genetics. DNA, Neoplasm / isolation & purification. Female. Genotype. Humans. Male. Middle Aged. Polymorphism, Genetic

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  • (PMID = 19446744.001).
  • [ISSN] 1873-4456
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm
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83. Buccoliero AM, Castiglione F, Rossi Degl'Innocenti D, Paglierani M, Maio V, Gheri CF, Garbini F, Moncini D, Taddei A, Sardi I, Sanzo M, Giordano F, Mussa F, Genitori L, Taddei GL: O6-Methylguanine-DNA-methyltransferase in recurring anaplastic ependymomas: PCR and immunohistochemistry. J Chemother; 2008 Apr;20(2):263-8
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  • [Title] O6-Methylguanine-DNA-methyltransferase in recurring anaplastic ependymomas: PCR and immunohistochemistry.
  • Ependymomas are the third most common brain tumor in children.
  • We evaluated the MGMT gene promoter methylation and the immunohistochemical MGMT protein expression in 12 recurrent anaplastic ependymomas affecting children.
  • Our purpose was to investigate the molecular rationale of the administration of alkylating agents to children affected by recurrent anaplastic ependymomas.
  • All ependymomas lacked MGMT promoter hypermethylation and 9 (75%) showed high MGMT protein expression (>50% tumoral cells).
  • These results may indicate MGMT as a factor of chemoresistance to alkylating drugs in anaplastic ependymomas and support the uncertainties regarding the actual benefit of chemotherapy for patients with anaplastic ependymomas.
  • [MeSH-major] Brain Neoplasms / enzymology. DNA Modification Methylases / biosynthesis. DNA Repair Enzymes / biosynthesis. Ependymoma / enzymology. Neoplasm Recurrence, Local / enzymology. Tumor Suppressor Proteins / biosynthesis
  • [MeSH-minor] Adolescent. Anaplasia. Child. Child, Preschool. DNA Methylation. Drug Resistance, Neoplasm. Female. Humans. Immunohistochemistry. Male. Polymerase Chain Reaction. Promoter Regions, Genetic

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  • (PMID = 18467255.001).
  • [ISSN] 1973-9478
  • [Journal-full-title] Journal of chemotherapy (Florence, Italy)
  • [ISO-abbreviation] J Chemother
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Tumor Suppressor Proteins; EC 2.1.1.- / DNA Modification Methylases; EC 2.1.1.63 / MGMT protein, human; EC 6.5.1.- / DNA Repair Enzymes
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84. El-Bahy K: Telovelar approach to the fourth ventricle: operative findings and results in 16 cases. Acta Neurochir (Wien); 2005 Feb;147(2):137-42; discussion 142
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  • The inferior medullary velum was thinned out and stretched as a neural tissue sheet over the tumour surface in 10 cases (4 ependymomas, 2 meningiomas, 2 epidermoids, one dermoid and one choroid plexus papilloma).
  • Subtotal removal was achieved in the remaining patients (31.25%); three ependymomas, one medulloblastoma, and one anaplastic astrocytoma.
  • [MeSH-minor] Adolescent. Adult. Astrocytoma / pathology. Astrocytoma / surgery. Cerebellar Ataxia / etiology. Cerebellar Ataxia / pathology. Cerebellar Ataxia / physiopathology. Child. Choroid Plexus / pathology. Choroid Plexus / surgery. Cranial Fossa, Posterior / surgery. Dermoid Cyst / pathology. Dermoid Cyst / surgery. Ependymoma / pathology. Ependymoma / surgery. Female. Humans. Magnetic Resonance Imaging. Male. Medulloblastoma / pathology. Medulloblastoma / surgery. Middle Aged. Neoplasm Recurrence, Local / prevention & control. Papilloma, Choroid Plexus / pathology. Papilloma, Choroid Plexus / surgery. Postoperative Complications / etiology. Postoperative Complications / pathology. Postoperative Complications / physiopathology. Retrospective Studies. Treatment Outcome

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  • (PMID = 15605202.001).
  • [ISSN] 0001-6268
  • [Journal-full-title] Acta neurochirurgica
  • [ISO-abbreviation] Acta Neurochir (Wien)
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] Austria
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85. Nafe R, Yan B, Schlote W, Schneider B: Application of different methods for nuclear shape analysis with special reference to the differentiation of brain tumors. Anal Quant Cytol Histol; 2006 Apr;28(2):69-77
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  • STUDY DESIGN: At least 300 tumor cell nuclei per case were measured by means of a digital image analysis system.
  • (1) oligodendrogliomas WHO grade II (n = 13) vs. grade III (n = 11), (2) medulloblastomas WHO grade IV (n = 14) vs. anaplastic ependymomas WHO grade III (n = 12), (3) Ki-67-positive vs. Ki-67-negative tumor cell nuclei in the 14 medulloblastomas.
  • CONCLUSION: Fourier analysis provided an optimal statistical discrimination between different brain tumor entities and between data sets from proliferating and nonproliferating tumor cell nuclei.

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  • (PMID = 16637509.001).
  • [ISSN] 0884-6812
  • [Journal-full-title] Analytical and quantitative cytology and histology
  • [ISO-abbreviation] Anal. Quant. Cytol. Histol.
  • [Language] ENG
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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86. Shu HK, Sall WF, Maity A, Tochner ZA, Janss AJ, Belasco JB, Rorke-Adams LB, Phillips PC, Sutton LN, Fisher MJ: Childhood intracranial ependymoma: twenty-year experience from a single institution. Cancer; 2007 Jul 15;110(2):432-41
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  • [Title] Childhood intracranial ependymoma: twenty-year experience from a single institution.
  • BACKGROUND: Because few large studies of pediatric ependymoma treatment are available, the authors believed that a retrospective review of treatment outcomes from a single institution would yield potentially valuable information regarding potential prognostic factors.
  • METHODS: Medical records were reviews of patients with intracranial ependymoma who received their initial treatment at the Children's Hospital of Philadelphia (CHOP)/Hospital of the University of Pennsylvania (HUP) between January 1980 and December 2000.
  • Of the 61 patients who were identified, 49 patients underwent primary therapy at CHOP/HUP and formed the basis for the study.
  • Anaplastic histology predicted for decreased PFS.
  • Patients who had a favorable prognosis (aged >/=3 years, no dissemination or cord extension, complete resection, and radiation dose >/=54 grays [Gy]) had 5-year OS and PFS rates of 83.1% and 60.6%, respectively.
  • CONCLUSIONS: In this study of patients with pediatric intracranial ependymoma, OS and PFS rates were concordant with the rates published in other modern series.
  • Tumor extension to the cervical spine was identified as a predictor for failure outside of the primary site.
  • [MeSH-major] Brain Neoplasms / pathology. Ependymoma / pathology


87. Busse C, Nazeer T, Kanwar VS, Wolden S, LaQuaglia MP, Rosenblum M: Sacrococcygeal immature teratoma with malignant ependymoma component. Pediatr Blood Cancer; 2009 Oct;53(4):680-1
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  • [Title] Sacrococcygeal immature teratoma with malignant ependymoma component.
  • [MeSH-major] Ependymoma / pathology. Sacrococcygeal Region. Teratoma / pathology


88. Sangoi AR, Lim M, Dulai M, Vogel H, Chang S: Suprasellar giant cell ependymoma: a rare neoplasm in a unique location. Hum Pathol; 2008 Sep;39(9):1396-401
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  • [Title] Suprasellar giant cell ependymoma: a rare neoplasm in a unique location.
  • Ependymomas are glial tumors that usually present in the posterior fossa in children and in the spinal cord in adults.
  • Giant cell ependymoma, a rare ependymal subtype only recently recognized as a diagnostic entity in the last decade, demonstrates pleomorphic giant cells admixed with features of typical ependymoma.
  • Although only 8 giant cell ependymomas have been reported to date, none have been reported in the suprasellar space.
  • Moreover, as these neoplasms demonstrate a high incidence of anaplastic grade, recognition of this ependymal subtype is paramount.
  • We describe the presentation and pertinent radiologic, histologic, immunologic, and ultrastructural findings in conjunction with relevant clinical implications of the first reported case of a suprasellar giant cell ependymoma occurring in a 34-year-old female 7 years after an initial diagnosis of a medullary ependymoma with rare atypical giant cells, a potential tumor seeding culprit.
  • [MeSH-major] Ependymoma / pathology. Supratentorial Neoplasms / pathology

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  • (PMID = 18602668.001).
  • [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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89. Cerase A, Venturi C, Oliveri G, De Falco D, Miracco C: Intradural extramedullary spinal anaplastic ependymoma. Case illustration. J Neurosurg Spine; 2006 Nov;5(5):476
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  • [Title] Intradural extramedullary spinal anaplastic ependymoma. Case illustration.
  • [MeSH-major] Ependymoma / pathology. Ependymoma / surgery. Spinal Cord Neoplasms / pathology. Spinal Cord Neoplasms / surgery

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  • (PMID = 17120902.001).
  • [ISSN] 1547-5654
  • [Journal-full-title] Journal of neurosurgery. Spine
  • [ISO-abbreviation] J Neurosurg Spine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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90. Hamano E, Tsutsumi S, Nonaka Y, Abe Y, Yasumoto Y, Saeki H, Ito M: Huge supratentorial extraventricular anaplastic ependymoma presenting with massive calcification--case report. Neurol Med Chir (Tokyo); 2010;50(2):150-3
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  • [Title] Huge supratentorial extraventricular anaplastic ependymoma presenting with massive calcification--case report.
  • A 15-year-old boy presented with an anaplastic supratentorial ependymoma causing massive intratumoral calcification, without contributory medical and family history, and manifesting as persistent headache for 2 months.
  • Magnetic resonance imaging confirmed the tumor as an assembly of medullated masses with extraventricular location, 7 x 6.5 x 6.5 cm in diameter, and appearing as heterogeneous intensity on both T(1)- and T(2)-weighted images with inhomogeneous enhancement except for the central cores.
  • The patient underwent tumor resection.
  • Intraoperative findings revealed that the cortical veins overlying the tumor were reddish and moderately engorged.
  • The hypervascular tumor, entirely extraventricular in location, was totally resected without neurological deterioration.
  • Histological examination revealed that the tumor was highly cellular with hyperchromatic nuclei and cell atypia.
  • The findings were compatible with anaplastic ependymoma (World Health Organization classification grade 3).
  • Ependymoma should be included in the differential diagnosis of a supratentorially located, extraventricular mass with massive intratumoral calcification.
  • [MeSH-major] Brain Neoplasms / pathology. Calcinosis / pathology. Cerebrum / pathology. Ependymoma / pathology
  • [MeSH-minor] Adolescent. Biomarkers, Tumor / analysis. Biomarkers, Tumor / metabolism. Brain Edema / etiology. Brain Edema / pathology. Brain Edema / radiography. Cerebral Veins / pathology. Disease Progression. Humans. Lateral Ventricles / pathology. Magnetic Resonance Imaging. Male. Mitotic Index. Neoplasm Invasiveness / pathology. Neoplasm Invasiveness / physiopathology. Neurosurgical Procedures. Occipital Lobe / blood supply. Occipital Lobe / pathology. Occipital Lobe / surgery. Parietal Lobe / blood supply. Parietal Lobe / pathology. Parietal Lobe / surgery. Skull / pathology. Skull / radiography. Tomography, X-Ray Computed. Treatment Outcome. Vision, Low / etiology

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  • (PMID = 20185883.001).
  • [ISSN] 1349-8029
  • [Journal-full-title] Neurologia medico-chirurgica
  • [ISO-abbreviation] Neurol. Med. Chir. (Tokyo)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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91. Shervington A, Patel R, Lu C, Cruickshanks N, Lea R, Roberts G, Dawson T, Shervington L: Telomerase subunits expression variation between biopsy samples and cell lines derived from malignant glioma. Brain Res; 2007 Feb 23;1134(1):45-52
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  • [Title] Telomerase subunits expression variation between biopsy samples and cell lines derived from malignant glioma.
  • In this study, a recurrent anaplastic ependymoma and seven glioblastoma biopsy samples, four cell lines and four controls including two normal brain tissues were analysed for telomerase subunit expression profiles together with telomerase activity.
  • Tankyrase was expressed in 85% of the glioblastoma tissues and was down-regulated in the recurrent anaplastic ependymoma tissue control cell lines.
  • Dyskerin was expressed in all cell lines and tissues apart from U87-MG and NHA cells and the recurrent anaplastic ependymoma tissue.
  • [MeSH-major] Biomarkers, Tumor / genetics. Brain Neoplasms / enzymology. Brain Neoplasms / genetics. Glioma / enzymology. Glioma / genetics. Telomerase / genetics
  • [MeSH-minor] Biopsy. Carrier Proteins / genetics. Cell Cycle Proteins / genetics. Cell Line, Tumor. Cell Transformation, Neoplastic / genetics. Cell Transformation, Neoplastic / metabolism. Gene Expression Regulation, Neoplastic / genetics. Humans. Nuclear Proteins / genetics. Tankyrases / genetics

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  • (PMID = 17196947.001).
  • [ISSN] 0006-8993
  • [Journal-full-title] Brain research
  • [ISO-abbreviation] Brain Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Carrier Proteins; 0 / Cell Cycle Proteins; 0 / DKC1 protein, human; 0 / Nuclear Proteins; 0 / TEP1 protein, human; EC 2.4.2.30 / Tankyrases; EC 2.4.4.30 / TNKS protein, human; EC 2.7.7.49 / TERT protein, human; EC 2.7.7.49 / Telomerase
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92. 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.
  • Ependymomas account for 3-9% of all neuroepithelial tumors.
  • 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.
  • Histological, immunohistochemical and electron microscopic findings were consistent with high-grade ependymoma.
  • 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.
  • [MeSH-major] Brain Neoplasms / pathology. Ependymoma / pathology. Lateral Ventricles

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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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93. Niazi TN, Jensen EM, Jensen RL: WHO Grade II and III supratentorial hemispheric ependymomas in adults: case series and review of treatment options. J Neurooncol; 2009 Feb;91(3):323-8
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  • [Title] WHO Grade II and III supratentorial hemispheric ependymomas in adults: case series and review of treatment options.
  • Supratentorial ependymomas and their anaplastic variants are relatively uncommon central nervous system neoplasms that afflict both adults and children.
  • In our case series of three adult patients with supratentorial ependymomas, two patients had tumors of WHO Grade III (anaplastic variant) and one had tumor of WHO Grade II.
  • Additional radiation therapy was administered in the Grade III patients.
  • Twenty-four-month follow-up in case 1 yielded no tumor recurrence and no requirement of adjuvant chemotherapy.
  • In case 2, tumor recurred with leptomeningeal gliomatosis by 6 months.
  • In case 3 (WHO Grade II), no radiation therapy was required.
  • Tumor did not recur during the 42-month follow-up.
  • In our experience, gross total resection was achieved in all patients with hemispheric supratentorial WHO Grade II or Grade III ependymomas with additional radiation therapy for Grade III variants.
  • The role of chemotherapy is still uncertain but may be necessary in younger patients who may have tumors that behave more like the pediatric ependymomas.
  • [MeSH-major] Brain Neoplasms / pathology. Brain Neoplasms / therapy. Ependymoma / pathology. Ependymoma / therapy. Frontal Lobe / pathology. Functional Laterality

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  • (PMID = 18974933.001).
  • [ISSN] 1573-7373
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 18
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94. Asano K, Miyamoto S, Kubo O, Kikkukawa T, Yagihashi A, Ohkuma H: A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination. Brain Tumor Pathol; 2006 Apr;23(1):55-63
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A case of anaplastic pleomorphic xanthoastrocytoma presenting with tumor bleeding and cerebrospinal fluid dissemination.
  • Pleomorphic xanthoastrocytoma (PXA) has been considered an astrocytic tumor with a relatively favorable prognosis.
  • However, PXA cases having several recurrent patterns with poor prognosis have been reported in recent years, and a new concept of anaplastic PXA has been proposed.
  • The present case was a 59-year-old woman who presented with tumor bleeding onset and cerebrospinal fluid dissemination.
  • After emergency surgery had removed the hematoma, postoperative contrast-enhanced CT scan revealed a left temporal tumor.
  • A second surgery was therefore performed for initial tumor removal 2 months later.
  • Histopathological findings showed that the tumor was typical PXA with strong pleomorphism and xanthomatous changes and contained an ependymoma-like component in the center area.
  • From these findings, the histopathological diagnosis was anaplastic PXA.
  • This case report is the first case in which PXA presented with tumor bleeding onset.
  • Histopathological findings suggested anaplastic PXA from the first surgical specimens, and PXA recurred many times.
  • We thus believe that the patient displayed primary anaplastic PXA rather than secondary anaplastic PXA that results in malignant transformation.
  • [MeSH-minor] Biomarkers, Tumor. Fatal Outcome. Female. Humans. Image Processing, Computer-Assisted. Magnetic Resonance Imaging. Middle Aged. Mitosis / physiology. Neoplasm Recurrence, Local. Tomography, X-Ray Computed

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  • (PMID = 18095120.001).
  • [ISSN] 1433-7398
  • [Journal-full-title] Brain tumor pathology
  • [ISO-abbreviation] Brain Tumor Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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95. Sasayama T, Nishihara M, Kondoh T, Hosoda K, Kohmura E: MicroRNA-10b is overexpressed in malignant glioma and associated with tumor invasive factors, uPAR and RhoC. Int J Cancer; 2009 Sep 15;125(6):1407-13
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] MicroRNA-10b is overexpressed in malignant glioma and associated with tumor invasive factors, uPAR and RhoC.
  • Although the oncogenic and tumor suppressive functions of several miRNAs have been characterized, the role of miRNAs in mediating tumor invasion and migration remains largely unexplored.
  • Here, we performed real-time reverse transcriptase polymerase chain reaction (RT-PCR) assays on 43 glioma samples (17 glioblastoma, 6 anaplastic astrocytoma, 10 low-grade astrocytoma, 6 oligodendroglioma and 4 ependymoma) and 6 glioma cell lines.
  • The expression levels of miR-10b were associated with higher grade glioma.
  • In addition, mRNA expressions of RhoC and urokinase-type plasminogen activator receptor (uPAR), which were thought to be regulated by miR-10b via HOXD10, were statistically significantly correlated with the expression of miR-10b (p < 0.001, p = 0.001, respectively).
  • Finally, multifocal lesions on enhanced MRI of 7 malignant gliomas were associated with higher expression levels of miR-10b (p = 0.02).
  • [MeSH-minor] Biomarkers, Tumor / genetics. Biomarkers, Tumor / metabolism. Humans. Immunoenzyme Techniques. Neoplasm Invasiveness. Neoplasm Staging. Oligonucleotide Array Sequence Analysis. RNA, Messenger / genetics. RNA, Messenger / metabolism. Reverse Transcriptase Polymerase Chain Reaction. Tumor Cells, Cultured. Up-Regulation

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  • [Copyright] 2009 UICC
  • (PMID = 19536818.001).
  • [ISSN] 1097-0215
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / LILRB2 protein, human; 0 / Membrane Glycoproteins; 0 / RHOC protein, human; 0 / RNA, Messenger; 0 / Receptors, Immunologic; 0 / Receptors, Urokinase Plasminogen Activator; EC 3.6.5.2 / rho GTP-Binding Proteins
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96. Aghili M, Zahedi F, Rafiee E: Hydroxyglutaric aciduria and malignant brain tumor: a case report and literature review. J Neurooncol; 2009 Jan;91(2):233-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Hydroxyglutaric aciduria and malignant brain tumor: a case report and literature review.
  • To make a definitive diagnosis, an anomalous accumulation of L -2-hydroxyglutaric acid must be detected in body fluids.
  • Here, we present a 17-year-old boy with L: -2-OHGA who developed an anaplastic ependymoma during the course of this disease.
  • We also present a literature review including seven other patients who developed malignant brain tumors during the course of L -2-OHGA.
  • [MeSH-major] Brain Diseases, Metabolic, Inborn / complications. Ependymoma / complications. Frontal Lobe / pathology. Hydroxy Acids / urine


97. Lin YH, Huang CI, Wong TT, Chen MH, Shiau CY, Wang LW, Ming-Tak Ho D, Yen SH: Treatment of spinal cord ependymomas by surgery with or without postoperative radiotherapy. J Neurooncol; 2005 Jan;71(2):205-10
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of spinal cord ependymomas by surgery with or without postoperative radiotherapy.
  • PURPOSE: To evaluate the effectiveness of complete resection and postoperative radiotherapy in spinal cord ependymomas.
  • METHODS AND MATERIALS: We conducted a retrospective study over 20 patients (13 males and 7 females) with histologically confirmed spinal cord ependymomas between July 1985 and April 2001.
  • Among them, 13 patients had ependymomas, 6 had myxopapillary ependymomas, and 1 had anaplastic ependymoma.
  • All patients received radical surgery for tumor removal with 13 patients achieving complete resection and 7 incomplete resection due to technical difficulty.
  • Among those with incomplete resection, 6 patients received postoperative radiotherapy to tumor bed and only one patient with anaplastic ependymoma received surgery alone.
  • The total tumor dose ranged from 50 to 60 Gy.
  • One patient with anaplastic ependymoma and no postoperative radiotherapy developed leptomeningeal seeding 9 months after surgery.
  • The patient expired 34 months from the initial diagnosis due to progression of leptomeningeal seeding.
  • CONCLUSION: Complete resection alone in spinal cord ependymoma can achieve excellent local control and survival.
  • [MeSH-major] Ependymoma / radiotherapy. Ependymoma / surgery. Spinal Cord Neoplasms / radiotherapy. Spinal Cord Neoplasms / surgery
  • [MeSH-minor] Adult. Aged. Child. Combined Modality Therapy. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local / therapy. Nervous System / physiopathology. Retrospective Studies. Salvage Therapy. Survival Analysis. Treatment Outcome

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  • (PMID = 15690140.001).
  • [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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98. Schroeder TM, Chintagumpala M, Okcu MF, Chiu JK, Teh BS, Woo SY, Paulino AC: Intensity-modulated radiation therapy in childhood ependymoma. Int J Radiat Oncol Biol Phys; 2008 Jul 15;71(4):987-93
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Intensity-modulated radiation therapy in childhood ependymoma.
  • PURPOSE: To determine the patterns of failure after intensity-modulated radiation therapy (IMRT) for localized intracranial ependymoma.
  • METHODS AND MATERIALS: From 1994 to 2005, 22 children with pathologically proven, localized, intracranial ependymoma were treated with adjuvant IMRT.
  • Of the patients, 12 (55%) had an infratentorial tumor and 14 (64%) had anaplastic histology.
  • The clinical target volume encompassed the tumor bed and any residual disease plus margin (median dose 54 Gy).
  • [MeSH-major] Brain Neoplasms / radiotherapy. Ependymoma / radiotherapy. Neoplasm Recurrence, Local / prevention & control. Neoplasm Recurrence, Local / radiotherapy. Radiotherapy, Conformal / methods

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  • (PMID = 18258381.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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99. Torii K, Tsuyuguchi N, Kawabe J, Sunada I, Hara M, Shiomi S: Correlation of amino-acid uptake using methionine PET and histological classifications in various gliomas. Ann Nucl Med; 2005 Dec;19(8):677-83
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OBJECTIVE: The uptake of L-methyl-11C-methionine (MET) by gliomas is greater than that by intact tissue, making methionine very useful for evaluation of tumor extent.
  • Tumors included diffuse astrocytoma, anaplastic astrocytoma, glioblastoma, ependymoma, oligodendroglioma, medulloblastoma, dysembryoplastic neuroepithelial tumor, choroid plexus papilloma, central neurocytoma, optic glioma, gliomatosis cerebri, pleomorphic xanthoastrocytoma, and ganglioglioma.
  • Tumor activity and degree of malignancy were evaluated using Ki-67LI (LI: labeling index) and Kaplan-Meier survival curves.
  • The correlations between methionine uptake and tumor proliferation (tumor versus contralateral gray matter ratio (T/N) and Ki-67LI) were determined for the group of all subjects.
  • Ki-67LI differed significantly between the high-grade group and low-grade group at T/N levels between 1.5 and 1.8 on analysis using tumor proliferative potential (p = 0.019-0.031).
  • The prognosis differed significantly between the high-grade and low-grade groups when T/N was in the range of 1.6-1.8 (p = 0.028-0.032).
  • CONCLUSIONS: When analysis was confined to cases of astrocytic tumor, a correlation was noted between methionine accumulation and Ki-67LI.
  • The cut-off level of T/N ratio for distinction between high-grade and low-grade astrocytoma appears to lie between 1.5 and 1.6.


100. Saito R, Kumabe T, Kanamori M, Sonoda Y, Tominaga T: Dissemination limits the survival of patients with anaplastic ependymoma after extensive surgical resection, meticulous follow up, and intensive treatment for recurrence. Neurosurg Rev; 2010 Apr;33(2):185-91; discussion 191-2
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Dissemination limits the survival of patients with anaplastic ependymoma after extensive surgical resection, meticulous follow up, and intensive treatment for recurrence.
  • The extent of resection is the most consistent factor affecting outcome of intracranial ependymomas.
  • The outcomes in patients with intracranial anaplastic ependymomas who underwent more than subtotal resection and intensive treatment for recurrence were reviewed retrospectively.
  • High resection rate, meticulous follow-up, and intensive treatment for recurrence improved the survival of patients with anaplastic ependymoma.
  • [MeSH-major] Brain Neoplasms / mortality. Brain Neoplasms / pathology. Ependymoma / mortality. Ependymoma / pathology
  • [MeSH-minor] Adolescent. Adult. Chemotherapy, Adjuvant. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Kaplan-Meier Estimate. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Recurrence, Local / therapy. Radiotherapy, Adjuvant. Retrospective Studies. Survival Rate






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