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1. Kao CL, Chiou SH, Chen YJ, Singh S, Lin HT, Liu RS, Lo CW, Yang CC, Chi CW, Lee CH, Wong TT: Increased expression of osteopontin gene in atypical teratoid/rhabdoid tumor of the central nervous system. Mod Pathol; 2005 Jun;18(6):769-78
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Increased expression of osteopontin gene in atypical teratoid/rhabdoid tumor of the central nervous system.
  • The atypical teratoid/rhabdoid tumor, primary to the central nervous system, is a highly malignant and aggressive neoplasm of infancy and childhood.
  • Although having distinct biological features and clinical outcomes, it is frequently misdiagnosed as primitive neuroectodermal tumor/medulloblastoma.
  • To further distinguish the underlying pathogenesis and to identify biological markers for clinical use, an atypical teratoid/rhabdoid tumor-derived cell line was established and its gene expression pattern analyzed in comparison to the human astrocyte SVG12 cell line and the human DAOY medulloblastoma cell line using a complementary DNA microarray method.
  • The osteopontin gene was found specifically upregulated in atypical teratoid/rhabdoid tumor cells.
  • This specificity was confirmed by immunohistochemistry in pathological sections of tissues from atypical teratoid/rhabdoid tumor patients.
  • Even though the role of osteopontin in the cytopathogenesis of atypical teratoid/rhabdoid tumor still needs to be determined, our data support that overexpressed osteopontin is a potential diagnostic marker for atypical teratoid/rhabdoid tumor.
  • [MeSH-major] Central Nervous System Neoplasms / pathology. Gene Expression Profiling. Rhabdoid Tumor / pathology. Sialoglycoproteins / genetics. Teratoma / pathology
  • [MeSH-minor] Cell Line. Cell Line, Tumor. Cluster Analysis. Gene Expression Regulation, Neoplastic / genetics. Humans. Immunohistochemistry. Medulloblastoma / genetics. Medulloblastoma / metabolism. Medulloblastoma / pathology. Nucleic Acid Hybridization / methods. Oligonucleotide Array Sequence Analysis. Osteopontin. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 15776015.001).
  • [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 / SPP1 protein, human; 0 / Sialoglycoproteins; 106441-73-0 / Osteopontin
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2. Nicolaides T, Tihan T, Horn B, Biegel J, Prados M, Banerjee A: High-dose chemotherapy and autologous stem cell rescue for atypical teratoid/rhabdoid tumor of the central nervous system. J Neurooncol; 2010 May;98(1):117-23
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] High-dose chemotherapy and autologous stem cell rescue for atypical teratoid/rhabdoid tumor of the central nervous system.
  • Atypical Teratoid/Rhabdoid tumors (AT/RT) of the central nervous system are rare but aggressive tumors of childhood.
  • Diagnosis was confirmed using molecular markers.
  • There are two long-term survivors (78 and 98 months from diagnosis).

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  • (PMID = 19936623.001).
  • [ISSN] 1573-7373
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / R01 CA046274-18; United States / NCI NIH HHS / CA / R01 CA046274; United States / NCI NIH HHS / CA / CA46274; United States / NCI NIH HHS / CA / T32 CA108462; United States / NCI NIH HHS / CA / CA046274-18; United States / NCI NIH HHS / CA / T32 CA108462-01
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS184133; NLM/ PMC2880232
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3. Samaras V, Stamatelli A, Samaras E, Stergiou I, Konstantopoulou P, Varsos V, Judkins AR, Biegel JA, Barbatis C: Atypical teratoid/rhabdoid tumor of the central nervous system in an 18-year-old patient. Clin Neuropathol; 2009 Jan-Feb;28(1):1-10
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Atypical teratoid/rhabdoid tumor of the central nervous system in an 18-year-old patient.
  • OBJECTIVE: Atypical teratoid/rhabdoid tumors are aggressive neoplasms of the central nervous system occurring mainly in the early childhood and rarely in adults.
  • We described a case of this tumor in an 18-year-old male patient without previous medical history.
  • MATERIAL AND METHODS: The neoplasm was localized in the right frontotemporal area of the brain and was totally excised.
  • The histological and immunohistochemical features of the neoplasm were assessed, while sequencing analysis as well as interphase fluorescence in situ hybridization (FISH) were performed.
  • RESULTS: Histological and immunohistochemical analysis demonstrated atypical rhabdoid cells strongly and diffusely positive for EMA and Vimentin as well as focally immunoreactive for SMA and GFAP.
  • INI1 immunostaining demonstrated diffuse loss of nuclear INI1 expression in tumor cells.
  • Taken together, the results were consistent with a diagnosis of atypical teratoid/rhabdoid tumor (ATRT).
  • To our knowledge, this is the eighth case of an ATRT reported in an adult patient having genetic confirmation and the first one in which the tumor is, partly, localized in the right temporal area of the brain.
  • This unusual presentation underlines the necessity of considering this devastating neoplasm in the differential diagnosis of malignant brain tumors of young adults.

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  • (PMID = 19216214.001).
  • [ISSN] 0722-5091
  • [Journal-full-title] Clinical neuropathology
  • [ISO-abbreviation] Clin. Neuropathol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA046274-17A2; United States / NCI NIH HHS / CA / R01 CA046274; United States / NCI NIH HHS / CA / CA 46274; United States / NCI NIH HHS / CA / R01 CA046274-17A2
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / SMARCB1 protein, human; 0 / Transcription Factors
  • [Other-IDs] NLM/ NIHMS113796; NLM/ PMC2712356
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4. Selcuk N, Elevli M, Inanc D, Arslan H: Atypical teratoid/rhabdoid tumor mimicking tuberculous meningitis. Indian Pediatr; 2008 Apr;45(4):325-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Atypical teratoid/rhabdoid tumor mimicking tuberculous meningitis.
  • Atypical teratoid/rhabdoid tumor of the central nervous system is a highly malignant neoplasm in infants and young children.
  • We report a 6 year-old girl with atypical teratoid/rhabdoid tumor.
  • Pathologic result revealed high grade atypical teratoid/rhabdoid tumor.
  • [MeSH-major] Rhabdoid Tumor / diagnosis. Teratoma / diagnosis. Tuberculosis, Meningeal / diagnosis
  • [MeSH-minor] Cerebrospinal Fluid. Child. Diagnosis, Differential. Female. Humans. Magnetic Resonance Imaging

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  • (PMID = 18451456.001).
  • [ISSN] 0019-6061
  • [Journal-full-title] Indian pediatrics
  • [ISO-abbreviation] Indian Pediatr
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] India
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5. Athale UH, Duckworth J, Odame I, Barr R: Childhood atypical teratoid rhabdoid tumor of the central nervous system: a meta-analysis of observational studies. J Pediatr Hematol Oncol; 2009 Sep;31(9):651-63
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Childhood atypical teratoid rhabdoid tumor of the central nervous system: a meta-analysis of observational studies.
  • PURPOSE: Therapy for central nervous system (CNS) atypical teratoid rhabdoid tumor (ATRT) is controversial.
  • RESULTS: The median OS for patients treated with multiagent chemotherapy (n=79) was 17.3 months (range, 1.5-93 mo); unrelated to age at diagnosis, sex, tumor site, and extent of resection.
  • Patients (n=30) treated with intrathecal (IT) chemotherapy had significantly higher 2-year OS [64% (95% confidence interval, 46.5-82.0) vs. 17.3% (95% confidence interval, 5.4-29.3); P<0.0001] and lower prevalence of distant CNS metastasis compared with those without IT therapy (n=49) (20% vs. 59.2%; P=0.001).
  • CONCLUSIONS: Despite dismal OS, multimodal therapy can induce remission even in metastatic CNS ATRT with partial resection.
  • [MeSH-major] Brain Neoplasms / epidemiology. Rhabdoid Tumor / epidemiology. Teratoma / epidemiology
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Craniotomy. Cyclophosphamide / administration & dosage. Dactinomycin / administration & dosage. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Fatal Outcome. Female. Humans. Infant. Infant, Newborn. Injections, Spinal. Kaplan-Meier Estimate. Male. Prognosis. Prospective Studies. Spinal Neoplasms / diagnosis. Spinal Neoplasms / drug therapy. Spinal Neoplasms / epidemiology. Spinal Neoplasms / radiotherapy. Spinal Neoplasms / surgery. Treatment Outcome. Vincristine / administration & dosage

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  • (PMID = 19707161.001).
  • [ISSN] 1536-3678
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Meta-Analysis
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin
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6. de León-Bojorge B, Rueda-Franco F, Anaya-Jara M: Central nervous system atypical teratoid rhabdoid tumor: experience at the National Institute of Pediatrics, Mexico City. Childs Nerv Syst; 2008 Mar;24(3):307-12
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  • [Title] Central nervous system atypical teratoid rhabdoid tumor: experience at the National Institute of Pediatrics, Mexico City.
  • OBJECTIVE: The purpose of this study is to present our experience with ten cases of Central nervous system atypical teratoid rhabdoid tumor (CNS/ATRT).
  • PATIENTS AND METHODS: A series of ten patients with CNS/ATRT, were diagnosed and treated between 1990 and 2005, at the National Institute of Pediatrics, in Mexico City.
  • The gender, age of presentation, clinical features, tumor localization, imaging studies, grade of tumor resection, complications, adjuvant therapy, and survival are presented.
  • RESULTS: The mean age at diagnosis was 37.8 months, seven cases were male, and their average clinical course was 1.3 months.
  • CONCLUSIONS: Preliminary results, show that in older children, we can improve their survival with the subtotal or total resection of the tumor and the addition of chemotherapy and radiotherapy.
  • [MeSH-major] Central Nervous System Neoplasms / pathology. Infratentorial Neoplasms / pathology. Rhabdoid Tumor / pathology. Supratentorial Neoplasms / pathology. Teratoma / pathology

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  • (PMID = 17876589.001).
  • [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] Journal Article
  • [Publication-country] Germany
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7. Umredkar A, Bal A, Vashista RK: Atypical teratoid/rhabdoid tumour of the central nervous system in adult: case report. Br J Neurosurg; 2010 Dec;24(6):699-704

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Atypical teratoid/rhabdoid tumour of the central nervous system in adult: case report.
  • Atypical teratoid/rhabdoid tumours (AT/RT) are aggressive neoplasms of the central nervous system occurring mainly in the paediatric population.
  • The neoplasm was localised in the left frontal region and was totally excised.
  • This unusual presentation underlines the necessity of considering this devastating neoplasm in the differential diagnosis of malignant brain tumours of adults.
  • [MeSH-major] Central Nervous System Neoplasms / pathology. Rhabdoid Tumor / pathology. Teratoma / pathology

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  • (PMID = 21070155.001).
  • [ISSN] 1360-046X
  • [Journal-full-title] British journal of neurosurgery
  • [ISO-abbreviation] Br J Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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8. Rahmat K, Kua CH, Ramli N: A child with atypical teratoid/rhabdoid tumour of the posterior cranial fossa. Singapore Med J; 2008 Dec;49(12):e365-8

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A child with atypical teratoid/rhabdoid tumour of the posterior cranial fossa.
  • Primary central nervous system (CNS) atypical teratoid/rhabdoid tumours (ATRT) are highly malignant neoplasms which usually present in infancy or early childhood.
  • Although ATRT may arise anywhere within the CNS, the majority (approximately two-thirds) arise in the cerebellum or posterior fossa, and the remainder in the cerebrum.
  • We described the imaging characteristics of CNS ATRT in the posterior cranial fossa of a 14-month-old boy.
  • [MeSH-major] Cranial Fossa, Posterior. Rhabdoid Tumor / diagnosis. Skull Base Neoplasms / diagnosis. Teratoma / diagnosis

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  • (PMID = 19122937.001).
  • [ISSN] 0037-5675
  • [Journal-full-title] Singapore medical journal
  • [ISO-abbreviation] Singapore Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Singapore
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9. Cheng YC, Lirng JF, Chang FC, Guo WY, Teng MM, Chang CY, Wong TT, Ho DM: Neuroradiological findings in atypical teratoid/rhabdoid tumor of the central nervous system. Acta Radiol; 2005 Feb;46(1):89-96
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Neuroradiological findings in atypical teratoid/rhabdoid tumor of the central nervous system.
  • PURPOSE: To evaluate the computed tomography (CT) and magnetic resonance imaging (MRI) findings of atypical teratoid tumor/rhabdoid tumor (AT/RT) of the central nervous system (CNS).
  • MATERIAL AND METHODS: Twenty cases of CNS AT/RT have been found over the past 23 years in our hospital; these involving 11 boys and 9 girls whose mean age at diagnosis was 5.5 years.
  • The diagnosis mainly depends on the pathologic findings.
  • However, AT/RT should still remain in the differential diagnosis of brain tumors in young children, especially those located in the cerebellar hemisphere and with eccentric cysts.
  • [MeSH-major] Central Nervous System Neoplasms / pathology. Central Nervous System Neoplasms / radiography. Rhabdoid Tumor / pathology. Rhabdoid Tumor / radiography. Teratoma / pathology. Teratoma / radiography
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Diagnosis, Differential. Female. Humans. Infant. Magnetic Resonance Imaging. Male. Prognosis. Retrospective Studies. Survival Rate. Tomography, X-Ray Computed

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  • (PMID = 15841745.001).
  • [ISSN] 0284-1851
  • [Journal-full-title] Acta radiologica (Stockholm, Sweden : 1987)
  • [ISO-abbreviation] Acta Radiol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Sweden
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10. Ertan Y, Sezak M, Turhan T, Kantar M, Erşahin Y, Mutluer S, Vergin C, Oniz H, Akalin T: Atypical teratoid/rhabdoid tumor of the central nervous system: clinicopathologic and immunohistochemical features of four cases. Childs Nerv Syst; 2009 Jun;25(6):707-11
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Atypical teratoid/rhabdoid tumor of the central nervous system: clinicopathologic and immunohistochemical features of four cases.
  • BACKGROUND: Atypical teratoid/rhabdoid tumor (AT/RT) is a rare aggressive infantile neoplasm of uncertain origin.
  • Histopathologically, the tumors were composed of rhabdoid cells and undifferentiated small cells mixed with epithelial or mesenchymal components.
  • All of the patients died within a mean of 14 months due to tumor progression despite the chemotherapy.
  • Only one of our patients lived for 40 months after the diagnosis.
  • Morphologically, a large spectrum can be seen, like predominantly sarcoma in appearance, but immunohistochemistry is helpful in the correct diagnosis.
  • [MeSH-major] Brain Neoplasms / pathology. Rhabdoid Tumor / pathology. Teratoma / pathology
  • [MeSH-minor] Actins / analysis. Brain / pathology. Brain Chemistry. Child. Child, Preschool. Chromosomal Proteins, Non-Histone / analysis. DNA-Binding Proteins / analysis. Desmin / analysis. Diagnosis, Differential. Female. Glial Fibrillary Acidic Protein / analysis. Humans. Immunohistochemistry. Infant. Keratins / analysis. Male. Mucin-1 / analysis. S100 Proteins / analysis. SMARCB1 Protein. Synaptophysin / analysis. Transcription Factors / analysis. Vimentin / analysis

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  • (PMID = 19212771.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Actins; 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / Desmin; 0 / Glial Fibrillary Acidic Protein; 0 / Mucin-1; 0 / S100 Proteins; 0 / SMARCB1 Protein; 0 / SMARCB1 protein, human; 0 / Synaptophysin; 0 / Transcription Factors; 0 / Vimentin; 68238-35-7 / Keratins
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11. Zimmerman MA, Goumnerova LC, Proctor M, Scott RM, Marcus K, Pomeroy SL, Turner CD, Chi SN, Chordas C, Kieran MW: Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor. J Neurooncol; 2005 Mar;72(1):77-84
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  • [Title] Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor.
  • Atypical teratoid/rhabdoid tumors (AT/RT) are highly malignant lesions of childhood that carry a very poor prognosis.
  • AT/RT can occur in the central nervous system (CNS AT/RT) and disease in this location carries an even worse prognosis with a median survival of 7 months.
  • In spite of multiple treatment regimens consisting of maximal surgical resection (including second look surgery), radiation therapy (focal and craniospinal), and multi-agent intravenous, oral and intrathecal chemotherapy, with or without high-dose therapy and stem cell rescue, only seven long-term survivors of CNS AT/RT have been reported, all in patients with newly diagnosed disease.
  • We now report on four children, two with newly diagnosed CNS AT/RT and two with progressive disease after multi-agent chemotherapy who are long term survivors (median follow-up of 37 months) using a combination of surgery, radiation therapy, and intensive chemotherapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / therapy. Neoplasm Recurrence, Local / therapy. Rhabdoid Tumor / therapy. Teratoma / therapy

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  • (PMID = 15803379.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 37
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12. Singh A, Jairajpuri Z, Gupta V, Sharma S, Chand K: Atypical teratoid/rhabdoid tumor of the central nervous system associated with congenital cataract. Indian J Pathol Microbiol; 2008 Jul-Sep;51(3):389-91
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  • [Title] Atypical teratoid/rhabdoid tumor of the central nervous system associated with congenital cataract.
  • Atypical teratoid /rhabdoid tumor (AT/RT) of the central nervous system is a rare but highly aggressive neoplasm that usually affects young children and infants and follows a rapidly fatal course.
  • [MeSH-major] Central Nervous System Neoplasms / diagnosis. Central Nervous System Neoplasms / pathology. Rhabdoid Tumor / diagnosis. Rhabdoid Tumor / pathology. Teratoma / diagnosis. Teratoma / pathology
  • [MeSH-minor] Cataract / complications. Cataract / congenital. Central Nervous System / pathology. Fatal Outcome. Humans. Infant. Male

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  • (PMID = 18723966.001).
  • [ISSN] 0377-4929
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
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13. Wang Z, Fan QH, Yu MN, Zhang WM: [Clinicopathologic and immunohistochemical study of atypical teratoid/rhabdoid tumor of central nervous system]. Zhonghua Bing Li Xue Za Zhi; 2006 Aug;35(8):458-61
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  • [Title] [Clinicopathologic and immunohistochemical study of atypical teratoid/rhabdoid tumor of central nervous system].
  • OBJECTIVE: To study the clinicopathologic features and differential diagnosis of atypical teratoid/rhabdoid tumor (AT/RT) occurring in the central nervous system.
  • RESULTS: Histologically, AT/RT was characterized by the presence of rhabdoid cells associated with various degrees of primitive neuroectodermal, epithelial or mesenchymal differentiation.
  • The tumor cells were positive for vimentin, CD99, epithelial membrane antigen, cytokeratin, glial fibrillary acidic protein, S-100 protein, neurofilament, desmin and smooth muscle actin.
  • CONCLUSIONS: AT/RT is a highly malignant tumor occurring in the central nervous system.
  • The tumor is characterized by a heterogeneous histologic and immunohistochemical phenotype.
  • It needs to be distinguished from a number of central nervous system tumors, including medulloblastoma, primitive neuroectodermal tumor, germ cell neoplasm and rhabdoid meningioma.
  • [MeSH-major] Brain Neoplasms / pathology. Rhabdoid Tumor / pathology. Teratoma / pathology

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  • (PMID = 17069697.001).
  • [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] Case Reports; English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Actins; 0 / Antigens, CD; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / Desmin; 0 / Glial Fibrillary Acidic Protein; 0 / Mucin-1; 0 / Neurofilament Proteins; 0 / S100 Proteins; 0 / Vimentin; 68238-35-7 / Keratins
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14. Gidwani P, Levy A, Goodrich J, Weidenheim K, Kolb EA: Successful outcome with tandem myeloablative chemotherapy and autologous peripheral blood stem cell transplants in a patient with atypical teratoid/rhabdoid tumor of the central nervous system. J Neurooncol; 2008 Jun;88(2):211-5
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  • [Title] Successful outcome with tandem myeloablative chemotherapy and autologous peripheral blood stem cell transplants in a patient with atypical teratoid/rhabdoid tumor of the central nervous system.
  • Atypical teratoid rhabdoid tumors (ATRT) are highly malignant tumors of the central nervous system with a peak incidence in children less than 3 years of age.
  • We herein report the case of a 4-month-old boy with ATRT with partial resection of his tumor who achieved complete remission using tandem high-dose therapy followed by autologous peripheral blood stem cell re-infusions despite having biopsy proven disease at the time of starting the tandem regimens.
  • [MeSH-major] Central Nervous System Neoplasms / therapy. Myeloablative Agonists / therapeutic use. Peripheral Blood Stem Cell Transplantation / methods. Rhabdoid Tumor / therapy

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  • (PMID = 18317689.001).
  • [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 / Myeloablative Agonists
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15. Chen YW, Wong TT, Ho DM, Huang PI, Chang KP, Shiau CY, Yen SH: Impact of radiotherapy for pediatric CNS atypical teratoid/rhabdoid tumor (single institute experience). Int J Radiat Oncol Biol Phys; 2006 Mar 15;64(4):1038-43
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  • [Title] Impact of radiotherapy for pediatric CNS atypical teratoid/rhabdoid tumor (single institute experience).
  • PURPOSE: To assess outcomes and prognostic factors in radiotherapy of pediatric central nervous system atypical teratoid/rhabdoid tumor (AT/RT).
  • METHODS AND MATERIALS: Seventeen patients with central nervous system AT/RT were retrospectively reviewed after curative radiotherapy as primary or adjuvant therapy between January 1990 and December 2003.
  • The 3 longest-surviving patients were older, underwent gross tumor removal, and completed both craniospinal and focal boost irradiation.
  • [MeSH-major] Brain Neoplasms / radiotherapy. Rhabdoid Tumor / radiotherapy. Teratoma / radiotherapy

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  • [CommentIn] Int J Radiat Oncol Biol Phys. 2006 Jul 15;65(4):1273; author reply 1273-4 [16798419.001]
  • (PMID = 16406394.001).
  • [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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16. Bing F, Nugues F, Grand S, Bessou P, Salon C: Primary intracranial extra-axial and supratentorial atypical rhabdoid tumor. Pediatr Neurol; 2009 Dec;41(6):453-6
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  • [Title] Primary intracranial extra-axial and supratentorial atypical rhabdoid tumor.
  • An atypical teratoid/rhabdoid tumor of the central nervous system is an aggressive infantile embryonal neoplasm, usually presenting as an infratentorial and intraparenchymatous lesion.
  • We report on magnetic resonance imaging findings of a 22-month-old boy with a biopsy-proven primary rhabdoid tumor, presenting as a single intracranial supratentorial extra-axial mass.
  • Based on the patient's age and imaging features (perfusion, diffusion magnetic resonance imaging, and magnetic resonance spectroscopy), a diagnosis of atypical teratoid/rhabdoid tumor was more accurate than diagnoses of meningioma and primitive neuroectodermal tumor.
  • Although this entity is relatively rare, it should be considered in the differential diagnosis of dural-based, space-occupying central nervous system lesions.
  • [MeSH-major] Brain Neoplasms / diagnosis. Brain Neoplasms / pathology. Rhabdoid Tumor / diagnosis. Rhabdoid Tumor / pathology. Supratentorial Neoplasms / diagnosis. Supratentorial Neoplasms / pathology
  • [MeSH-minor] Brain / metabolism. Brain / pathology. Brain / surgery. Brain Edema / pathology. Diagnosis, Differential. Diffusion Magnetic Resonance Imaging. Humans. Infant. Magnetic Resonance Imaging. Magnetic Resonance Spectroscopy. Male. Neoplasm Recurrence, Local. Treatment Outcome

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  • (PMID = 19931170.001).
  • [ISSN] 1873-5150
  • [Journal-full-title] Pediatric neurology
  • [ISO-abbreviation] Pediatr. Neurol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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17. Biegel JA: Molecular genetics of atypical teratoid/rhabdoid tumor. Neurosurg Focus; 2006;20(1):E11
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  • [Title] Molecular genetics of atypical teratoid/rhabdoid tumor.
  • Rhabdoid tumors are extremely aggressive malignancies that generally occur in infants and young children.
  • The most common locations are the kidney and central nervous system (atypical teratoid/rhabdoid tumor [RT]), although RTs can also arise in most soft-tissue sites.
  • Rhabdoid tumors in all anatomical locations have a similar molecular origin.
  • [MeSH-major] Central Nervous System Neoplasms / genetics. Chromosomes, Human, Pair 22. Kidney Neoplasms / genetics. Molecular Biology / methods. Rhabdoid Tumor / genetics

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  • (PMID = 16459991.001).
  • [ISSN] 1092-0684
  • [Journal-full-title] Neurosurgical focus
  • [ISO-abbreviation] Neurosurg Focus
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA46274
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / SMARCB1 protein, human; 0 / Transcription Factors
  • [Number-of-references] 44
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18. Kazan S, Göksu E, Mihci E, Gökhan G, Keser I, Gürer I: Primary atypical teratoid/rhabdoid tumor of the clival region. Case report. J Neurosurg; 2007 Apr;106(4 Suppl):308-11
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  • [Title] Primary atypical teratoid/rhabdoid tumor of the clival region. Case report.
  • An atypical teratoid/rhabdoid tumor of the central nervous system (CNS) is a rare, aggressive neoplasm found in infants and children that has similar characteristics to CNS primitive neuroectodermal tumors/medulloblastomas.
  • The authors present the case of a patient with an atypical teratoid/rhabdoid tumor and discuss the imaging, histopathological, immunohistochemical, and cytogenetic findings.
  • Tumor cells displayed positive reactions for vimentin, epithelial membrane antigen, and cytokeratin, and they displayed no reaction for glial fibrillary acidic protein, desmin, and actin.
  • The phenotype of an atypical teratoid/rhabdoid tumor appears heterogeneous when examined by histological, immunohistochemical, and genetic analysis.
  • The authors describe the case of a 4-year-old boy who harbored an atypical teratoid/rhabdoid tumor in the clivus, which appeared as a chordoma on neuroimages.
  • To their knowledge, this location of an atypical teratoid/rhabdoid tumor has not been described in the literature.
  • [MeSH-major] Cranial Fossa, Posterior. Rhabdoid Tumor / pathology. Skull Base Neoplasms / pathology. Teratoma / pathology

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  • (PMID = 17465367.001).
  • [ISSN] 0022-3085
  • [Journal-full-title] Journal of neurosurgery
  • [ISO-abbreviation] J. Neurosurg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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19. Jackson EM, Shaikh TH, Zhang F, Wainwright LM, Storm PB, Hakonarson H, Zackai EH, Biegel JA: Atypical teratoid/rhabdoid tumor in a patient with Beckwith-Wiedemann syndrome. Am J Med Genet A; 2007 Aug 1;143A(15):1767-70
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Atypical teratoid/rhabdoid tumor in a patient with Beckwith-Wiedemann syndrome.
  • Here we describe a patient with BWS who developed a central nervous system atypical teratoid/rhabdoid tumor (AT/RT).
  • Due to the high propensity of these patients to develop childhood tumors, in addition to routine diagnostic tests, analysis of the tumor DNA using the Illumina Infinium whole-genome genotyping 550K Beadchip was performed to investigate a possible common underlying mechanism for his BWS and AT/RT.
  • The only alteration detected was monosomy 22, which was accompanied by a somatic mutation in the INI1 rhabdoid tumor gene.
  • [MeSH-major] Beckwith-Wiedemann Syndrome / genetics. Rhabdoid Tumor / complications. Teratoma / complications

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17603804.001).
  • [ISSN] 1552-4833
  • [Journal-full-title] American journal of medical genetics. Part A
  • [ISO-abbreviation] Am. J. Med. Genet. A
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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20. Sasani M, Oktenoglu T, Ozer AF, Sarioglu AC: Giant supratentorial atypical teratoid/rhabdoid tumor presentation: a case of a five-year-old child with favorable outcome and review of the literature. Pediatr Neurosurg; 2007;43(2):149-54
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  • [Title] Giant supratentorial atypical teratoid/rhabdoid tumor presentation: a case of a five-year-old child with favorable outcome and review of the literature.
  • Atypical teratoid/rhabdoid tumor of the central nervous system is a highly malignant neoplasm and that usually arises in the posterior fossa, survival from this is frequently poor.
  • We present a unique case in a 21-month-old girl who had an atypical teratoid/rhabdoid tumor with cystic components located in the right fronto-parietal lobe.
  • Two years later at the last follow-up visit, there was no evidence of a tumor relapse on MRI, and the examination was symptom free.
  • It is possible the favorable outcome of the patient resulted from a rapid diagnosis, prompt management, radical surgical intervention and aggressive chemotherapy.
  • [MeSH-major] Frontal Lobe / surgery. Parietal Lobe / surgery. Rhabdoid Tumor / surgery. Supratentorial Neoplasms / surgery. Teratoma / surgery
  • [MeSH-minor] Actins / analysis. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers, Tumor / analysis. Chemotherapy, Adjuvant. Combined Modality Therapy. Diagnosis, Differential. Female. Follow-Up Studies. Glial Fibrillary Acidic Protein / analysis. Humans. Infant. Keratins / analysis. Magnetic Resonance Imaging. Microsurgery. Mitotic Index. Necrosis. Neurologic Examination. Vimentin / analysis

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  • [Copyright] Copyright (c) 2007 S. Karger AG, Basel.
  • (PMID = 17337931.001).
  • [ISSN] 1016-2291
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Actins; 0 / Biomarkers, Tumor; 0 / Glial Fibrillary Acidic Protein; 0 / Vimentin; 68238-35-7 / Keratins
  • [Number-of-references] 14
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21. Judkins AR, Burger PC, Hamilton RL, Kleinschmidt-DeMasters B, Perry A, Pomeroy SL, Rosenblum MK, Yachnis AT, Zhou H, Rorke LB, Biegel JA: INI1 protein expression distinguishes atypical teratoid/rhabdoid tumor from choroid plexus carcinoma. J Neuropathol Exp Neurol; 2005 May;64(5):391-7
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  • [Title] INI1 protein expression distinguishes atypical teratoid/rhabdoid tumor from choroid plexus carcinoma.
  • Central nervous system atypical teratoid/rhabdoid tumor (AT/RT) and choroid plexus carcinoma (CPC) are rare, highly malignant tumors that predominantly arise in infants and young children.
  • Overlapping clinical, histologic, ultrastructural, or immunophenotypic features may obscure the diagnosis in some cases.
  • AT/RT is characterized by deletions and/or mutations of the INI1 tumor-suppressor gene on chromosome band 22q11.2.
  • Negative staining of tumor cells resulting from inactivation of the INI1 gene is a consistent feature of AT/RT.
  • We examined 28 tumors with a submitted diagnosis of CPC.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Carcinoma / metabolism. Choroid Plexus Neoplasms / metabolism. DNA-Binding Proteins / metabolism. Gene Expression Regulation, Neoplastic / physiology. Teratoma / metabolism

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  • (PMID = 15892296.001).
  • [ISSN] 0022-3069
  • [Journal-full-title] Journal of neuropathology and experimental neurology
  • [ISO-abbreviation] J. Neuropathol. Exp. Neurol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA46274; United States / NCI NIH HHS / CA / CA98543
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / Mucin-1; 0 / SMARCB1 protein, human; 0 / Transcription Factors
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22. Lee IH, Yoo SY, Kim JH, Eo H, Kim OH, Kim IO, Cheon JE, Jung AY, Yoon BJ: Atypical teratoid/rhabdoid tumors of the central nervous system: imaging and clinical findings in 16 children. Clin Radiol; 2009 Mar;64(3):256-64
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  • [Title] Atypical teratoid/rhabdoid tumors of the central nervous system: imaging and clinical findings in 16 children.
  • AIM: To investigate the imaging and clinical findings of central nervous system (CNS) atypical teratoid/rhabdoid tumours (AT/RTs) in children.
  • MATERIALS AND METHODS: The computed tomography (CT) and magnetic resonance imaging (MRI) findings and clinical records of 16 children with CNS AT/RTs were retrospectively reviewed.
  • Tumour location, size, composition, enhancement pattern, peritumoural oedema, signal intensity (SI) on MRI and CT attenuation were evaluated.
  • Tumour location was infratentorial for 11 lesions and supratentorial for six lesions.
  • The mean diameter of the largest dimension for a tumour was 4 cm.
  • The tumour was mainly solid in 65% of cases, and solid and cystic or cystic and solid in 35% of cases.
  • In spite of a large tumour size, peritumoural oedema was minimal or mild except in four cases.
  • Rapid growth of the tumour was demonstrated in three cases.
  • Seven patients died from tumour progression, with a mean survival time of 8.4 months (range 2-12 months).
  • Rapid growth of the tumour was seen during the follow-up period.
  • [MeSH-major] Brain Neoplasms / diagnosis. Rhabdoid Tumor / diagnosis

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  • (PMID = 19185655.001).
  • [ISSN] 1365-229X
  • [Journal-full-title] Clinical radiology
  • [ISO-abbreviation] Clin Radiol
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] England
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23. Wu X, Dagar V, Algar E, Muscat A, Bandopadhayay P, Ashley D, Wo Chow C: Rhabdoid tumour: a malignancy of early childhood with variable primary site, histology and clinical behaviour. Pathology; 2008 Dec;40(7):664-70
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  • [Title] Rhabdoid tumour: a malignancy of early childhood with variable primary site, histology and clinical behaviour.
  • AIMS: To correlate the immunostaining for INI1 protein and mutations in INI1 gene in possible rhabdoid tumours (RT) and atypical teratoid/rhabdoid tumours (AT/RT) seen at the Royal Children's Hospital in the last 10 years, and to study the clinicopathological features of those patients with negative nuclear staining.
  • In these 13 patients, the primary tumour was in the central nervous system (CNS) in seven, in the soft tissue in three, in the liver in two and in the kidney in one.
  • Only five tumours showed large areas of rhabdoid cells.
  • In two an alternative diagnosis, ependymoma or myoepithelial carcinoma of soft tissue, was initially suggested.
  • All the CNS tumours were positive for EMA, GFAP, and SMA.
  • As relatively few tumours showed uniform populations of rhabdoid cells, and some showed features suggesting another diagnosis, INI1 staining should be checked in all high grade CNS tumours and malignant extraCNS tumours where the diagnosis is unclear.
  • [MeSH-major] Biomarkers, Tumor / genetics. Chromosomal Proteins, Non-Histone / genetics. DNA-Binding Proteins / genetics. Rhabdoid Tumor / genetics. Rhabdoid Tumor / pathology. Transcription Factors / genetics

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  • (PMID = 18985520.001).
  • [ISSN] 0031-3025
  • [Journal-full-title] Pathology
  • [ISO-abbreviation] Pathology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [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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24. Feiden S, Sartorius E, Feiden W: [The role of diagnostic neuropathology in familial tumour syndromes]. Pathologe; 2010 Oct;31(6):464-70

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [The role of diagnostic neuropathology in familial tumour syndromes].
  • Inherited cancer syndromes often involve the central and peripheral nervous system.
  • For the surgical neuropathologist the possibility in individual patients of a familial tumour syndrome needs to be considered in the case of special tumours such as malignant peripheral nerve sheath tumour (MPNST), medulloblastoma with extensive nodularity (MBEN) or even atypical teratoid/rhabdoid tumour (AT/RT) of the brain.
  • Furthermore, tumour location and patient age may point to a familial tumour syndrome as in the case of neurofibromatosis type 2 (NF2) with typical bilateral vestibular schwannoma in young age.
  • [MeSH-minor] Central Nervous System Neoplasms / genetics. Central Nervous System Neoplasms / pathology. Chromosome Mapping. Chromosomes, Human, Pair 16. Chromosomes, Human, Pair 17. Chromosomes, Human, Pair 22. Chromosomes, Human, Pair 9. Genes, Neurofibromatosis 1. Genes, Neurofibromatosis 2. Humans. Li-Fraumeni Syndrome / genetics. Li-Fraumeni Syndrome / pathology. Neurofibromatosis 1 / genetics. Neurofibromatosis 1 / pathology. Neurofibromatosis 2 / genetics. Neurofibromatosis 2 / pathology. Neuroma, Acoustic / genetics. Neuroma, Acoustic / pathology. Peripheral Nervous System Neoplasms / genetics. Peripheral Nervous System Neoplasms / pathology. Rhabdoid Tumor / genetics. Rhabdoid Tumor / pathology. Teratoma / genetics. Teratoma / pathology. Tuberous Sclerosis / genetics. Tuberous Sclerosis / pathology

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  • [Cites] Genet Med. 2010 Jan;12(1):1-11 [20027112.001]
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  • (PMID = 20848106.001).
  • [ISSN] 1432-1963
  • [Journal-full-title] Der Pathologe
  • [ISO-abbreviation] Pathologe
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] Germany
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25. Rezanko T, Tunakan M, Kahraman A, Sucu HK, Gelal F, Akkol I: Primary rhabdoid tumor of the brain in an adult. Neuropathology; 2006 Feb;26(1):57-61
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  • [Title] Primary rhabdoid tumor of the brain in an adult.
  • Rhabdoid tumor (RT) is an uncommon childhood neoplasm that typically arises within the kidney.
  • Since its description in 1978, several cases of primary extrarenal RT, including a CNS localization, have been reported.
  • The first case in the CNS was reported in 1985 and was defined as "rhabdoid tumor" initially, and was classified as grade IV in the most recent classification of the World Health Organization under the term of "atypical teratoid/rhabdoid tumor".
  • Nearly 200 cases of atypical teratoid/rhabdoid tumor of the CNS have been reported to date, most of them occurring in childhood.
  • This tumor, which was composed purely of rhabdoid cells with no additional primitive neuroectodermal, epithelial and mesenchymal components, was in a 27-year-old male patient.
  • In conclusion, RT should be considered also in the differential diagnosis of intracerebral neoplasms of adult patients.
  • [MeSH-major] Brain Neoplasms / pathology. Rhabdoid Tumor / pathology
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Diagnosis, Differential. Humans. Immunohistochemistry. Male. Meningioma / pathology

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  • (PMID = 16521480.001).
  • [ISSN] 0919-6544
  • [Journal-full-title] Neuropathology : official journal of the Japanese Society of Neuropathology
  • [ISO-abbreviation] Neuropathology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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26. Chi SN, Zimmerman MA, Yao X, Cohen KJ, Burger P, Biegel JA, Rorke-Adams LB, Fisher MJ, Janss A, Mazewski C, Goldman S, Manley PE, Bowers DC, Bendel A, Rubin J, Turner CD, Marcus KJ, Goumnerova L, Ullrich NJ, Kieran MW: Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol; 2009 Jan 20;27(3):385-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor.
  • PURPOSE: Atypical teratoid rhabdoid tumor (ATRT) of the CNS is a highly malignant neoplasm primarily affecting young children, with a historic median survival ranging from 6 to 11 months.
  • Based on a previous pilot series, a prospective multi-institutional trial was conducted for patients with newly diagnosed CNS ATRT.
  • Radiation therapy (RT) was prescribed, either focal (54 Gy) or craniospinal (36 Gy, plus primary boost), depending on age and extent of disease at diagnosis.
  • Median age at diagnosis was 26 months (range, 2.4 months to 19.5 years).
  • Gross total resection of the primary tumor was achieved in 11 patients.
  • Fourteen patients had M0 disease at diagnosis, one patient had M2 disease, and five patients had M3 disease.
  • CONCLUSION: This intensive multimodality regimen has resulted in a significant improvement in time to progression and overall survival for patients with this previously poor-prognosis tumor.
  • [MeSH-major] Brain Neoplasms / therapy. Rhabdoid Tumor / therapy. Teratoma / therapy

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  • (PMID = 19064966.001).
  • [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 / R01 CA046274; United States / NCI NIH HHS / CA / R01 CA046274-17A2; United States / NCI NIH HHS / CA / CA46274
  • [Publication-type] Journal Article; Multicenter Study; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC2645855
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27. Arcaro A, Doepfner KT, Boller D, Guerreiro AS, Shalaby T, Jackson SP, Schoenwaelder SM, Delattre O, Grotzer MA, Fischer B: Novel role for insulin as an autocrine growth factor for malignant brain tumour cells. Biochem J; 2007 Aug 15;406(1):57-66
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  • [Title] Novel role for insulin as an autocrine growth factor for malignant brain tumour cells.
  • AT/RTs (atypical teratoid/rhabdoid tumours) of the CNS (central nervous system) are childhood malignancies associated with poor survival rates due to resistance to conventional treatments such as chemotherapy.
  • We characterized a panel of human AT/RT and MRT (malignant rhabdoid tumour) cell lines for expression of RTKs (receptor tyrosine kinases) and their involvement in tumour growth and survival.
  • Taken together, our results reveal a novel role for autocrine signalling by insulin and the IR in growth and survival of malignant human CNS tumour cells via the PI3K/Akt pathway.
  • [MeSH-minor] Cell Line, Tumor. Cell Proliferation / drug effects. Child, Preschool. Chromosomal Proteins, Non-Histone / metabolism. Culture Media, Serum-Free. DNA-Binding Proteins / metabolism. Down-Regulation / drug effects. Down-Regulation / genetics. Enzyme Activation / drug effects. Female. Humans. Infant. Isoenzymes / metabolism. Male. Phosphatidylinositol 3-Kinases / metabolism. Proto-Oncogene Proteins c-akt / metabolism. RNA, Small Interfering / metabolism. Receptor, IGF Type 1 / metabolism. Receptor, Insulin / genetics. Receptor, Insulin / metabolism. Signal Transduction / drug effects. Transcription Factors / metabolism

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  • (PMID = 17506723.001).
  • [ISSN] 1470-8728
  • [Journal-full-title] The Biochemical journal
  • [ISO-abbreviation] Biochem. J.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Chromosomal Proteins, Non-Histone; 0 / Culture Media, Serum-Free; 0 / DNA-Binding Proteins; 0 / Growth Substances; 0 / Insulin; 0 / Isoenzymes; 0 / RNA, Small Interfering; 0 / SMARCB1 protein, human; 0 / Transcription Factors; EC 2.7.1.- / Phosphatidylinositol 3-Kinases; EC 2.7.10.1 / Receptor, IGF Type 1; EC 2.7.10.1 / Receptor, Insulin; EC 2.7.11.1 / Proto-Oncogene Proteins c-akt
  • [Other-IDs] NLM/ PMC1948991
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28. Kao CL, Chiou SH, Ho DM, Chen YJ, Liu RS, Lo CW, Tsai FT, Lin CH, Ku HH, Yu SM, Wong TT: Elevation of plasma and cerebrospinal fluid osteopontin levels in patients with atypical teratoid/rhabdoid tumor. Am J Clin Pathol; 2005 Feb;123(2):297-304
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Elevation of plasma and cerebrospinal fluid osteopontin levels in patients with atypical teratoid/rhabdoid tumor.
  • Osteopontin, a cancer metastasis-associated gene, is specifically up-regulated in central nervous system (CNS) atypical teratoid/rhabdoid tumor (AT/RT), but its biological behavior in the progression of CNS AT/RT has never been studied.
  • The differences in osteopontin expression in plasma, CSF, and tumor samples in AT/RT and medulloblastoma correlated with survival differences.
  • [MeSH-major] Brain Neoplasms / metabolism. Rhabdoid Tumor / metabolism. Sialoglycoproteins. Teratoma / metabolism
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Enzyme-Linked Immunosorbent Assay. Humans. Infant. Infant, Newborn. Medulloblastoma / metabolism. Medulloblastoma / mortality. Medulloblastoma / pathology. Neoplasm Recurrence, Local. Osteopontin. Survival Rate

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  • (PMID = 15842057.001).
  • [ISSN] 0002-9173
  • [Journal-full-title] American journal of clinical pathology
  • [ISO-abbreviation] Am. J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / SPP1 protein, human; 0 / Sialoglycoproteins; 106441-73-0 / Osteopontin
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29. Pipp I, Wagner L, Rössler K, Budka H, Preusser M: Secretagogin expression in tumours of the human brain and its coverings. APMIS; 2007 Apr;115(4):319-26
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  • We found focal or widespread secretagogin expression in tumour cells in 1/18 oligoastrocytomas, 1/19 oligodendrogliomas, 2/20 anaplastic oligodendrogliomas, 2/9 ependymomas, 2/11 anaplastic ependymomas, 2/10 glioblastomas, 3/11 gangliogliomas and 1/2 anaplastic gangliogliomas, 10/10 central neurocytomas, 5/10 classic medulloblastomas, 4/5 desmoplastic medulloblastomas, 3/5 large cell/anaplastic medulloblastomas, 3/5 neuroblastomas, 3/10 meningiomas, 2/10 haemangioblastomas, and 13/19 pituitary adenomas.
  • We detected no secretagogin expression in fibrillary astrocytoma, pilocytic astrocytoma, DNT, pineocytoma, pineoblastoma, subependymal giant cell astrocytoma (SEGA), atypical teratoid/rhabdoid tumour (AT/RT), or primary central nervous system lymphoma (PCNSL).
  • Our findings indicate that secretagogin is involved in the calcium metabolism of tumour cells and endothelial cells in a subset of neoplasms of the brain and its coverings.
  • [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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30. de León-Bojorge B, Rueda-Franco F, Anaya-Jara M: Atypical teratoid/rhabdoid tumor of the central nervous system. Childs Nerv Syst; 2009 Nov;25(11):1387; author reply 1389
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Atypical teratoid/rhabdoid tumor of the central nervous system.
  • [MeSH-major] Central Nervous System Neoplasms / pathology. Rhabdoid Tumor / pathology. Teratoma / pathology

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  • [CommentOn] Childs Nerv Syst. 2009 Jun;25(6):707-11 [19212771.001]
  • [Cites] Childs Nerv Syst. 2008 Mar;24(3):307-12 [17876589.001]
  • [Cites] Childs Nerv Syst. 2009 Jun;25(6):707-11 [19212771.001]
  • (PMID = 19636570.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Comment; Letter
  • [Publication-country] Germany
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