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1
atypical teratoid rhabdoid tumor of central nervous system 2005:2010[pubdate] *count=100
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Items 1 to 87 of about 87
1.
Chiou SH, Kao CL, Chen YW, Chien CS, Hung SC, Lo JF, Chen YJ, Ku HH, Hsu MT, Wong TT:
Identification of CD133-positive radioresistant cells in atypical teratoid/rhabdoid tumor.
PLoS One
; 2008;3(5):e2090
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[Title]
Identification of CD133-positive radioresistant cells in
atypical teratoid
/
rhabdoid tumor
.
Atypical teratoid
/
rhabdoid tumor
(AT/RT) is an extremely
malignant neoplasm
in the
central nervous system
(
CNS
) which occurs in infancy and childhood.
[MeSH-major]
Antigens, CD / immunology. Glycoproteins / immunology. Peptides / immunology. Radiation Tolerance.
Rhabdoid Tumor
/
diagnosis
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(PMID = 18509505.001).
[ISSN]
1932-6203
[Journal-full-title]
PloS one
[ISO-abbreviation]
PLoS ONE
[Language]
eng
[Publication-type]
Journal Article; Research Support, Non-U.S. Gov't
[Publication-country]
United States
[Chemical-registry-number]
0 / AC133 antigen; 0 / Antigens, CD; 0 / Glycoproteins; 0 / Peptides; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / RNA, Small Interfering
[Other-IDs]
NLM/ PMC2396792
2.
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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.
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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
3.
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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[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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.
MedlinePlus Health Information.
consumer health - Brain Tumors
.
MedlinePlus Health Information.
consumer health - Childhood Brain Tumors
.
COS Scholar Universe.
author profiles
.
Hazardous Substances Data Bank.
CIS-DIAMINEDICHLOROPLATINUM
.
Hazardous Substances Data Bank.
DOXORUBICIN
.
Hazardous Substances Data Bank.
ETOPOSIDE
.
Hazardous Substances Data Bank.
CYCLOPHOSPHAMIDE
.
Hazardous Substances Data Bank.
DACTINOMYCIN
.
Hazardous Substances Data Bank.
VINCRISTINE
.
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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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4.
Agrawal A, Bhake A, Cincu R:
Giant lumbar paraspinal atypical teratoid/rhabdoid tumor in a child.
J Cancer Res Ther
; 2009 Oct-Dec;5(4):318-20
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[Title]
Giant lumbar paraspinal
atypical teratoid
/
rhabdoid tumor
in a child.
Atypical teratoid
/
rhabdoid tumor
(AT/RT) is a highly aggressive and uncommon
tumor of
the
central nervous system
, primarily affecting young children.
[MeSH-major]
Rhabdoid Tumor
/ pathology. Spinal Neoplasms / pathology. Teratoma / pathology
Genetic Alliance.
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.
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(PMID = 20160373.001).
[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
[Chemical-registry-number]
0 / Antineoplastic Agents
5.
Hasselblatt M, Oyen F, Gesk S, Kordes U, Wrede B, Bergmann M, Schmid H, Frühwald MC, Schneppenheim R, Siebert R, Paulus W:
Cribriform neuroepithelial tumor (CRINET): a nonrhabdoid ventricular tumor with INI1 loss and relatively favorable prognosis.
J Neuropathol Exp Neurol
; 2009 Dec;68(12):1249-55
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[Title]
Cribriform neuroepithelial
tumor
(CRINET): a nonrhabdoid ventricular
tumor
with INI1 loss and relatively favorable prognosis.
Atypical teratoid
/
rhabdoid
tumors are
malignant
embryonal tumors characterized by the presence
of rhabdoid
cells, genetic alterations affecting the SMARCB1 gene (hSNF5/INI1), and a poor prognosis.
Whether INI1 plays a role in the pathogenesis of other
central nervous system
tumors is uncertain.
Histological and immunohistochemical features did not correspond to established
tumor
types, including
atypical teratoid
/
rhabdoid
tumors, medulloepithelioma, choroid plexus carcinoma, and ependymoma.
We suggest that cribriform neuroepithelial
tumor
(CRINET) is a nonrhabdoid ventricular
tumor
that shows loss of tumoral INI1 protein and has a relatively favorable prognosis.
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(PMID = 19915490.001).
[ISSN]
1554-6578
[Journal-full-title]
Journal of neuropathology and experimental neurology
[ISO-abbreviation]
J. Neuropathol. Exp. Neurol.
[Language]
eng
[Publication-type]
Case Reports; 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, human; 0 / Transcription Factors
6.
D'cunja J, Shalaby T, Rivera P, von Büren A, Patti R, Heppner FL, Arcaro A, Rorke-Adams LB, Phillips PC, Grotzer MA:
Antisense treatment of IGF-IR induces apoptosis and enhances chemosensitivity in central nervous system atypical teratoid/rhabdoid tumours cells.
Eur J Cancer
; 2007 Jul;43(10):1581-9
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[Title]
Antisense treatment of IGF-IR induces apoptosis and enhances chemosensitivity in
central nervous system atypical teratoid
/
rhabdoid
tumours cells.
Central nervous system
(
CNS
)
atypical teratoid
/
rhabdoid
tumours (AT/RT) are among the paediatric
malignant
tumours with the worst prognosis and fatal outcome.
Moreover, we found IGF-I and IGF-II mRNA in BT-16
CNS AT
/RT cells and IGF-II mRNA in BT-12
CNS AT
/RT cells, and autophosphorylated IGF-IR in both cell lines, indicating the potential presence of an autocrine/paracrine IGF-I/II/IGF-IR loop in
CNS AT
/RT.
IGF-IR antisense oligonucleotide treatment of human
CNS AT
/RT cells resulted in significant down-regulation of IGF-IR mRNA and protein expression, induction of apoptosis, and chemosensitisation to doxorubicin and cisplatin.
These studies provide evidence for the influence of IGF-IR on cellular responses to chemotherapy and raise the possibility that curability of selected
CNS AT
/RT may be improved by pharmaceutical strategies directed towards the IGF-IR.
[MeSH-major]
Apoptosis / drug effects.
Central Nervous System
Neoplasms / drug therapy. Oligoribonucleotides, Antisense / therapeutic use. Receptor, IGF Type 1 / drug effects.
Rhabdoid Tumor
/ drug therapy. Teratoma / drug therapy
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.
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.
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(PMID = 17446062.001).
[ISSN]
0959-8049
[Journal-full-title]
European journal of cancer (Oxford, England : 1990)
[ISO-abbreviation]
Eur. J. Cancer
[Language]
eng
[Publication-type]
Journal Article; Research Support, Non-U.S. Gov't
[Publication-country]
England
[Chemical-registry-number]
0 / Antineoplastic Agents; 0 / Oligoribonucleotides, Antisense; 67763-96-6 / Insulin-Like Growth Factor I; 80168379AG / Doxorubicin; EC 2.7.10.1 / Receptor, IGF Type 1; Q20Q21Q62J / Cisplatin
7.
Huddleston BJ, Sjostrom CM, Collins BT:
Atypical teratoid/rhabdoid tumor involving cerebrospinal fluid: a case report.
Acta Cytol
; 2010 Sep-Oct;54(5 Suppl):958-62
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[Title]
Atypical teratoid
/
rhabdoid tumor
involving cerebrospinal fluid: a case report.
BACKGROUND:
Atypical teratoid
/
rhabdoid tumor
(AT/RT) is a rare, aggressive
tumor of
the
central nervous system
.
It is important for pathologists to recognize the cytomorphologic features of AT/RT in the CSF of patients with this
tumor
to help determine prognosis and disease progression.
[MeSH-major]
Rhabdoid Tumor
/ cerebrospinal fluid.
Rhabdoid Tumor
/ pathology. Spinal Neoplasms / cerebrospinal fluid. Spinal Neoplasms / pathology. Teratoma / cerebrospinal fluid. Teratoma / pathology
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(PMID = 21053577.001).
[ISSN]
0001-5547
[Journal-full-title]
Acta cytologica
[ISO-abbreviation]
Acta Cytol.
[Language]
eng
[Publication-type]
Case Reports; Journal Article
[Publication-country]
United States
8.
Niwa T, Aida N, Tanaka M, Okubo J, Sasano M, Shishikura A, Fujita K, Ito S, Tanaka Y, Kigasawa H:
Diffusion-weighted imaging of an atypical teratoid/rhabdoid tumor of the cervical spine.
Magn Reson Med Sci
; 2009;8(3):135-8
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[Title]
Diffusion-weighted imaging of an
atypical teratoid
/
rhabdoid tumor
of the cervical spine.
Spinal
atypical teratoid
/
rhabdoid tumor
(AT/RT) is a rare, aggressive
malignant neoplasm
of the
central nervous system
usually seen in young children and infants.
High signal on DWI and low apparent diffusion coefficients may represent high cellularity of the
tumor
.
These findings indicated a highly
malignant
tumor
.
[MeSH-major]
Cervical Vertebrae. Diffusion Magnetic Resonance Imaging / methods.
Rhabdoid Tumor
/ pathology. Spinal Cord Neoplasms / pathology. Teratoma / pathology
[MeSH-minor]
Child.
Diagnosis
, Differential. Humans. Immunohistochemistry. Male
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(PMID = 19783876.001).
[ISSN]
1880-2206
[Journal-full-title]
Magnetic resonance in medical sciences : MRMS : an official journal of Japan Society of Magnetic Resonance in Medicine
[ISO-abbreviation]
Magn Reson Med Sci
[Language]
eng
[Publication-type]
Case Reports; Journal Article
[Publication-country]
Japan
9.
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.
Seventeen of the 26 tumors showed morphologically characteristic features of AT/RTs, whereas 9 embryonal tumors did not display
rhabdoid
features.
Tumors without
rhabdoid
phenotype but lack of INI1 showed an aggressive clinical course and poor response to conventional treatment regimens.
Furthermore, a certain number of embryonal tumors without
rhabdoid
features but lack of INI1 protein and aggressive biologic behavior can be detected.
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
10.
Morgenstern DA, Gibson S, Brown T, Sebire NJ, Anderson J:
Clinical and pathological features of paediatric malignant rhabdoid tumours.
Pediatr Blood Cancer
; 2010 Jan;54(1):29-34
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[Title]
Clinical and pathological features of paediatric
malignant
rhabdoid
tumours.
BACKGROUND:
Malignant
rhabdoid
tumours (MRT) and their
central nervous system
(
CNS
) counterparts
atypical teratoid
/
rhabdoid
tumours (ATRT) are rare, highly aggressive
malignant
neoplasms of childhood.
[MeSH-major]
Rhabdoid Tumor
/ pathology. Teratoma / pathology
[MeSH-minor]
Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers,
Tumor
/ analysis. Child. Combined Modality Therapy. Female. Humans. Immunoenzyme Techniques. Infant. Male.
Neoplasm
Staging. Prognosis. Radiotherapy. Retrospective Studies. Survival Rate. Treatment Outcome
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[Copyright]
Copyright 2009 Wiley-Liss, Inc.
(PMID = 19653294.001).
[ISSN]
1545-5017
[Journal-full-title]
Pediatric blood & cancer
[ISO-abbreviation]
Pediatr Blood Cancer
[Language]
eng
[Publication-type]
Case Reports; Journal Article
[Publication-country]
United States
[Chemical-registry-number]
0 / Biomarkers, Tumor
11.
Santagata S, Hornick JL, Ligon KL:
Comparative analysis of germ cell transcription factors in CNS germinoma reveals diagnostic utility of NANOG.
Am J Surg Pathol
; 2006 Dec;30(12):1613-8
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[Title]
Comparative analysis of germ cell transcription factors in
CNS
germinoma reveals diagnostic utility of NANOG.
To assess the diagnostic utility of NANOG in
central nervous system
(
CNS
) germ cell tumors, we analyzed its expression by immunohistochemistry in a series of 12
CNS
germinomas and compared its expression with other stem cell markers.
Strong nuclear expression of NANOG was demonstrated in >90% of the
tumor
cells in all cases.
NANOG was not detected in
tumor
types frequently considered in the differential
diagnosis
of
CNS
germinoma: pineoblastoma, primitive neuroectodermal tumors, medulloblastoma, lymphoma, pituitary adenoma,
atypical teratoid
/
rhabdoid tumor
, Langerhans cell histiocytosis, and gliomas.
These findings demonstrate that NANOG is a sensitive and specific marker of
CNS
germinoma.
These findings also suggest a potential biologic role for NANOG in maintenance of
CNS
germinoma.
[MeSH-minor]
Adolescent. Adult. Alkaline Phosphatase / metabolism. Biomarkers,
Tumor
/ metabolism. Cell Nucleus / metabolism. Child. HMGB Proteins / metabolism. Humans. Isoenzymes / metabolism. Octamer Transcription Factor-3 / metabolism. SOXB1 Transcription Factors. Transcription Factors / metabolism
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(PMID = 17122519.001).
[ISSN]
0147-5185
[Journal-full-title]
The American journal of surgical pathology
[ISO-abbreviation]
Am. J. Surg. Pathol.
[Language]
eng
[Grant]
United States / NINDS NIH HHS / NS / NS047213
[Publication-type]
Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
[Publication-country]
United States
[Chemical-registry-number]
0 / Biomarkers, Tumor; 0 / DNA-Binding Proteins; 0 / HMGB Proteins; 0 / Homeodomain Proteins; 0 / Isoenzymes; 0 / NANOG protein, human; 0 / Octamer Transcription Factor-3; 0 / POU5F1 protein, human; 0 / SOX2 protein, human; 0 / SOXB1 Transcription Factors; 0 / Transcription Factors; 0 / germ-cell AP isoenzyme; EC 3.1.3.1 / Alkaline Phosphatase
12.
Antonelli M, Cenacchi G, Modena P, Morra I, Forni M, Giangaspero F:
Ultrastructural evidence of ependymal differentiation in a genetically proven atypical teratoid/rhabdoid tumor.
Childs Nerv Syst
; 2009 Dec;25(12):1627-31
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[Title]
Ultrastructural evidence of ependymal differentiation in a genetically proven
atypical teratoid
/
rhabdoid tumor
.
INTRODUCTION: We describe a case of genetically proven
atypical teratoid
/
rhabdoid tumor
(ATRT), showing ultrastructural evidence of ependymal differentiation.
CONCLUSION: This finding supports the concept that ATRTs as the majority
of central nervous system
embryonal tumors may derive from an immature and pluripotent neuroectodermal cell capable of differentiating along multiple lineages.
[MeSH-major]
Cerebral Ventricle Neoplasms / ultrastructure. Ependyma / ultrastructure.
Rhabdoid Tumor
/ ultrastructure. Teratoma / ultrastructure
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[Cites]
Cancer Res. 2005 May 15;65(10 ):4012-9
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15899790.001
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Mod Pathol. 2004 Jun;17(6):679-83
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Ultrastruct Pathol. 1997 Jul-Aug;21(4):369-78
[
9206002.001
]
(PMID = 19554334.001).
[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
13.
Gardner SL, Asgharzadeh S, Green A, Horn B, McCowage G, Finlay J:
Intensive induction chemotherapy followed by high dose chemotherapy with autologous hematopoietic progenitor cell rescue in young children newly diagnosed with central nervous system atypical teratoid rhabdoid tumors.
Pediatr Blood Cancer
; 2008 Aug;51(2):235-40
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[Title]
Intensive induction chemotherapy followed by high dose chemotherapy with autologous hematopoietic progenitor cell rescue in young children newly diagnosed with
central nervous system atypical teratoid rhabdoid
tumors.
BACKGROUND:
Central nervous system
(
CNS
)
atypical teratoid rhabdoid
tumors (AT/RT) are rare tumors of childhood with a dismal prognosis.
Historically, surgery and standard dose chemotherapy have resulted in a median survival of 8.5 months from
diagnosis
.
METHODS: Thirteen children newly diagnosed with
CNS AT
/RT were treated with either the "Head Start I" (HS I) or "Head Start II" (HS II) regimens.
There are presently three event-free survivors 42+, 54+, and 67+ months following
diagnosis
; none received RT.
CONCLUSION: Three of seven children with
CNS AT
/RT treated on HS II have experienced long term remissions.
Long term survival can be achieved in a subset of young children with
CNS AT
/RT following resection with the use of multi-drug chemotherapy including high dose methotrexate and myeloablative chemotherapy without radiation therapy (RT).
[MeSH-major]
Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / therapy. Hematopoietic Stem Cell Transplantation.
Rhabdoid Tumor
/ therapy. Teratoma / therapy
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(PMID = 18381756.001).
[ISSN]
1545-5017
[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
14.
Cheng JX, Tretiakova M, Gong C, Mandal S, Krausz T, Taxy JB:
Renal medullary carcinoma: rhabdoid features and the absence of INI1 expression as markers of aggressive behavior.
Mod Pathol
; 2008 Jun;21(6):647-52
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[Title]
Renal medullary carcinoma:
rhabdoid
features and the absence of INI1 expression as markers of aggressive behavior.
Renal medullary carcinoma is a rare, well-recognized highly aggressive
tumor of
varied histopathology, which occurs in young patients with sickle cell trait or disease.
Rhabdoid
elements, occasionally seen in high-grade renal tumors including renal medullary carcinoma, possibly represent a pathologic marker of aggressive behavior.
Loss of this factor in mice results in aggressive
rhabdoid
tumors or lymphomas.
In humans, the loss of INI1 expression has been reported in pediatric renal
rhabdoid
tumors,
central nervous system atypical teratoid
/
rhabdoid
tumors and epithelioid sarcomas, a possible primary soft tissue
rhabdoid tumor
.
This study compares five renal medullary carcinomas with 10 high-grade renal cell carcinomas (five with
rhabdoid
features), two urothelial carcinomas and two pediatric renal
rhabdoid
tumors.
All five renal medullary carcinomas, irrespective of histopathology, showed complete loss of INI1 expression similar to that seen in pediatric renal
rhabdoid
tumors.
In contrast, all renal cell carcinomas or urothelial carcinomas, including those with histological
rhabdoid
features, expressed INI1.
Clinically, all five of the patients with renal medullary carcinoma and the two patients with
rhabdoid
tumors presented with extra-renal metastases at the time of
diagnosis
.
This study demonstrates that renal medullary carcinoma and renal
rhabdoid tumor
share a common molecular/genetic alteration, which is closely linked to their aggressive biological behavior.
However, the absence of INI1 expression is not necessarily predictive
of rhabdoid
histopathology but remains associated with aggressive behavior in renal medullary carcinoma.
[MeSH-major]
Biomarkers,
Tumor
/ analysis. Carcinoma, Renal Cell / pathology. Chromosomal Proteins, Non-Histone / biosynthesis. DNA-Binding Proteins / biosynthesis. Kidney Neoplasms / pathology.
Rhabdoid Tumor
/ pathology. Transcription Factors / biosynthesis
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(PMID = 18327209.001).
[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]
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
15.
Patereli A, Alexiou GA, Stefanaki K, Moschovi M, Doussis-Anagnostopoulou I, Prodromou N, Karentzou O:
Expression of epidermal growth factor receptor and HER-2 in pediatric embryonal brain tumors.
Pediatr Neurosurg
; 2010;46(3):188-92
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BACKGROUND/AIMS: Medulloblastomas (MBs),
atypical teratoid rhabdoid
tumors (AT/RTs) and
central nervous system
primitive neuroectodermal tumors (PNETs) are aggressive embryonal brain neoplasms in children with overlapping histological features but with different pathogenetic pathways.
The immunohistochemical expression of EGFR and HER-2 was correlated to histology, expression of the Ki-67/MIB-1 proliferative index, p53
tumor
suppressor oncoprotein and prognosis.
[MeSH-minor]
Adolescent. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Infant. Infant, Newborn. Ki-67 Antigen / metabolism. Male. Prognosis. Retrospective Studies. Risk Factors.
Tumor
Suppressor Protein p53 / metabolism
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[Copyright]
Copyright © 2010 S. Karger AG, Basel.
(PMID = 20962551.001).
[ISSN]
1423-0305
[Journal-full-title]
Pediatric neurosurgery
[ISO-abbreviation]
Pediatr Neurosurg
[Language]
eng
[Publication-type]
Journal Article
[Publication-country]
Switzerland
[Chemical-registry-number]
0 / Ki-67 Antigen; 0 / TP53 protein, human; 0 / Tumor Suppressor Protein p53; EC 2.7.10.1 / EGFR protein, human; EC 2.7.10.1 / ERBB2 protein, human; EC 2.7.10.1 / Receptor, Epidermal Growth Factor; EC 2.7.10.1 / Receptor, ErbB-2
16.
Cecen E, Gunes D, Uysal KM, Yuceer N, Ozer E:
Atypical teratoid/rhabdoid tumor in an infant conceived by in vitro fertilization.
Childs Nerv Syst
; 2010 Feb;26(2):263-6
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[Title]
Atypical teratoid
/
rhabdoid tumor
in an infant conceived by in vitro fertilization.
BACKGROUND:
Atypical teratoid
/
rhabdoid tumor
(ATsRT) is a rare
tumor
and extremely aggressive embryonal
neoplasm
of the
central nervous system
.
[MeSH-major]
Brain Neoplasms / pathology. Diseases in Twins / pathology. Fertilization in Vitro.
Rhabdoid Tumor
/ pathology. Teratoma / pathology
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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
[Publication-country]
Germany
17.
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
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[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
18.
Tez S, Köktener A, Güler G, Ozişik P:
Atypical teratoid/rhabdoid tumors: imaging findings of two cases and review of the literature.
Turk Neurosurg
; 2008 Jan;18(1):30-4
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[Title]
Atypical teratoid
/
rhabdoid
tumors: imaging findings of two cases and review of the literature.
Atypical teratoid
/
rhabdoid tumor
(AT/RT) is a
malignant
embryonal
central nervous system
(
CNS
)
tumor
, manifesting in children, and composed
of rhabdoid
cells, with or without fields resembling a classical primitive neuroectodermal
tumor
(PNET), epithelial tissue and neoplastic mesenchyme.
Around 200 cases of
CNS AT
/RT have been documented in the literature.
Although the clinical and pathological findings have been defined in large series previously, and AT/RT has become increasingly recognized, awareness of typical AT/RT is important in making the correct
diagnosis
of this uncommon but probably underdiagnosed entity.
Neuroradiologists rarely mention AT/RT in their differential
diagnosis
and this paper presents two additional cases in which clinical and pathological findings are combined with neuroradiological presentation.
[MeSH-major]
Brain Neoplasms / pathology. Magnetic Resonance Imaging.
Rhabdoid Tumor
/ pathology. Teratoma / pathology
[MeSH-minor]
Child.
Diagnosis
, Differential. Fatal Outcome. Female. Humans. Infant. Male. Tomography, X-Ray Computed
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(PMID = 18382974.001).
[ISSN]
1019-5149
[Journal-full-title]
Turkish neurosurgery
[ISO-abbreviation]
Turk Neurosurg
[Language]
eng
[Publication-type]
Case Reports; Journal Article; Review
[Publication-country]
Turkey
[Number-of-references]
18
19.
Momota H, Holland EC:
Mouse models of CNS embryonal tumors.
Brain Tumor Pathol
; 2009;26(2):43-50
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[Title]
Mouse models of
CNS
embryonal tumors.
Central nervous system
(
CNS
) embryonal tumors are devastating cancers in children, consisting of medulloblastomas,
CNS
primitive neuroectodermal tumors, and
atypical teratoid
/
rhabdoid
tumors.
One of the reasons that
CNS
embryonal tumors remain difficult to treat is their rarity, which makes conducting clinical trials for these tumors difficult.
Based on the genetic alterations found in humans, multiple models of human
CNS
embryonal tumors have been generated in genetically engineered mice.
These mouse models are valuable tools for understanding brain
tumor
biology and discovering novel therapeutic targets and drugs.
In this article, we review molecular and cytogenetic characteristics of human
CNS
embryonal tumors and corresponding mouse models that have been developed.
These findings indicate that common genetic abnormalities are seen in variants of human
CNS
embryonal tumors, and multiple histological variants of these tumors can be generated from a single set of genetic abnormalities in mice.
These data provide insight into the biology and classification of
CNS
embryonal tumors.
[MeSH-major]
Central Nervous System
Neoplasms / genetics. Disease Models, Animal. Neoplasms, Germ Cell and Embryonal / genetics
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(PMID = 19856214.001).
[ISSN]
1861-387X
[Journal-full-title]
Brain tumor pathology
[ISO-abbreviation]
Brain Tumor Pathol
[Language]
eng
[Grant]
United States / NCI NIH HHS / CA / U01 CA141502
[Publication-type]
Journal Article; Review
[Publication-country]
Japan
[Number-of-references]
109
20.
Meyers SP, Khademian ZP, Biegel JA, Chuang SH, Korones DN, Zimmerman RA:
Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes.
AJNR Am J Neuroradiol
; 2006 May;27(5):962-71
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[Title]
Primary intracranial
atypical teratoid
/
rhabdoid
tumors of infancy and childhood: MRI features and patient outcomes.
BACKGROUND AND PURPOSE: Primary
atypical teratoid
/
rhabdoid
tumors (AT/RTs) are rare
malignant
intracranial neoplasms, usually occurring in young children.
The objectives of this study were to characterize the MR imaging features and locations of primary intracranial AT/RTs, to determine the frequency of disseminated disease in the
central nervous system
(
CNS
)
at diagnosis
and postoperatively, and to assess patient outcomes.
METHODS: The preoperative cranial MR images of 13 patients with AT/RTs were retrospectively reviewed for evaluation of lesion location, size, MR signal intensity and enhancement characteristics, and the presence of disseminated intracranial
tumor
.
Postoperative MR images of the head and spine for 17 patients were reviewed for the presence of locally recurrent or residual
tumor
and disseminated
neoplasm
.
A germ-line mutation of the hSNF5/INI1
tumor
-suppressor gene was responsible for the simultaneous occurrence of an intracranial AT/RT and a
malignant
renal
rhabdoid tumor
in a 4-month-old patient.
Mean
tumor
sizes were 3.6 x 3.8 x 3.9 cm.
The fraction
of tumor
volume showing enhancement was greater than two thirds in 58%, between one third and two thirds in 33%, and less than one third in 9%.
Disseminated
tumor
in the leptomeninges was seen with MR imaging in 24% of patients
at diagnosis
/initial staging and occurred in another 35% from 4 months to 2.8 years (mean, 1.1 years) after surgery and earlier imaging examinations with negative findings.
Patients with MR imaging evidence of disseminated leptomeningeal
tumor
had a median survival rate of 16 months compared with 149 months for those without disseminated
tumor
(P < .004, logrank test).
Poor prognosis is associated with MR imaging evidence of disseminated leptomeningeal
tumor
.
[MeSH-major]
Brain Neoplasms /
diagnosis
. Magnetic Resonance Imaging.
Rhabdoid Tumor
/
diagnosis
. Teratoma /
diagnosis
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(PMID = 16687525.001).
[ISSN]
0195-6108
[Journal-full-title]
AJNR. American journal of neuroradiology
[ISO-abbreviation]
AJNR Am J Neuroradiol
[Language]
eng
[Publication-type]
Journal Article
[Publication-country]
United States
21.
Chen ML, McComb JG, Krieger MD:
Atypical teratoid/rhabdoid tumors of the central nervous system: management and outcomes.
Neurosurg Focus
; 2005 Jun 15;18(6A):E8
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[Title]
Atypical teratoid
/
rhabdoid
tumors of the
central nervous system
: management and outcomes.
OBJECT:
Atypical teratoid
/
rhabdoid
tumors (ATRTs) represent a relatively newly categorized neoplastic entity.
Signs and symptoms began, on average, a little more than 1 month before
diagnosis
and included the following: headache (36%), nausea and vomiting (46%), lethargy (18%), seizures (27%), cranial nerve findings (46%), ataxia (18%), long tract findings (18%), and hydrocephalus (46%).
Tumor
location was cortical in four patients, in the pineal region in four, in the posterior fossa in two, and spinal in one.
In one patient disseminated disease was revealed on the initial imaging study; seven patients had disseminated
tumor
subsequently.
CONCLUSIONS:
Atypical teratoid
/
rhabdoid
tumors are
malignant
lesions with rapid progression.
[MeSH-major]
Central Nervous System
Neoplasms / therapy.
Rhabdoid Tumor
/ therapy. Teratoma / therapy
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(PMID = 16048294.001).
[ISSN]
1092-0684
[Journal-full-title]
Neurosurgical focus
[ISO-abbreviation]
Neurosurg Focus
[Language]
eng
[Publication-type]
Comparative Study; Journal Article
[Publication-country]
United States
22.
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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23.
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.
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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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[
16625210.001
]
(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
24.
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
25.
Varan A, Sari N, Akalan N, Söylemezoğlu F, Akyüz C, Kutluk T, Büyükpamukçu M:
Extraneural metastasis in intracranial tumors in children: the experience of a single center.
J Neurooncol
; 2006 Sep;79(2):187-90
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Our aim is to evaluate the clinical features and outcomes of children with primary
central nervous system
(
CNS
) tumors who develop extraneural metastasis (ENM).
We retrospectively evaluated children diagnosed with primary
CNS
tumors treated at our institution between 1972 and 2004.
The histopathologic
diagnosis
was medulloblastoma in six patients, germ cell tumors in two patients, and ependymoma and
atypical teratoid rhabdoid tumor
(ATRT) in one patient each.
In six patients, the primary
tumor
was located in the posterior fossa; it had a supratentorial location in the patient with ATRT, was located in the sellar and suprasellar region in the two patients with germ cell tumors, and was found in the distal spinal cord in the patient with an ependymoma.
In two patients ENM was detected at the time of
diagnosis
.
In other patients ENM developed between 9 and 25 months after
diagnosis
.
One patient with dysgerminoma is alive, without disease, 117.80 months after
diagnosis
of the ENM.
One patient with germ cell
tumor
is alive with disease 11.3 months after
diagnosis
of the ENM.
The outcome is poor in patients with
CNS
tumors with ENM.
[MeSH-major]
Central Nervous System
Neoplasms / pathology. Liver Neoplasms / secondary. Lung Neoplasms / secondary. Medulloblastoma / secondary. Neoplasms, Germ Cell and Embryonal / pathology
[MeSH-minor]
Adolescent. Bone Marrow Neoplasms / mortality. Bone Marrow Neoplasms / secondary. Bone Neoplasms / mortality. Bone Neoplasms / secondary. Child. Child, Preschool. Ependymoma / mortality. Ependymoma / secondary. Female. Humans. Male.
Neoplasm
Metastasis. Prognosis. Retrospective Studies.
Rhabdoid Tumor
/ mortality.
Rhabdoid Tumor
/ secondary. Teratoma / mortality. Teratoma / secondary
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[ISSN]
0167-594X
[Journal-full-title]
Journal of neuro-oncology
[ISO-abbreviation]
J. Neurooncol.
[Language]
eng
[Publication-type]
Journal Article
[Publication-country]
United States
26.
Finkelstein-Shechter T, Gassas A, Mabbott D, Huang A, Bartels U, Tabori U, Janzen L, Hawkins C, Taylor M, Bouffet E:
Atypical teratoid or rhabdoid tumors: improved outcome with high-dose chemotherapy.
J Pediatr Hematol Oncol
; 2010 Jul;32(5):e182-6
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[Title]
Atypical teratoid
or
rhabdoid
tumors: improved outcome with high-dose chemotherapy.
PURPOSE: To retrospectively review an institutional experience in managing
atypical teratoid
/
rhabdoid
tumors (AT/RT) of the
Central Nervous System
with high-dose chemotherapy in infants and children less than 4 years old.
Tumor
location was supratentorial in 3 cases, infratentorial in 3 cases, and multifocal in 2 patients.
RESULTS: At a median follow-up of 52 months, 4 patients are alive without evidence
of tumor
.
[MeSH-major]
Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy.
Rhabdoid Tumor
/ drug therapy. Teratoma / drug therapy
[MeSH-minor]
Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Cranial Irradiation. Cyclophosphamide / administration & dosage. Etoposide / administration & dosage. Female. Humans. Infant. Male.
Neoplasm
Metastasis. Radiotherapy Dosage. Retrospective Studies. Survival Rate. Treatment Outcome. Vincristine / administration & dosage
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VINCRISTINE
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[ErratumIn]
J Pediatr Hematol Oncol. 2011 Jul;33(5):400. Laura, Janzen [corrected to Janzen, Laura]
(PMID = 20495479.001).
[ISSN]
1536-3678
[Journal-full-title]
Journal of pediatric hematology/oncology
[ISO-abbreviation]
J. Pediatr. Hematol. Oncol.
[Language]
eng
[Publication-type]
Journal Article; Research Support, Non-U.S. Gov't
[Publication-country]
United States
[Chemical-registry-number]
5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin
27.
Yano S, Hida K, Kobayashi H, Iwasaki Y:
Successful multimodal therapies for a primary atypical teratoid/rhabdoid tumor in the cervical spine.
Pediatr Neurosurg
; 2008;44(5):406-13
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[Title]
Successful multimodal therapies for a primary
atypical teratoid
/
rhabdoid tumor
in the cervical spine.
Atypical teratoid
/
rhabdoid tumor
(AT/RT) occurring in the
central nervous system
is a high-grade
malignant
tumor
, and its prognosis is poor for patients younger than 3 years of age.
At the age of nearly 3 years, she received radiation therapy to the local
tumor
bed and craniospinal axis.
The success of this treatment for the patient was that we could prevent
tumor
recurrence until she was able to receive radiotherapy.
[MeSH-major]
Cervical Vertebrae / pathology.
Rhabdoid Tumor
/ drug therapy. Spinal Neoplasms / drug therapy. Teratoma / drug therapy
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[Copyright]
(c) 2008 S. Karger AG, Basel.
(PMID = 18703889.001).
[ISSN]
1423-0305
[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 / Antineoplastic Agents
[Number-of-references]
22
28.
Tekautz TM, Fuller CE, Blaney S, Fouladi M, Broniscer A, Merchant TE, Krasin M, Dalton J, Hale G, Kun LE, Wallace D, Gilbertson RJ, Gajjar A:
Atypical teratoid/rhabdoid tumors (ATRT): improved survival in children 3 years of age and older with radiation therapy and high-dose alkylator-based chemotherapy.
J Clin Oncol
; 2005 Mar 1;23(7):1491-9
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[Title]
Atypical teratoid
/
rhabdoid
tumors (ATRT): improved survival in children 3 years of age and older with radiation therapy and high-dose alkylator-based chemotherapy.
PURPOSE: To describe clinical features, therapeutic approaches, and prognostic factors in pediatric patients with
atypical teratoid
/
rhabdoid
tumors (ATRT) treated at St Jude Children's Research Hospital (SJCRH).
PATIENTS AND METHODS: Primary
tumor
samples from patients diagnosed with ATRT at SJCRH between July 1984 and June 2003 were identified.
Posterior fossa primary lesions and metastatic disease
at diagnosis
were more common in younger patients with ATRT.
[MeSH-major]
Central Nervous System
Neoplasms / therapy. Neoplasms, Germ Cell and Embryonal / therapy.
Rhabdoid Tumor
/ therapy. Teratoma / therapy
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(PMID = 15735125.001).
[ISSN]
0732-183X
[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 / CA 21765
[Publication-type]
Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
[Publication-country]
United States
[Chemical-registry-number]
0 / SMARCC1 protein, human; 0 / Transcription Factors
29.
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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[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
30.
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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[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
31.
Ferri Niguez B, MartÃnez-Lage JF, Almagro MJ, Fuster JL, Serrano C, Torroba MA, Sola J:
Embryonal tumor with abundant neuropil and true rosettes (ETANTR): a new distinctive variety of pediatric PNET: a case-based update.
Childs Nerv Syst
; 2010 Aug;26(8):1003-8
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[Title]
Embryonal
tumor
with abundant neuropil and true rosettes (ETANTR): a new distinctive variety of pediatric PNET: a case-based update.
BACKGROUND: Embryonal
central nervous system
(
CNS
) tumors are currently classified into three types: medulloblastoma,
atypical rhabdoid
/
teratoid
tumors, and primitive neuroectodermal
tumor
(PNET).
A distinctive subtype of PNET called "embryonal
tumor
with abundant neuropil and true rosettes" (ETANTR) was reported in 2000.
It has been suggested that this
neoplasm
should be considered as a separate entity.
ETANTR is an eminently pediatric
tumor
that has been reported exclusively in children younger than 4 years.
ILLUSTRATIVE CASES: A 9-month-old girl underwent subtotal resection of a brainstem
neoplasm
.
A 23-month-old girl was submitted to surgery for a frontoparietal
tumor
.
In both instances, the histopathological
diagnosis
confirmed ETANTR.
CONCLUSIONS: By reporting these two new instances of ETANTR, we want to contribute to the knowledge of this highly
malignant CNS
embryonal
neoplasm
that occurs only in young children, given its present lethal prognosis, the scarcity of reported cases, and the lack of treatment guidelines.
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(PMID = 20499240.001).
[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 / Antineoplastic Agents
32.
Judkins AR:
Immunohistochemistry of INI1 expression: a new tool for old challenges in CNS and soft tissue pathology.
Adv Anat Pathol
; 2007 Sep;14(5):335-9
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[Title]
Immunohistochemistry of INI1 expression: a new tool for old challenges in
CNS
and soft tissue pathology.
Loss of expression of INI1 as detected by immunohistochemical staining correlates with deletion and mutations of the INI1 gene in patients with
malignant
rhabdoid
tumors and
central nervous system atypical teratoid
/
rhabdoid
tumors and as such is a sensitive and specific marker for these tumors.
Loss of INI1 expression may rarely be encountered in other
tumor
types.
[MeSH-major]
Biomarkers,
Tumor
/ metabolism.
Central Nervous System
Neoplasms /
diagnosis
. Chromosomal Proteins, Non-Histone / metabolism. DNA-Binding Proteins / metabolism. Immunohistochemistry / methods. Soft Tissue Neoplasms /
diagnosis
. Transcription Factors / metabolism
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(PMID = 17717433.001).
[ISSN]
1072-4109
[Journal-full-title]
Advances in anatomic pathology
[ISO-abbreviation]
Adv Anat Pathol
[Language]
eng
[Publication-type]
Journal Article; Review
[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
[Number-of-references]
20
33.
Narendran A, Coppes L, Jayanthan A, Coppes M, Teja B, Bernoux D, George D, Strother D:
Establishment of atypical-teratoid/rhabdoid tumor (AT/RT) cell cultures from disseminated CSF cells: a model to elucidate biology and potential targeted therapeutics.
J Neurooncol
; 2008 Nov;90(2):171-80
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[Title]
Establishment
of atypical
-
teratoid
/
rhabdoid tumor
(AT/RT) cell cultures from disseminated CSF cells: a model to elucidate biology and potential targeted therapeutics.
Atypical teratoid
/
rhabdoid tumor
(AT/RT) is a highly
malignant
central nervous system
neoplasm
that usually affects infants and young children.
In this report, we describe culture conditions that enabled the sustained growth
of tumor
cells obtained from the cerebrospinal fluid (CSF) of an infant with AT/RT.
These cells retained the morphological and biomarker characteristics of the original
tumor
.
In addition to previously described cell lines and xenograft models, continuous culture of CSF derived cells may also provide an effective way to study the biology of AT/RT and to identify potential targets for future therapeutics for this
tumor
.
[MeSH-major]
Platelet Aggregation Inhibitors / therapeutic use.
Rhabdoid Tumor
/ cerebrospinal fluid.
Rhabdoid Tumor
/ drug therapy
[MeSH-minor]
Actins / metabolism. Cell Proliferation. Chromosomal Proteins, Non-Histone / metabolism. DNA-Binding Proteins / metabolism. Dose-Response Relationship, Drug. Glial Fibrillary Acidic Protein / metabolism. Humans. Infant. Inhibitory Concentration 50. Male. Models, Biological. Nuclear Proteins / metabolism. Receptor Protein-Tyrosine Kinases / metabolism. SMARCB1 Protein. Transcription Factors / metabolism.
Tumor
Cells, Cultured / pathology. Vimentin / metabolism
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.
Cellosaurus - a cell line knowledge resource.
culture/stock collections - Cellosaurus - a cell line knowledge resource
.
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]
(PMID = 18651103.001).
[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 / Actins; 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / Glial Fibrillary Acidic Protein; 0 / Nuclear Proteins; 0 / Platelet Aggregation Inhibitors; 0 / SMARCB1 Protein; 0 / SMARCB1 protein, human; 0 / Transcription Factors; 0 / Vimentin; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases
34.
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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[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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.
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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
35.
Fouladi M, Gururangan S, Moghrabi A, Phillips P, Gronewold L, Wallace D, Sanford RA, Gajjar A, Kun LE, Heideman R:
Carboplatin-based primary chemotherapy for infants and young children with CNS tumors.
Cancer
; 2009 Jul 15;115(14):3243-53
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[Title]
Carboplatin-based primary chemotherapy for infants and young children with
CNS
tumors.
BACKGROUND: A carboplatin-based chemotherapy regimen was used as primary postoperative therapy in infants with
central nervous system
(
CNS
) tumors to limit renal and ototoxicity and to target systemic exposure.
METHODS: Fifty-three patients aged <age 3 years with embryonal
CNS
tumor
medulloblastoma (n = 20), ependymoma (EP, n = 21), choroid plexus carcinoma (CPCA, n = 5), and primitive embryonal neoplasms including
atypical teratoid rhabdoid
tumors (n = 7) were treated with cyclophosphamide, etoposide, and carboplatin.
For medulloblastoma, the 5-year PFS was 26% +/- 9%; for EP it was 33% +/- 10%; for CPCA it was 80% +/- 18%; and for primitive neuroectodermal and
atypical teratoid rhabdoid
tumors it was 0%.
[MeSH-major]
Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Carboplatin / administration & dosage.
Central Nervous System
Neoplasms / drug therapy
COS Scholar Universe.
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.
Hazardous Substances Data Bank.
ETOPOSIDE
.
Hazardous Substances Data Bank.
CARBOPLATIN
.
Hazardous Substances Data Bank.
CYCLOPHOSPHAMIDE
.
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(PMID = 19484793.001).
[ISSN]
0008-543X
[Journal-full-title]
Cancer
[ISO-abbreviation]
Cancer
[Language]
eng
[Grant]
United States / NCI NIH HHS / CA / P30 CA021765; United States / NCI NIH HHS / CA / P30 CA021765-30; United States / NCI NIH HHS / CA / R25 CA023944; United States / NCI NIH HHS / CA / CA21765
[Publication-type]
Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
[Publication-country]
United States
[Chemical-registry-number]
6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; BG3F62OND5 / Carboplatin
[Other-IDs]
NLM/ NIHMS124374; NLM/ PMC4307774
36.
Lafay-Cousin L, Strother D:
Current treatment approaches for infants with malignant central nervous system tumors.
Oncologist
; 2009 Apr;14(4):433-44
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[Title]
Current treatment approaches for infants with
malignant
central nervous system
tumors.
Nearly three decades of infant brain
tumor
clinical trials have led to significant progress in the delineation of prognostic factors and improvements in outcome.
This review covers the most recent therapeutic advances for the most common histological subtypes of
malignant
infant brain tumors: medulloblastoma, supratentorial primitive neuroectodermal
tumor
, ependymoma,
atypical teratoid rhabdoid tumor
, choroid plexus carcinoma, and high-grade glioma.
[MeSH-minor]
Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Carcinoma / therapy. Cerebellar Neoplasms / therapy. Chemotherapy, Adjuvant. Choroid Plexus Neoplasms / therapy. Clinical Trials as Topic. Clinical Trials, Phase III as Topic. Ependymoma / therapy. Evidence-Based Medicine. Glioma / therapy. Humans. Infant. Medulloblastoma / therapy. Meta-Analysis as Topic. Neuroectodermal Tumors, Primitive / therapy. Prognosis. Radiotherapy Dosage. Radiotherapy, Adjuvant.
Rhabdoid Tumor
/ therapy. Stem Cell Transplantation. Supratentorial Neoplasms / therapy
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(PMID = 19342475.001).
[ISSN]
1549-490X
[Journal-full-title]
The oncologist
[ISO-abbreviation]
Oncologist
[Language]
eng
[Publication-type]
Journal Article; Review
[Publication-country]
United States
[Number-of-references]
116
37.
Makuria AT, Rushing EJ, McGrail KM, Hartmann DP, Azumi N, Ozdemirli M:
Atypical teratoid rhabdoid tumor (AT/RT) in adults: review of four cases.
J Neurooncol
; 2008 Jul;88(3):321-30
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[Title]
Atypical teratoid rhabdoid tumor
(AT/RT) in adults: review of four cases.
Atypical teratoid
/
rhabdoid
(AT/RT)
tumor
is a rare, highly
malignant
tumor of
the
central nervous system
(
CNS
) most commonly found in children less than 5 years of age.
In many instances, a reliable
diagnosis
is not possible without demonstrating the lack of nuclear INI1 protein expression by immunohistochemical methods.
In all cases,
diagnosis
during intraoperative consultation and preliminary
diagnosis
was different from the final
diagnosis
after immunohistochemical analysis.
Immunohistochemical staining showed that the
tumor
cells were positive for vimentin and reacted variably for keratin, epithelial membrane antigen (EMA), synaptophysin, neurofilament protein, CD34, and smooth muscle actin (SMA).
One patient is alive with no evidence of disease 17 years after the
diagnosis
.
In adult examples of AT/RT, the
diagnosis
requires a high index of suspicion, with early tissue
diagnosis
and a low threshold for investigation with INI1 immunohistochemistry to differentiate this entity from other morphologically similar tumors.
[MeSH-major]
Brain Neoplasms / metabolism. Brain Neoplasms / pathology.
Rhabdoid Tumor
/ metabolism.
Rhabdoid Tumor
/ pathology
[MeSH-minor]
Adult. Chromosomal Proteins, Non-Histone / metabolism. DNA-Binding Proteins / metabolism.
Diagnosis
, Differential. Female. Humans. Immunohistochemistry. Male. SMARCB1 Protein. Transcription Factors / metabolism
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[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 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / SMARCB1 Protein; 0 / SMARCB1 protein, human; 0 / Transcription Factors
38.
Feiden S, Sartorius E, Feiden W:
[The role of diagnostic neuropathology in familial tumour syndromes].
Pathologe
; 2010 Oct;31(6):464-70
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[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
]
[Cites]
Genet Med. 2009 Sep;11(9):599-610
[
19652604.001
]
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J Neurosurg. 1999 Dec;91(6):971-7
[
10584843.001
]
(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
39.
Ertan Y, Sezak M, Turhan T, Kantar M, ErÅŸahin 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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[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
Genetic Alliance.
consumer health - Rhabdoid tumor
.
MedlinePlus Health Information.
consumer health - Brain Tumors
.
MedlinePlus Health Information.
consumer health - Childhood Brain Tumors
.
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[CommentIn]
Childs Nerv Syst. 2009 Nov;25(11):1387; author reply 1389
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[
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]
[Cites]
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[
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]
[Cites]
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[
9737241.001
]
[Cites]
J Neuropathol Exp Neurol. 2005 May;64(5):391-7
[
15892296.001
]
[Cites]
Ultrastruct Pathol. 1997 Jul-Aug;21(4):369-78
[
9206002.001
]
[Cites]
Childs Nerv Syst. 2008 Jan;24(1):143-7
[
17968559.001
]
(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
40.
Sarkar C, Deb P, Sharma MC:
Recent advances in embryonal tumours of the central nervous system.
Childs Nerv Syst
; 2005 Apr;21(4):272-93
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[Title]
Recent advances in embryonal tumours of the
central nervous system
.
INTRODUCTION: Embryonal tumours of the
central nervous system
(
CNS
) are the commonest
malignant
paediatric brain tumours.
This group includes medulloblastomas, supratentorial primitive neuroectodermal tumours,
atypical teratoid
/
rhabdoid
tumours, ependymoblastomas, and medulloepitheliomas.
[MeSH-major]
Central Nervous System
Neoplasms / embryology. Neoplasms, Germ Cell and Embryonal / genetics. Neoplasms, Germ Cell and Embryonal / pathology
COS Scholar Universe.
author profiles
.
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[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; Review
[Publication-country]
Germany
[Chemical-registry-number]
0 / Transcription Factors
[Number-of-references]
236
41.
Severino M, Schwartz ES, Thurnher MM, Rydland J, Nikas I, Rossi A:
Congenital tumors of the central nervous system.
Neuroradiology
; 2010 Jun;52(6):531-48
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[Title]
Congenital tumors of the
central nervous system
.
Congenital tumors of the
central nervous system
(
CNS
) are often arbitrarily divided into "definitely congenital" (present or producing symptoms at birth), "probably congenital" (present or producing symptoms within the first week of life), and "possibly congenital" (present or producing symptoms within the first 6 months of life).
The prenatal
diagnosis
of congenital
CNS
tumors, while based on ultrasonography, has significantly benefited from the introduction of prenatal magnetic resonance imaging studies.
Teratomas constitute about one third to one half of these tumors and are the most common neonatal brain
tumor
.
They are often immature because of primitive neural elements and, rarely, a component of mixed
malignant
germ cell tumors.
Other tumors include astrocytomas, choroid plexus papilloma, primitive neuroectodermal tumors,
atypical teratoid
/
rhabdoid
tumors, and medulloblastomas.
The prognosis for patients with congenital tumors is generally poor, usually because of the massive size of the
tumor
.
[MeSH-major]
Central Nervous System
Neoplasms / congenital.
Central Nervous System
Neoplasms /
diagnosis
. Magnetic Resonance Imaging / methods. Tomography, X-Ray Computed / methods
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[ISSN]
1432-1920
[Journal-full-title]
Neuroradiology
[ISO-abbreviation]
Neuroradiology
[Language]
eng
[Publication-type]
Journal Article
[Publication-country]
Germany
42.
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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.
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.
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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
43.
Tena-Suck ML, Gómez-Amador JL, Ortiz-Plata A, Salina-Lara C, Rembao-Bojórquez D, Vega-Orozco R:
Rhabdoid choroid plexus carcinoma: a rare histological type.
Arq Neuropsiquiatr
; 2007 Sep;65(3A):705-9
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[Title]
Rhabdoid
choroid plexus carcinoma: a rare histological type.
Primary
central nervous system atypical teratoid
/
rhabdoid
tumors mostly occur during early childhood and are almost invariably fatal.
These tumors show similar histological and radiological features to primitive neuroectodermal
tumor
, medulloblastoma and choroid plexus carcinoma, but present different biological behaviors.
CT-scan and MRI revealed a posterior fossa
tumor
.
Histological examination showed two different main growth patterns: solid sheets of undifferentiated polygonal cells with papillary features and
rhabdoid
cells.
Immunohistochemically, these
rhabdoid
cells were positive for vimentin, epithelial membrane antigen, smooth-muscle actin, cytokeratin, S-100 protein, and glial fibrillary acidic protein.
Electro-microscopically, the typical
rhabdoid
cells contained whorled bundles of intermediate filaments in their cytoplasm.
A
rhabdoid tumor
is a clinical-pathological entity and emphasizes the necessity to distinguish this unique
tumor
from other pediatric
central nervous system
neoplasms.
[MeSH-major]
Carcinoma / ultrastructure. Choroid Plexus Neoplasms / ultrastructure.
Rhabdoid Tumor
/ ultrastructure
Genetic Alliance.
consumer health - Choroid Plexus Carcinoma
.
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(PMID = 17876420.001).
[ISSN]
0004-282X
[Journal-full-title]
Arquivos de neuro-psiquiatria
[ISO-abbreviation]
Arq Neuropsiquiatr
[Language]
eng
[Publication-type]
Case Reports; Journal Article
[Publication-country]
Brazil
44.
Garrè ML, Tekautz T:
Role of high-dose chemotherapy (HDCT) in treatment of atypical teratoid/rhabdoid tumors (AT/RTs).
Pediatr Blood Cancer
; 2010 Apr;54(4):647-8
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[Title]
Role of high-dose chemotherapy (HDCT) in treatment
of atypical teratoid
/
rhabdoid
tumors (AT/RTs).
Atypical teratoid
/
rhabdoid
tumors (AT/RTs) of the
CNS
have been recently characterized as a distinct clinicopathologic entity with an unusually poor prognosis and with the highest incidence in the first 2 years of life.
It often arises in the posterior fossa and its distinctive immunohistochemical (negative stain for INI-1) and cytogenetic features (monosomy or deletion of chromosome 22) permit an adequate
diagnosis
in most of cases.
AT/RT of the
CNS
is a usually fatal disease virtually unresponsive to chemotherapy (CT) and radiotherapy (RT).
Rapid progression and
CNS
dissemination are commonly reported.
Whether combined regimens including high-dose CT are able to prolong survival or change the natural history of this
tumor
are under evaluation.
[MeSH-major]
Antineoplastic Agents / therapeutic use.
Central Nervous System
Neoplasms / drug therapy.
Rhabdoid Tumor
/ drug therapy. Teratoma / drug therapy
MedlinePlus Health Information.
consumer health - Cancer Chemotherapy
.
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(PMID = 20146222.001).
[ISSN]
1545-5017
[Journal-full-title]
Pediatric blood & cancer
[ISO-abbreviation]
Pediatr Blood Cancer
[Language]
eng
[Publication-type]
Journal Article; Review
[Publication-country]
United States
[Chemical-registry-number]
0 / Antineoplastic Agents
[Number-of-references]
18
45.
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
Genetic Alliance.
consumer health - Rhabdoid tumor
.
Genetic Alliance.
consumer health - Brain Tumor, Adult
.
MedlinePlus Health Information.
consumer health - Brain Tumors
.
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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
46.
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
NCI CPTC Antibody Characterization Program.
NCI CPTC Antibody Characterization Program
.
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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
47.
Squire SE, Chan MD, Marcus KJ:
Atypical teratoid/rhabdoid tumor: the controversy behind radiation therapy.
J Neurooncol
; 2007 Jan;81(1):97-111
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[Title]
Atypical teratoid
/
rhabdoid tumor
: the controversy behind radiation therapy.
To date, approximately 200 cases
of atypical teratoid
/
rhabdoid tumor
(AT/RT) of the
central nervous system
have been described in the literature.
This
CNS
tumor
tends to present at an age of less than 3 years, and most patients succumb to their disease within 1 year of
diagnosis
.
Prior to the rise in utilization of immunohistochemical (IHC) testing in the late 1990s, this
tumor
was likely mistaken as medulloblastoma and treated as such.
A standardized and effective approach to treating this usually fatal
tumor
remains elusive, and the role of radiation therapy presents a particular dilemma as young patients with this disease may experience devastating late effects of therapy if they achieve a long-term survival.
Our review underscores the importance or enrolling patients in multi-institutional prospective studies to further investigate the value of radiation to treat this pediatric
neoplasm
.
[MeSH-major]
Central Nervous System
Neoplasms / radiotherapy. Radiotherapy / methods.
Rhabdoid Tumor
/ radiotherapy. Teratoma / radiotherapy
Genetic Alliance.
consumer health - Rhabdoid tumor
.
MedlinePlus Health Information.
consumer health - Radiation Therapy
.
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[
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]
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[
9206002.001
]
(PMID = 16855864.001).
[ISSN]
0167-594X
[Journal-full-title]
Journal of neuro-oncology
[ISO-abbreviation]
J. Neurooncol.
[Language]
eng
[Publication-type]
Journal Article; Review
[Publication-country]
United States
[Chemical-registry-number]
0 / Antineoplastic Agents
[Number-of-references]
92
48.
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
49.
Wang CH, Hsu TR, Wong TT, Chang KP:
Efficacy of temozolomide for recurrent embryonal brain tumors in children.
Childs Nerv Syst
; 2009 May;25(5):535-41
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Temozolomide is a newly developed chemotherapeutic agent in
central nervous system
tumors.
MATERIALS AND METHODS: There were eight patients, including four with medulloblastoma (MB), three with
atypical teratoid
/
rhabdoid tumor
(AT/RT) and one with supratentorial primitive neuroectodermal
tumor
, whose tumors recurred after surgery and radiotherapy, with or without conventional intravenous cisplatin-based chemotherapy.
The follow-up MRI showed no
tumor
progression in five patients at 6 months and four patients at 12 months.
[MeSH-major]
Antineoplastic Agents, Alkylating / therapeutic use. Brain Neoplasms / drug therapy. Dacarbazine / analogs & derivatives. Medulloblastoma / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy.
Rhabdoid Tumor
/ drug therapy. Teratoma / drug therapy
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.
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.
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[Cites]
Ann Oncol. 2001 Feb;12 (2):259-66
[
11300335.001
]
[Cites]
J Neuropathol Exp Neurol. 2002 Mar;61(3):215-25; discussion 226-9
[
11895036.001
]
[Cites]
N Engl J Med. 1994 Mar 31;330(13):892-5
[
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]
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Cancer Invest. 2007 Sep;25(6):470-5
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]
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[
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]
[Cites]
Neoplasma. 2002;49(2):117-20
[
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]
[Cites]
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]
[Cites]
Cancer. 2005 Nov 15;104(10):2156-67
[
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]
[Cites]
Acta Neurol Belg. 2007 Jun;107(2):51-4
[
17710841.001
]
(PMID = 19107490.001).
[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]
Journal Article
[Publication-country]
Germany
[Chemical-registry-number]
0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; YF1K15M17Y / temozolomide
50.
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
51.
Pytel P, Lukas RV:
Update on diagnostic practice: tumors of the nervous system.
Arch Pathol Lab Med
; 2009 Jul;133(7):1062-77
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[Title]
Update on diagnostic practice: tumors of the
nervous system
.
The development of new treatment strategies also leads to different questions in the assessment of brain tumors, as seen in the example of pseudoprogression or the changes in
tumor
growth pattern in patients taking bevacizumab.
Finally, the discussion of primitive pediatric tumors stresses the clinical importance of the distinction between different entities, like
atypical teratoid rhabdoid tumor
, "
central
" (supratentorial) primitive neuroectodermal
tumor
, "peripheral" primitive neuroectodermal
tumor
, and medulloblastoma.
[MeSH-major]
Brain Neoplasms /
diagnosis
.
Nervous System
Neoplasms /
diagnosis
. World Health Organization
[MeSH-minor]
Glioblastoma / classification. Glioblastoma /
diagnosis
. Humans. Medulloblastoma / classification. Medulloblastoma /
diagnosis
. Oligodendroglioma / classification. Oligodendroglioma /
diagnosis
.
Rhabdoid Tumor
/ classification.
Rhabdoid Tumor
/
diagnosis
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(PMID = 19642733.001).
[ISSN]
1543-2165
[Journal-full-title]
Archives of pathology & laboratory medicine
[ISO-abbreviation]
Arch. Pathol. Lab. Med.
[Language]
eng
[Publication-type]
Journal Article; Review
[Publication-country]
United States
[Number-of-references]
139
52.
Erickson ML, Johnson R, Bannykh SI, de Lotbiniere A, Kim JH:
Malignant rhabdoid tumor in a pregnant adult female: literature review of central nervous system rhabdoid tumors.
J Neurooncol
; 2005 Sep;74(3):311-9
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[Title]
Malignant
rhabdoid tumor
in a pregnant adult female: literature review
of central nervous system rhabdoid
tumors.
Rhabdoid
tumors of the
central nervous system
are uncommon, aggressive childhood malignancies.
The 13 described adult cases comprise both primary
CNS
tumors and
malignant
transformation of previously existing gliomas, meningiomas, and astrocytomas.
Central nervous system rhabdoid
lesions of adults have been diagnosed as primary
malignant
rhabdoid
tumors,
atypical teratoid
/
rhabdoid
tumors, and more recently,
rhabdoid
glioblastomas.
Pathologic evaluation revealed histology, electron microscopy and immunohistochemistry consistent with the
diagnosis
of
malignant
rhabdoid tumor
.
FISH studies were negative for the INI-1 genetic mutations and chromosome 22q deletion associated with childhood
atypical rhabdoid
/
rhabdoid tumor
in 75% of cases.
We briefly describe the characteristics and current understanding
of rhabdoid
tumors, and review the literature comparing the 12 other cases
of central nervous system rhabdoid
tumors in adults.
[MeSH-major]
Brain Neoplasms / pathology. Pregnancy Complications, Neoplastic / pathology.
Rhabdoid Tumor
/ pathology
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[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
53.
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
54.
Choi YL, Kim CJ, Matsuo T, Gaetano C, Falconi R, Suh YL, Kim SH, Shin YK, Park SH, Chi JG, Thiele CJ:
HUlip, a human homologue of unc-33-like phosphoprotein of Caenorhabditis elegans; Immunohistochemical localization in the developing human brain and patterns of expression in nervous system tumors.
J Neurooncol
; 2005 May;73(1):19-27
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[Title]
HUlip, a human homologue of unc-33-like phosphoprotein of Caenorhabditis elegans; Immunohistochemical localization in the developing human brain and patterns of expression in
nervous system
tumors.
HUlip is a human homologue of a C. elegans gene, unc-33, that is developmentally regulated during maturation of the
nervous system
.
The purpose of this study was to investigate the pattern of hUlip expression in the developing human brain and
nervous system
tumors.
Ten human brains at different developmental stages and 118 cases
of nervous system tumor
tissues were examined by immunohistochemistry.
Twelve related
tumor
cell lines were also analyzed by northern blotting and immunoblotting.
Among tumors, hUlip expression was easily detected in
tumor
cells undergoing neuronal differentiation such as ganglioneuroblastomas and ganglioneuromas.
Furthermore, hUlip immunoreactivity was also found in various brain tumors showing neuronal differentiation:
central
neurocytomas (6 of 6 cases were positive), medulloblastomas (5/11),
atypical teratoid rhabdoid tumor
(1/1) and gangliogliomas (4/7).
The results of this study indicate that the expression of hUlip protein is distinctly restricted to the late fetal and early postnatal periods of human
nervous system
development and to certain subsets
of nervous system
tumors.
The exact function of hUlip needs to be further clarified; yet the results of our study strongly imply that hUlip function is important in human
nervous system
development and its aberrant expression in various types
of nervous system
tumors suggests a role of hUlip as an oncofetal neural antigen.
[MeSH-minor]
Astrocytes / cytology. Astrocytes / metabolism. Cell Differentiation / genetics. Cell Differentiation / physiology. Cell Line,
Tumor
. Female. Gestational Age. Humans. Immunohistochemistry. Male. Neuroblastoma / genetics. Neuroblastoma / metabolism. Neurons / cytology. Neurons / metabolism
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(PMID = 15933812.001).
[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 / DPYSL3 protein, human; 0 / Muscle Proteins
55.
Honda M, Baba H, Yonekura M, Iseki M:
Cerebral composite atypical teratoid/rhabdoid tumor and yolk sac tumor in the frontal lobe of an infant. Case report.
Neurol Med Chir (Tokyo)
; 2005 Jun;45(6):318-21
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[Title]
Cerebral composite
atypical teratoid
/
rhabdoid tumor
and yolk sac
tumor
in the frontal lobe of an infant. Case report.
A 1-year-old male infant presented with a rare cerebral composite
tumor
consisting
of atypical teratoid
/
rhabdoid tumor
(AT/RT) with epithelial and mesenchymal components and yolk sac
tumor
(YST) with Schiller-Duval bodies.
Computed tomography and magnetic resonance imaging revealed a large, intra-axial solid
tumor
with a cyst in the left frontal lobe.
Total resection of the
tumor
was performed.
Histological examination showed two different main growth patterns: solid sheets of undifferentiated polygonal cells and a few
rhabdoid
cells with rosette structures and rhabdomyoblastic cells; and reticular or papillary structures with occasional Schiller-Duval bodies in a myxoid matrix.
Initial total resection of the
tumor
was subsequently followed by local recurrence, hydrocephalus, and spinal metastasis.
AT/RT is a recently established entity of the
central nervous system
.
[MeSH-major]
Brain Neoplasms / pathology. Endodermal Sinus
Tumor
/ pathology. Frontal Lobe / pathology.
Rhabdoid Tumor
/ pathology. Teratoma / pathology
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(PMID = 15973067.001).
[ISSN]
0470-8105
[Journal-full-title]
Neurologia medico-chirurgica
[ISO-abbreviation]
Neurol. Med. Chir. (Tokyo)
[Language]
eng
[Publication-type]
Case Reports; Journal Article
[Publication-country]
Japan
56.
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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[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
57.
Frühwald MC, Hasselblatt M, Wirth S, Köhler G, Schneppenheim R, Subero JI, Siebert R, Kordes U, Jürgens H, Vormoor J:
Non-linkage of familial rhabdoid tumors to SMARCB1 implies a second locus for the rhabdoid tumor predisposition syndrome.
Pediatr Blood Cancer
; 2006 Sep;47(3):273-8
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[Title]
Non-linkage of familial
rhabdoid
tumors to SMARCB1 implies a second locus for the
rhabdoid tumor
predisposition syndrome.
BACKGROUND:
Rhabdoid
tumors represent an independent entity among embryonal neoplasms.
These tumors affect the kidney (RTK,
rhabdoid tumor
of kidney) and
central nervous system
(AT/RT,
atypical teratoid
,
rhabdoid tumor
), but may also be found in peripheral soft tissue.
Unifying features include immunohistochemical characteristics and inactivation of the putative
tumor
suppressor gene SMARCB1 (hSNF5/INI1) in chromosome 22q11.2.
Several familial cases have been published and summarized under the term
rhabdoid tumor
predisposition syndrome.
In all of the published familial cases, inactivation of SMARCB1 was detected in
tumor
tissues.
PROCEDURE AND RESULTS: We report on a family with three children, two of which were affected by
rhabdoid
tumors, one RTK, the other an AT/RT.
Immunohistochemistry for SMARCB1 showed normal expression within the nuclei
of tumor
cells.
CONCLUSIONS: We thus demonstrate a family with
rhabdoid tumor
predisposition syndrome without linkage to SMARCB1.
[MeSH-major]
Chromosomal Proteins, Non-Histone / genetics. DNA-Binding Proteins / genetics. Genetic Linkage.
Rhabdoid Tumor
/ genetics. Transcription Factors / genetics
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.
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[CommentIn]
Pediatr Blood Cancer. 2006 Sep;47(3):235-7
[
16304667.001
]
(PMID = 16206192.001).
[ISSN]
1545-5009
[Journal-full-title]
Pediatric blood & cancer
[ISO-abbreviation]
Pediatr Blood Cancer
[Language]
eng
[Publication-type]
Case Reports; Comparative Study; 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, human; 0 / Transcription Factors
58.
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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[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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J Clin Oncol. 2004 Jul 15;22(14):2877-84
[
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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
59.
Reddy AT:
Atypical teratoid/rhabdoid tumors of the central nervous system.
J Neurooncol
; 2005 Dec;75(3):309-13
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[Title]
Atypical teratoid
/
rhabdoid
tumors of the
central nervous system
.
Atypical teratoid
/
rhabdoid tumor
(AT/RT) is a highly
malignant
central nervous system
neoplasm
that usually affects very young children and is typically deadly despite very aggressive treatment.
Considered rare, the
tumor
was not recognized as a distinct entity until the 80's, due to its similar features with other primitive tumors.
AT/RT is the first pediatric brain
tumor
for which a candidate
tumor
suppressor gene has been identified.
[MeSH-major]
Central Nervous System
Neoplasms / genetics.
Central Nervous System
Neoplasms / pathology. Genes,
Tumor
Suppressor.
Rhabdoid Tumor
/ genetics.
Rhabdoid Tumor
/ pathology. Teratoma / genetics. Teratoma / pathology
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[ISSN]
0167-594X
[Journal-full-title]
Journal of neuro-oncology
[ISO-abbreviation]
J. Neurooncol.
[Language]
eng
[Publication-type]
Journal Article; Review
[Publication-country]
United States
[Number-of-references]
19
60.
Edgar MA, Rosenblum MK:
The differential diagnosis of central nervous system tumors: a critical examination of some recent immunohistochemical applications.
Arch Pathol Lab Med
; 2008 Mar;132(3):500-9
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[Title]
The differential
diagnosis
of central nervous system
tumors: a critical examination of some recent immunohistochemical applications.
These will be used for purposes
of tumor
subclassification, as prognostic markers, as identifiers of potential therapeutic targets, and as predictors of treatment response.
OBJECTIVE: To provide for nonspecialists a critical assessment of the peer-reviewed literature (necessarily colored by our own experience) as it pertains to several immunohistochemical reagents that have been recently forwarded as adjuncts to the histologic typing
of central nervous system
tumors.
CONCLUSIONS: Discussion concentrates on the use of 4 antibodies: BAF47 in the
diagnosis
of atypical teratoid
/
rhabdoid tumor
, OCT4 in intracranial germinoma, beta-catenin in craniopharyngioma, and NeuN as a marker of neuronal differentiation in neuroepithelial neoplasms.
[MeSH-major]
Biomarkers,
Tumor
/ analysis.
Central Nervous System
Neoplasms /
diagnosis
[MeSH-minor]
Diagnosis
, Differential. Humans. Immunohistochemistry
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(PMID = 18318590.001).
[ISSN]
1543-2165
[Journal-full-title]
Archives of pathology & laboratory medicine
[ISO-abbreviation]
Arch. Pathol. Lab. Med.
[Language]
eng
[Publication-type]
Journal Article; Review
[Publication-country]
United States
[Chemical-registry-number]
0 / Biomarkers, Tumor
[Number-of-references]
75
61.
Hashizume R, Gupta N, Berger MS, Banerjee A, Prados MD, Ayers-Ringler J, James CD, VandenBerg SR:
Morphologic and molecular characterization of ATRT xenografts adapted for orthotopic therapeutic testing.
Neuro Oncol
; 2010 Apr;12(4):366-76
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Atypical teratoid rhabdoid tumor
(ATRT) is a
malignant
tumor of
the
central nervous system
that most commonly arises in young children.
When established as orthotopic xenografts, the tumors predominantly display cells with a
rhabdoid
-like cellular morphology that show a spectrum of immunophenotypes similar to primary ATRT tumors.
These data suggest that an orthotopic ATRT xenograft model, in which BLI is used for monitoring
tumor
growth and response to therapy, should contribute to the identification of effective therapeutics and regimens for treating this highly aggressive pediatric brain
tumor
.
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[ISSN]
1523-5866
[Journal-full-title]
Neuro-oncology
[ISO-abbreviation]
Neuro-oncology
[Language]
ENG
[Grant]
United States / NCI NIH HHS / CA / P50CA097257
[Publication-type]
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
[Publication-country]
England
[Chemical-registry-number]
0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide
[Other-IDs]
NLM/ PMC2940601
62.
Varan A, Akalan N, Söylemezoğlu F, Zorlu F, Yalçin B, Akyüz C, Kutluk T, Büyükpamukçu M:
Central nervous system tumors in patients under three years of age: treatment results of a single institute.
Pediatr Neurosurg
; 2006;42(2):89-94
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[Title]
Central nervous system
tumors in patients under three years of age: treatment results of a single institute.
Eighty-six patients under 3 years of age with
central nervous system
tumors were retrospectively analyzed between 1972 and 2003.
Surgical resection was done in all patients except for those with optic glioma, pons glioma and pineal
tumor
.
In 48 patients, the
tumor
was located in the posterior fossa, and 29 patients had a supratentorial
tumor
.
We had 32 (37.2%) patients with embryonic tumors (21 medulloblastoma, 4 ependymoblastoma, 5 with
atypical teratoid rhabdoid
and 2 with supratentorial primitive neuroectodermal tumors), 21 (24.4%) with ependymoma, 14 (16.3%) with optic glioma, 10 (11.6%) with astrocytoma, 3 (3.5%) with pons glioma and 6 (7.0%) with others.
OS rates according to the
tumor
localizations were 40.9 and 68.1% in the posterior fossa and supratentorial localizations, respectively (p=0.001).
OS rates were 33.7, 41.3 and 88.8% for the medulloblastoma+primitive neuroectodermal
tumor
groups, ependymoma and astrocytoma, respectively (p=0.0001).
The best prognostic factors were
tumor
localization and histology.
[MeSH-major]
Central Nervous System
Neoplasms / mortality.
Central Nervous System
Neoplasms / therapy. Neoplasms, Germ Cell and Embryonal / mortality. Neoplasms, Germ Cell and Embryonal / therapy
[MeSH-minor]
Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child, Preschool. Female. Humans. Infant. Infant, Newborn. Male. Prognosis. Retrospective Studies.
Rhabdoid Tumor
/ mortality.
Rhabdoid Tumor
/ pathology.
Rhabdoid Tumor
/ therapy. Survival Rate
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[Copyright]
Copyright (c) 2006 S. Karger AG, Basel.
(PMID = 16465077.001).
[ISSN]
1016-2291
[Journal-full-title]
Pediatric neurosurgery
[ISO-abbreviation]
Pediatr Neurosurg
[Language]
eng
[Publication-type]
Journal Article
[Publication-country]
Switzerland
63.
Judkins AR, Ellison DW:
Ependymoblastoma: dear, damned, distracting diagnosis, farewell!*.
Brain Pathol
; 2010 Jan;20(1):133-9
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[Title]
Ependymoblastoma: dear, damned, distracting
diagnosis
, farewell!*.
Ependymoblastoma is a diagnostic label that has been applied to a variety of rare
central nervous system
(
CNS
) tumors over the last eight decades.
Three other cases showed rare ependymoblastic rosettes in the histopathological setting of a typical primitive neuroectodermal
tumor
(PNET), medulloblastoma (MB) or
atypical teratoid
/
rhabdoid tumor
(AT/RT).
Our results indicate that ependymoblastic rosettes are most frequently encountered in embryonal tumors with abundant neuropil and less frequently in other
CNS
embryonal neoplasms, including PNET, MB and AT/RT.
We believe that ependymoblastoma as a
diagnosis
is neither precise nor specific and that it is time once and for all to retire this
diagnosis
from the lexicon of neuropathology.
[MeSH-major]
Central Nervous System
Neoplasms /
diagnosis
.
Central Nervous System
Neoplasms / pathology. Neuroectodermal Tumors, Primitive /
diagnosis
. Neuroectodermal Tumors, Primitive / pathology
[MeSH-minor]
Cell Differentiation / physiology. Child. Child, Preschool. Female. Ganglia / cytology. Humans. Immunohistochemistry. Infant. Male. Medulloblastoma / pathology. Neoplasms, Germ Cell and Embryonal / pathology. Neurons / physiology. Neuropil / pathology.
Rhabdoid Tumor
/ pathology. Rosette Formation. Sex Factors. Teratoma / pathology. Terminology as Topic
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(PMID = 19120373.001).
[ISSN]
1750-3639
[Journal-full-title]
Brain pathology (Zurich, Switzerland)
[ISO-abbreviation]
Brain Pathol.
[Language]
eng
[Publication-type]
Journal Article
[Publication-country]
Switzerland
64.
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
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[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
65.
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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[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
66.
Gyure KA:
Newly defined central nervous system neoplasms.
Am J Clin Pathol
; 2005 Jun;123 Suppl:S3-12
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[Title]
Newly defined
central nervous system
neoplasms.
In recent years, numerous new entities or variants of recognized
central nervous system
tumors have been described in the literature, and the morphologic spectrum of these neoplasms is delineated incompletely.
The accurate
diagnosis
and classification of these lesions is important to ensure that patients receive adequate therapy and prognostic information.
The clinicopathologic features and differential
diagnosis
of 4 new entities, including the chordoid glioma of the third ventricle, cerebellar liponeurocytoma,
atypical teratoid
/
rhabdoid tumor
, and papillary glioneuronal
tumor
, are discussed in this review.
[MeSH-major]
Central Nervous System
Neoplasms / classification.
Central Nervous System
Neoplasms /
diagnosis
[MeSH-minor]
Adult. Cerebellar Neoplasms / classification. Cerebellar Neoplasms /
diagnosis
. Child. Chordoma / classification. Chordoma /
diagnosis
.
Diagnosis
, Differential. Female. Ganglioglioma / classification. Ganglioglioma /
diagnosis
. Glioma /
diagnosis
. Glioma / pathology. Humans. Hypothalamic Neoplasms / classification. Hypothalamic Neoplasms /
diagnosis
. Male. Medulloblastoma / classification. Medulloblastoma /
diagnosis
. Prognosis.
Rhabdoid Tumor
/ classification.
Rhabdoid Tumor
/
diagnosis
. Teratoma / classification. Teratoma /
diagnosis
. Third Ventricle / pathology
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(PMID = 16100866.001).
[ISSN]
0002-9173
[Journal-full-title]
American journal of clinical pathology
[ISO-abbreviation]
Am. J. Clin. Pathol.
[Language]
eng
[Publication-type]
Journal Article; Review
[Publication-country]
United States
[Number-of-references]
81
67.
El-Nabbout B, Shbarou R, Glasier CM, Saad AG:
Primary diffuse cerebral leptomeningeal atypical teratoid rhabdoid tumor: report of the first case.
J Neurooncol
; 2010 Jul;98(3):431-4
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[Title]
Primary diffuse cerebral leptomeningeal
atypical teratoid rhabdoid tumor
: report of the first case.
Atypical teratoid rhabdoid tumor
(AT/RT) of the
central nervous system
has been recently described as a distinct clinicopathological entity with characteristic morphologic, immunophenotypic and molecular characteristics.
[MeSH-major]
Central Nervous System
Neoplasms / pathology. Meningeal Neoplasms / pathology.
Rhabdoid Tumor
/ pathology
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[Cites]
Neurosurgery. 1995 Jan;36(1):166-8; discussion 169
[
7708153.001
]
[Cites]
Can J Neurol Sci. 1985 Aug;12 (3):278-81
[
4052890.001
]
[Cites]
Acta Neurochir (Wien). 2004 Sep;146(9):1033-8; discussion 1038
[
15340816.001
]
[Cites]
J Neurosurg. 1986 Jun;64(6):968-73
[
3701447.001
]
[Cites]
J Neurooncol. 1996 Jul;29(1):75-84
[
8817418.001
]
[Cites]
Am J Surg Pathol. 1998 Sep;22(9):1083-92
[
9737241.001
]
[Cites]
J Neurosurg. 1985 Aug;63(2):283-7
[
4020450.001
]
(PMID = 20020178.001).
[ISSN]
1573-7373
[Journal-full-title]
Journal of neuro-oncology
[ISO-abbreviation]
J. Neurooncol.
[Language]
eng
[Publication-type]
Case Reports; Journal Article
[Publication-country]
United States
68.
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.
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
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[Copyright]
Copyright (c) 2008 American Cancer Society.
(PMID = 18224664.001).
[ISSN]
0008-543X
[Journal-full-title]
Cancer
[ISO-abbreviation]
Cancer
[Language]
eng
[Grant]
United States / NCI NIH HHS / CA / R25 CA023944; United States / NCI NIH HHS / CA / CA 21765
[Publication-type]
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
[Publication-country]
United States
69.
Janson K, Nedzi LA, David O, Schorin M, Walsh JW, Bhattacharjee M, Pridjian G, Tan L, Judkins AR, Biegel JA:
Predisposition to atypical teratoid/rhabdoid tumor due to an inherited INI1 mutation.
Pediatr Blood Cancer
; 2006 Sep;47(3):279-84
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[Title]
Predisposition to
atypical teratoid
/
rhabdoid tumor
due to an inherited INI1 mutation.
BACKGROUND: Germline mutations of the INI1 gene predispose children to the development
of rhabdoid
tumors.
PROCEDURE: We have identified a three-generation family in which two half-brothers were diagnosed with
central nervous system atypical teratoid
/
rhabdoid
tumors (AT/RT).
A maternal uncle died in childhood from a brain
tumor
and a
malignant
rhabdoid tumor
of the kidney, and presumably carried the same germline mutation.
CONCLUSION: The identification of two unaffected carriers in a family segregating a germline mutation and
rhabdoid tumor
supports the hypothesis that there may be variable risks of development
of rhabdoid tumor
in the context of a germline mutation.
There may be a developmental window in which most
rhabdoid
tumors occur.
This family highlights the importance of mutation analysis in all patients with a suspected
rhabdoid tumor
.
[MeSH-major]
Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chromosomal Proteins, Non-Histone / genetics. DNA-Binding Proteins / genetics. Genetic Predisposition to Disease.
Rhabdoid Tumor
/ genetics. Teratoma / genetics. Transcription Factors / genetics
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[CommentIn]
Pediatr Blood Cancer. 2006 Sep;47(3):343-4
[
16609948.001
]
[CommentIn]
Pediatr Blood Cancer. 2006 Sep;47(3):235-7
[
16304667.001
]
(PMID = 16261613.001).
[ISSN]
1545-5009
[Journal-full-title]
Pediatric blood & cancer
[ISO-abbreviation]
Pediatr Blood Cancer
[Language]
eng
[Grant]
United States / NCI NIH HHS / CA / CA46274; United States / NCI NIH HHS / CA / CA98543
[Publication-type]
Case Reports; Comparative Study; Journal Article; Research Support, N.I.H., Extramural
[Publication-country]
United States
[Chemical-registry-number]
0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / SMARCB1 protein, human; 0 / Transcription Factors
70.
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
71.
Takei H, Bhattacharjee MB, Rivera A, Dancer Y, Powell SZ:
New immunohistochemical markers in the evaluation of central nervous system tumors: a review of 7 selected adult and pediatric brain tumors.
Arch Pathol Lab Med
; 2007 Feb;131(2):234-41
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[Title]
New immunohistochemical markers in the evaluation
of central nervous system
tumors: a review of 7 selected adult and pediatric brain tumors.
CONTEXT: Immunohistochemistry (IHC) has become an important tool in the
diagnosis
of brain tumors.
DATA SYNTHESIS: We review the features of new, useful or potentially applicable marker antibodies as well as the new uses of already established antibodies in the area of diagnostic neuro-oncologic pathology, focusing on the use of IHC for differential
diagnosis
and prognosis.
We discuss (1) placental alkaline phosphatase, c-Kit, and OCT4 for germinoma, (2) alpha-inhibin and D2-40 for capillary hemangioblastoma, (3) phosphohistone-H3 (PHH3), MIB-1/Ki-67, and claudin-1 for meningioma, (4) PHH3, MIB-1/Ki-67, and p53 for astrocytoma, (5) synaptophysin, microtubule-associated protein 2, neurofilament protein, and neuronal nuclei for medulloblastoma, (6) INI1 for
atypical teratoid
/
rhabdoid tumor
, and (7) epithelial membrane antigen for ependymoma.
All the markers presented here are used mainly for supporting or confirming the
diagnosis
, with the exception of the proliferation markers (MIB-1/Ki-67 and PHH3), which are primarily used to support grading and are reportedly associated with prognosis in certain categories of brain tumors.
CONCLUSIONS: Although conventional hematoxylin-eosin staining is the mainstay for pathologic
diagnosis
, IHC has played a major role in differential
diagnosis
and in improving diagnostic accuracy not only in general surgical pathology but also in neuro-oncologic pathology.
[MeSH-major]
Biomarkers,
Tumor
/ analysis.
Central Nervous System
Neoplasms /
diagnosis
.
Central Nervous System
Neoplasms / metabolism. Immunohistochemistry
[MeSH-minor]
Adult. Antibodies. Astrocytoma /
diagnosis
. Astrocytoma / metabolism. Child.
Diagnosis
, Differential. Ependymoma /
diagnosis
. Ependymoma / metabolism. Germinoma /
diagnosis
. Germinoma / metabolism. Hemangioblastoma /
diagnosis
. Hemangioblastoma / metabolism. Humans. Medulloblastoma /
diagnosis
. Medulloblastoma / metabolism. Meningioma /
diagnosis
. Meningioma / metabolism.
Rhabdoid Tumor
/
diagnosis
.
Rhabdoid Tumor
/ metabolism
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(PMID = 17284108.001).
[ISSN]
1543-2165
[Journal-full-title]
Archives of pathology & laboratory medicine
[ISO-abbreviation]
Arch. Pathol. Lab. Med.
[Language]
eng
[Publication-type]
Journal Article; Review
[Publication-country]
United States
[Chemical-registry-number]
0 / Antibodies; 0 / Biomarkers, Tumor
[Number-of-references]
96
72.
Behdad A, Perry A:
Central nervous system primitive neuroectodermal tumors: a clinicopathologic and genetic study of 33 cases.
Brain Pathol
; 2010 Mar;20(2):441-50
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[Title]
Central nervous system
primitive neuroectodermal tumors: a clinicopathologic and genetic study of 33 cases.
Central nervous system
(
CNS
) primitive neuroectodermal tumors (PNETs) include supratentorial, brain stem, and spinal cord tumors with medulloblastoma-like histopathology.
After re-
diagnosis
of three infantile cases as
atypical teratoid
/
rhabdoid tumor
(AT/RT), 33 remaining
CNS
PNETs were retrieved for clinicopathologic and fluorescence in situ hybridization studies.
We conclude that in
CNS
PNET: (i) routine application of INI1 immunohistochemistry helps rule out AT/RT, particularly in infants;.
(iii) involvement of
CNS
parenchyma by Ewing sarcoma/peripheral PNET is rare enough that EWS gene testing is not necessary unless significant dural involvement is present; and (iv) both anaplastic/large cell features and polysomies of 2 and 8 are associated with more aggressive clinical behavior.
[MeSH-minor]
Adolescent. Adult. Aneuploidy. Child. Child, Preschool. Chromosomes, Human, Pair 2. Chromosomes, Human, Pair 22. Chromosomes, Human, Pair 8. Female. Humans. Infant. Male. Middle Aged. Nuclear Proteins / genetics. Oncogene Proteins / genetics. RNA-Binding Protein EWS / genetics.
Rhabdoid Tumor
/
diagnosis
.
Rhabdoid Tumor
/ genetics.
Rhabdoid Tumor
/ pathology. Teratoma /
diagnosis
. Teratoma / genetics. Teratoma / pathology. Young Adult
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(PMID = 19725831.001).
[ISSN]
1750-3639
[Journal-full-title]
Brain pathology (Zurich, Switzerland)
[ISO-abbreviation]
Brain Pathol.
[Language]
eng
[Publication-type]
Journal Article
[Publication-country]
Switzerland
[Chemical-registry-number]
0 / MYCN protein, human; 0 / Nuclear Proteins; 0 / Oncogene Proteins; 0 / RNA-Binding Protein EWS
73.
Woehrer A, Slavc I, Waldhoer T, Heinzl H, Zielonke N, Czech T, Benesch M, Hainfellner JA, Haberler C, Austrian Brain Tumor Registry:
Incidence of atypical teratoid/rhabdoid tumors in children: a population-based study by the Austrian Brain Tumor Registry, 1996-2006.
Cancer
; 2010 Dec 15;116(24):5725-32
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[Title]
Incidence
of atypical teratoid
/
rhabdoid
tumors in children: a population-based study by the Austrian Brain
Tumor
Registry, 1996-2006.
BACKGROUND:
Atypical teratoid
/
rhabdoid
tumors are highly
malignant
embryonal
central nervous system
(
CNS
) tumors that were defined as an entity in 1996.
As compared with other
malignant CNS
tumors, their biological behavior is particularly aggressive, but patients may benefit from an intensified treatment.
Atypical teratoid
/
rhabdoid
tumors display a complex histomorphology, which renders them prone to misdiagnosis.
They occur predominantly in young children, with an estimated prevalence of 1% to 2% among all pediatric
CNS
tumors.
METHODS: A nation-wide survey of
malignant
high-grade
CNS
tumors (World Health Organization grade III/IV), diagnosed in children (aged birth to 14 years) from 1996 to 2006 was conducted by the Austrian Brain
Tumor
Registry.
A
central
histopathology review was performed including the assessment of SMARCB1 (INI1) protein status.
RESULTS: A total of 311 newly diagnosed,
malignant CNS
tumors were included.
Atypical teratoid
/
rhabdoid
tumors constituted the sixth most common entity (6.1%), referring to an age-standardized incidence rate of 1.38 per 1,000,000 person-years in children.
Peak incidence was found in the birth to 2 years age group, where they were as common as
CNS
primitive neuroectodermal tumors and medulloblastomas.
A total of 47.4%
of atypical teratoid
/
rhabdoid
tumors were initially diagnosed, whereas 52.6% were retrospectively detected by the
central
review.
The 5-year survival
of atypical teratoid
/
rhabdoid tumor
patients was 39.5%, with 66.7% in the correctly diagnosed group versus 15.0% in the not recognized group (P = .0469).
CONCLUSIONS: Clinicians and pathologists should be aware of the high incidence
of atypical teratoid
/
rhabdoid
tumors in young children to optimize diagnostic and therapeutic management of patients with these tumors.
[MeSH-major]
Brain Neoplasms / epidemiology.
Rhabdoid Tumor
/ epidemiology. Teratoma / epidemiology
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[Copyright]
Copyright © 2010 American Cancer Society.
(PMID = 20737418.001).
[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
[Investigator]
Azizi AA; Scarpatetti M; Ebetsberger G; Weis S; Jones N; Klein-Franke A; Sterlacci W; Jauk B; Kiefer A; Mueller G; Gruber-Moesenbacher U; Reiner-Concin A; Feichtinger H
74.
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
75.
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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.
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.
Hazardous Substances Data Bank.
CYTARABINE
.
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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
76.
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
77.
Moeller KK, Coventry S, Jernigan S, Moriarty TM:
Atypical teratoid/rhabdoid tumor of the spine.
AJNR Am J Neuroradiol
; 2007 Mar;28(3):593-5
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[Title]
Atypical teratoid
/
rhabdoid tumor
of the spine.
SUMMARY:
Atypical teratoid
/
rhabdoid tumor
(AT/RT) is a highly
malignant
central nervous system
neoplasm
usually seen in young children and infants.
[MeSH-major]
Magnetic Resonance Imaging.
Rhabdoid Tumor
/ pathology. Spinal Neoplasms / pathology. Teratoma / pathology
[MeSH-minor]
Child.
Diagnosis
, Differential. Humans. Lumbar Vertebrae / pathology. Male. Thoracic Vertebrae / pathology
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.
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.
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.
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(PMID = 17353344.001).
[ISSN]
0195-6108
[Journal-full-title]
AJNR. American journal of neuroradiology
[ISO-abbreviation]
AJNR Am J Neuroradiol
[Language]
eng
[Publication-type]
Case Reports; Journal Article
[Publication-country]
United States
78.
MacDonald TJ:
Aggressive infantile embryonal tumors.
J Child Neurol
; 2008 Oct;23(10):1195-204
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Histologically characterized as undifferentiated small, round cell tumors with divergent patterns of differentiation, these include medulloblastoma, the most common form of embryonal
tumor
, as well as supratentorial primitive neuroectodermal
tumor
, medulloepithelioma, ependymoblastoma, medullomyoblastoma, melanotic medulloblastoma, and
atypical teratoid
/
rhabdoid tumor
.
All are similarly aggressive and have a tendency to disseminate throughout the
central nervous system
.
Outcomes remain similarly poor among all the
tumor
types and, therefore, identification of specific molecular targets that have prognostic and therapeutic implications is crucial.
The molecular and clinical aspects of the 3 most common aggressive infantile embryonal tumors, medulloblastoma, supratentorial primitive neuroectodermal
tumor
, and
atypical teratoid
/
rhabdoid tumor
, are the focus of this review.
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[ISSN]
1708-8283
[Journal-full-title]
Journal of child neurology
[ISO-abbreviation]
J. Child Neurol.
[Language]
ENG
[Grant]
United States / NINDS NIH HHS / NS / R13 NS040925; United States / NINDS NIH HHS / NS / 5R13NS040925-09
[Publication-type]
Journal Article; Research Support, N.I.H., Extramural; Review
[Publication-country]
United States
[Chemical-registry-number]
0 / Biomarkers, Tumor
[Number-of-references]
70
[Other-IDs]
NLM/ NIHMS473079; NLM/ PMC3674573
79.
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
80.
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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[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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[Cites]
Clin Neuropathol. 2006 Mar-Apr;25(2):81-5
[
16550741.001
]
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[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
81.
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
82.
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.
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.
[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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.
NCI CPTC Antibody Characterization Program.
NCI CPTC Antibody Characterization Program
.
NCI CPTC Antibody Characterization Program.
NCI CPTC Antibody Characterization Program
.
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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
83.
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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[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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.
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.
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