rhabdoid tumor

Summary

Summary: A rare but highly lethal childhood tumor found almost exclusively in infants. Histopathologically, it resembles RHABDOMYOSARCOMA but the tumor cells are not of myogenic origin. Although it arises primarily in the kidney, it may be found in other parts of the body. The rhabdoid cytomorphology is believed to be the expression of a very primitive malignant cell. (From Holland et al., Cancer Medicine, 3d ed, p2210)

Top Publications

  1. ncbi Truncating mutations of hSNF5/INI1 in aggressive paediatric cancer
    I Versteege
    Laboratoire de Pathologie Moléculaire des Cancers, Section de Recherche, Institut Curie, Paris, France
    Nature 394:203-6. 1998
  2. pmc A remarkably simple genome underlies highly malignant pediatric rhabdoid cancers
    Ryan S Lee
    Department of Pediatric Oncology, Dana Farber Cancer Institute, Boston, MA, USA
    J Clin Invest 122:2983-8. 2012
  3. pmc Loss of the tumor suppressor Snf5 leads to aberrant activation of the Hedgehog-Gli pathway
    Zainab Jagani
    Novartis Institutes for BioMedical Research, Cambridge, Massachusetts, USA
    Nat Med 16:1429-33. 2010
  4. ncbi High-resolution genomic analysis suggests the absence of recurrent genomic alterations other than SMARCB1 aberrations in atypical teratoid/rhabdoid tumors
    Martin Hasselblatt
    Institute of Neuropathology, University Hospital Munster, Munster, Germany
    Genes Chromosomes Cancer 52:185-90. 2013
  5. ncbi Frequent hSNF5/INI1 germline mutations in patients with rhabdoid tumor
    Franck Bourdeaut
    CHU Nantes, Service d hémato oncologie pédiatrique, Nantes, France
    Clin Cancer Res 17:31-8. 2011
  6. pmc Small cell undifferentiated variant of hepatoblastoma: adverse clinical and molecular features similar to rhabdoid tumors
    Angela D Trobaugh-Lotrario
    Department of Hematology Oncology, Sacred Heart Children s Hospital, Spokane, Washington 99204, USA
    Pediatr Blood Cancer 52:328-34. 2009
  7. ncbi Germ-line and acquired mutations of INI1 in atypical teratoid and rhabdoid tumors
    J A Biegel
    The Children s Hospital of Philadelphia, and Department of Pediatrics, University of Pennsylvania School of Medicine, 19104, USA
    Cancer Res 59:74-9. 1999
  8. ncbi Survival outcomes in atypical teratoid rhabdoid tumor for patients undergoing radiotherapy in a Surveillance, Epidemiology, and End Results analysis
    Daniela L Buscariollo
    Vanderbilt University School of Medicine, Nashville, Tennessee, USA
    Cancer 118:4212-9. 2012
  9. ncbi INI1-deficient tumors: diagnostic features and molecular genetics
    Travis J Hollmann
    Department of Pathology, Brigham and Women s Hospital, Harvard Medical School, Boston, MA, USA
    Am J Surg Pathol 35:e47-63. 2011
  10. ncbi A novel case of rhabdoid colon carcinoma associated with a positive CpG island methylator phenotype and BRAF mutation
    Massimo Pancione
    Department of Biological and Environmental Sciences, University of Sannio, 82100 Benevento, Italy
    Hum Pathol 42:1047-52. 2011

Research Grants

  1. Role of hsnf5/BAF47 Loss in Human Cancer Development
    Bernard E Weissman; Fiscal Year: 2012
  2. GENETICS OF PEDIATRIC RHABDOID TUMORS
    Jaclyn A Biegel; Fiscal Year: 2012
  3. NATIONAL WILMS TUMOR STUDY -5
    Daniel Green; Fiscal Year: 2003
  4. TUMOR SUPPRESSORS AND IMPRINTING AT CHROMOSOME 11P155
    Michael Higgins; Fiscal Year: 2000
  5. RHABDOID TUMOR GENE
    Deborah Schofield; Fiscal Year: 2000
  6. ISOLATION OF THE NEUROBLASTOMA PREDISPOSITION GENE
    John Maris; Fiscal Year: 2006
  7. PEDIATRIC BRAIN TUMOR RESEARCH CENTER
    Susan Blaney; Fiscal Year: 2003
  8. SWI/SNF Chromatin Remodeling Loss and Human Cancer
    Bernard Weissman; Fiscal Year: 2007
  9. MOLECULAR ANALYSIS OF FANCONI'S ANEMIA C PROTEIN
    Sharon Plon; Fiscal Year: 2003
  10. Xenogen's IVIS 100 Imaging System
    Mark Kieran; Fiscal Year: 2004

Detail Information

Publications266 found, 100 shown here

  1. ncbi Truncating mutations of hSNF5/INI1 in aggressive paediatric cancer
    I Versteege
    Laboratoire de Pathologie Moléculaire des Cancers, Section de Recherche, Institut Curie, Paris, France
    Nature 394:203-6. 1998
    ..The observation of bi-allelic alterations of hSNF5/INI1 in MRTs suggests that loss-of-function mutations of hSNF5/INI1 contribute to oncogenesis...
  2. pmc A remarkably simple genome underlies highly malignant pediatric rhabdoid cancers
    Ryan S Lee
    Department of Pediatric Oncology, Dana Farber Cancer Institute, Boston, MA, USA
    J Clin Invest 122:2983-8. 2012
    ..Our results demonstrate that high mutation rates are dispensable for the genesis of cancers driven by mutation of a chromatin remodeling complex. Consequently, cancer can be a remarkably genetically simple disease...
  3. pmc Loss of the tumor suppressor Snf5 leads to aberrant activation of the Hedgehog-Gli pathway
    Zainab Jagani
    Novartis Institutes for BioMedical Research, Cambridge, Massachusetts, USA
    Nat Med 16:1429-33. 2010
    ..Therefore, our studies reveal that SNF5 is a key mediator of Hh signaling and that aberrant activation of GLI1 is a previously undescribed targetable mechanism contributing to the growth of MRT cells...
  4. ncbi High-resolution genomic analysis suggests the absence of recurrent genomic alterations other than SMARCB1 aberrations in atypical teratoid/rhabdoid tumors
    Martin Hasselblatt
    Institute of Neuropathology, University Hospital Munster, Munster, Germany
    Genes Chromosomes Cancer 52:185-90. 2013
    Atypical teratoid/rhabdoid tumor (AT/RT) is a rare malignant pediatric brain tumor characterized by genetic alterations affecting the SMARCB1 (hSNF5/INI1) locus in chromosome band 22q11.2...
  5. ncbi Frequent hSNF5/INI1 germline mutations in patients with rhabdoid tumor
    Franck Bourdeaut
    CHU Nantes, Service d hémato oncologie pédiatrique, Nantes, France
    Clin Cancer Res 17:31-8. 2011
    ..Our study provides the first precise overview of the prevalence of RPS within a large cohort of RT...
  6. pmc Small cell undifferentiated variant of hepatoblastoma: adverse clinical and molecular features similar to rhabdoid tumors
    Angela D Trobaugh-Lotrario
    Department of Hematology Oncology, Sacred Heart Children s Hospital, Spokane, Washington 99204, USA
    Pediatr Blood Cancer 52:328-34. 2009
    ..Small cell undifferentiated (SCU) histology in patients with stage I hepatoblastoma (HB) predicts an increased risk of relapse. We sought to determine the significance of SCU histology in patients with unresectable HB...
  7. ncbi Germ-line and acquired mutations of INI1 in atypical teratoid and rhabdoid tumors
    J A Biegel
    The Children s Hospital of Philadelphia, and Department of Pediatrics, University of Pennsylvania School of Medicine, 19104, USA
    Cancer Res 59:74-9. 1999
    ..and rhabdoid tumors of the brain and 7 renal and 4 extrarenal rhabdoid tumors for mutations in the candidate rhabdoid tumor suppressor gene, INI1...
  8. ncbi Survival outcomes in atypical teratoid rhabdoid tumor for patients undergoing radiotherapy in a Surveillance, Epidemiology, and End Results analysis
    Daniela L Buscariollo
    Vanderbilt University School of Medicine, Nashville, Tennessee, USA
    Cancer 118:4212-9. 2012
    Atypical teratoid rhabdoid tumor (ATRT) is a rare central nervous system malignancy with a poor prognosis that affects mostly young children...
  9. ncbi INI1-deficient tumors: diagnostic features and molecular genetics
    Travis J Hollmann
    Department of Pathology, Brigham and Women s Hospital, Harvard Medical School, Boston, MA, USA
    Am J Surg Pathol 35:e47-63. 2011
    ..which encodes a subunit of the SWI/SNF chromatin remodeling complex, were first described in the malignant rhabdoid tumor of infancy...
  10. ncbi A novel case of rhabdoid colon carcinoma associated with a positive CpG island methylator phenotype and BRAF mutation
    Massimo Pancione
    Department of Biological and Environmental Sciences, University of Sannio, 82100 Benevento, Italy
    Hum Pathol 42:1047-52. 2011
    ..This is the first case of a colorectal carcinoma with rhabdoid features, "CpG island methylator phenotype," high microsatellite instability associated with a BRAF mutation, and patient poorer outcome...
  11. pmc Identification of CD133-positive radioresistant cells in atypical teratoid/rhabdoid tumor
    Shih Hwa Chiou
    Department of Medical Research and Education, Taipei Veterans General Hospital and National Yang Ming University, Taipei, Taiwan
    PLoS ONE 3:e2090. 2008
    Atypical teratoid/rhabdoid tumor (AT/RT) is an extremely malignant neoplasm in the central nervous system (CNS) which occurs in infancy and childhood...
  12. ncbi Childhood atypical teratoid rhabdoid tumor of the central nervous system: a meta-analysis of observational studies
    Uma H Athale
    Division of Hematology Oncology, Department of Pediatrics, McMaster University, Hamilton, Canada
    J Pediatr Hematol Oncol 31:651-63. 2009
    Therapy for central nervous system (CNS) atypical teratoid rhabdoid tumor (ATRT) is controversial. We describe 4 children treated with sarcoma-like therapy and review the literature to evaluate outcome in relation to treatment modalities.
  13. pmc The histone deacetylase inhibitor SAHA acts in synergism with fenretinide and doxorubicin to control growth of rhabdoid tumor cells
    Kornelius Kerl
    Department of Pediatric Hematology and Oncology, University Childrens Hospital Muenster, Muenster, Germany
    BMC Cancer 13:286. 2013
    ..Rhabdoid tumors are highly aggressive malignancies affecting infants and very young children. In many instances these tumors are resistant to conventional type chemotherapy necessitating alternative approaches...
  14. pmc Reexpression of hSNF5 in malignant rhabdoid tumor cell lines causes cell cycle arrest through a p21(CIP1/WAF1)-dependent mechanism
    Yasumichi Kuwahara
    Department of Pathology and Laboratory Medicine, UNC Lineberger Comprehensive Cancer Center, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599 7295, USA
    Cancer Res 70:1854-65. 2010
    Loss of hSNF5 function is usually observed in malignant rhabdoid tumor (MRT), a highly aggressive pediatric neoplasm...
  15. ncbi Ependymoblastoma: dear, damned, distracting diagnosis, farewell!*
    Alexander R Judkins
    Division of Neuropathology, Department of Pathology and Laboratory Medicine, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Brain Pathol 20:133-9. 2010
    ..setting of a typical primitive neuroectodermal tumor (PNET), medulloblastoma (MB) or atypical teratoid/rhabdoid tumor (AT/RT). The remaining cases were all embryonal tumors with structures that mimicked ependymoblastic rosettes...
  16. pmc Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor
    Susan N Chi
    Dana Farber Cancer Institute, Children s Hospital Boston, Boston, MA, USA
    J Clin Oncol 27:385-9. 2009
    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...
  17. ncbi Atypical teratoid/rhabdoid tumors (ATRT): improved survival in children 3 years of age and older with radiation therapy and high-dose alkylator-based chemotherapy
    Tanya M Tekautz
    Department of Hematology Oncology, Mail Stop 260, St Jude Children s Research Hospital, 332 N Lauderdale St, Memphis, TN 38105, USA
    J Clin Oncol 23:1491-9. 2005
    ..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)...
  18. ncbi Disabling c-Myc in childhood medulloblastoma and atypical teratoid/rhabdoid tumor cells by the potent G-quadruplex interactive agent S2T1-6OTD
    Tarek Shalaby
    Department of Oncology, University Children s Hospital Zurich, Zurich, Switzerland
    Mol Cancer Ther 9:167-79. 2010
    ..On in vivo animal testing, S2T1-6OTD may well represent a novel therapeutic strategy for childhood brain tumors...
  19. ncbi Malignant rhabdoid tumours of the kidney (MRTKs), registered on recent SIOP protocols from 1993 to 2005: a report of the SIOP renal tumour study group
    Marry M van den Heuvel-Eibrink
    Department of Pediatric Oncology Hematology, Erasmus MC Sophia Children s Hospital, Rotterdam, The Netherlands
    Pediatr Blood Cancer 56:733-7. 2011
    ..Survival data of malignant rhabdoid tumour of the kidney (MRTK) registered in SIOP trials, advocating preoperative chemotherapy, are not available...
  20. pmc Mutation of the INI1 gene in composite rhabdoid tumor of the endometrium
    Ludvik R Donner
    Department of Pathology, Scott and White Memorial Hospital and Clinic, Scott, Sherwood and Brindley Foundation, Texas A and M University Health Science Center College of Medicine, Temple, TX 76508, USA
    Hum Pathol 38:935-9. 2007
    ..that contain a component of rhabdoid cells, which are characteristic of the aggressive childhood malignant rhabdoid tumor. Pediatric rhabdoid tumors are characterized by the inactivation of the hSNF5/INI1/SMARCB1 gene, with ..
  21. ncbi Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry
    Joanne M Hilden
    Department of Pediatric Hematology Oncology, The Children s Hospital, The Cleveland Clinic, 9500 Euclid Avenue, Desk S20, Cleveland, OH 44195, USA
    J Clin Oncol 22:2877-84. 2004
    Atypical teratoid/rhabdoid tumor (AT/RT) of the CNS is an extremely rare and aggressive tumor of early childhood. The poor outcome with conventional infant brain tumor therapy has resulted in a lack of clear treatment guidelines...
  22. ncbi Rhabdoid carcinoma of the colon: a distinct entity with a very aggressive behavior: a case report associated with a polyposis coli and review of the literature
    Andrea Remo
    Department of Pathology, Mater Salutis Hospital, ULSS 21 Legnago, Verona, Italy
    Int J Surg Pathol 20:185-90. 2012
    ..It is argued that RCT could be a very aggressive entity of colon, which could benefit from new biological colonic treatments...
  23. ncbi Inhibitors of histone deacetylases as potential therapeutic tools for high-risk embryonal tumors of the nervous system of childhood
    Sarah E Furchert
    Department of Pediatric Hematology and Oncology, University Children s Hospital Muenster, Muenster, Germany
    Int J Cancer 120:1787-94. 2007
    ..Treatment with HDI induced the reactivation of growth regulatory genes and consequently apoptosis. Our results warrant further studies and may help in the design of new protocols geared at the treatment of high risk embryonal tumors...
  24. ncbi Atypical teratoid or rhabdoid tumors: improved outcome with high-dose chemotherapy
    Tal Finkelstein-Shechter
    Bone Marrow Transplant Program, Hospital for Sick Children, Toronto, Canada
    J Pediatr Hematol Oncol 32:e182-6. 2010
    ..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...
  25. pmc High expression of BMP pathway genes distinguishes a subset of atypical teratoid/rhabdoid tumors associated with shorter survival
    Diane K Birks
    University of Colorado, Denver Anschutz Medical Campus, Department of Neurosurgery, Aurora, CO 80045, USA
    Neuro Oncol 13:1296-307. 2011
    ..Our study indicates that molecular subgroups exist in AT/RTs and that molecular profiling of these comparatively rare tumors may be of diagnostic, prognostic, and therapeutic value...
  26. ncbi A unique occurrence of a cerebral atypical teratoid/rhabdoid tumor in an infant and a spinal canal primitive neuroectodermal tumor in her father
    Ewa Izycka-Swieszewska
    Department of Pathology, Medical University of Gdansk, Poland
    J Neurooncol 61:219-25. 2003
    ..genetic data of two rare malignant neoplasms of the central nervous system (CNS)--a cerebral atypical teratoid/rhabdoid tumor (AT/RT) in a 5-month-old girl and a spinal canal primitive neuroectodermal tumor (PNET) in her father...
  27. ncbi Establishment of atypical-teratoid/rhabdoid tumor (AT/RT) cell cultures from disseminated CSF cells: a model to elucidate biology and potential targeted therapeutics
    Aru Narendran
    Translational Research Laboratories, Southern Alberta Children s Cancer Program, The University of Calgary, Calgary, AB, Canada
    J Neurooncol 90:171-80. 2008
    Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant central nervous system neoplasm that usually affects infants and young children...
  28. ncbi Immunohistochemical analysis of hSNF5/INI1 distinguishes renal and extra-renal malignant rhabdoid tumors from other pediatric soft tissue tumors
    Andrew C Hoot
    Department of Pathology, Children s Hospital of Philadelphia and The University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Am J Surg Pathol 28:1485-91. 2004
    Malignant rhabdoid tumor (MRT) is a highly aggressive neoplasm that occasionally demonstrates phenotypic overlap with other soft tissue malignancies...
  29. pmc Genomic analysis using high-density single nucleotide polymorphism-based oligonucleotide arrays and multiplex ligation-dependent probe amplification provides a comprehensive analysis of INI1/SMARCB1 in malignant rhabdoid tumors
    Eric M Jackson
    Department of Neurosurgery and Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
    Clin Cancer Res 15:1923-30. 2009
    ....
  30. ncbi Molecular genetics of atypical teratoid/rhabdoid tumor
    Jaclyn A Biegel
    Division of Human Genetics, Department of Pediatrics, The Children s Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Neurosurg Focus 20:E11. 2006
    ..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...
  31. ncbi INI1 induces interferon signaling and spindle checkpoint in rhabdoid tumors
    Alexei Morozov
    Department of Molecular Genetics, Albert Einstein College of Medicine, Bronx, New York 10461, USA
    Clin Cancer Res 13:4721-30. 2007
    ..Reintroduction of INI1 causes cell arrest and senescence in rhabdoid cells. Our purpose was to identify INI1-downstream genes and to determine their functional and therapeutic significance for rhabdoid tumors...
  32. ncbi Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor
    Mary Ann Zimmerman
    Department of Pediatric Oncology, Pediatric Neuro Oncology, Dana Farber Cancer Institute, Rm SW331, 44 Binny Street, Boston, MA 02115, USA
    J Neurooncol 72:77-84. 2005
    ..More importantly, we report on the first two survivors after relapse with multi-agent intravenous and intrathecal chemotherapy treated with this modified regimen...
  33. ncbi Alterations of the hSNF5/INI1 gene in central nervous system atypical teratoid/rhabdoid tumors and renal and extrarenal rhabdoid tumors
    Jaclyn A Biegel
    Division of Human Genetics and Department of Pathology, The Children s Hospital of Philadelphia, Pennsylvania 19104, USA
    Clin Cancer Res 8:3461-7. 2002
    ..The majority of rhabdoid tumors from all sites contained deletions and/or mutations of the INI1 gene. Specific mutations were nonrandomly associated with anatomical site...
  34. ncbi The role of INI1 and the SWI/SNF complex in the development of rhabdoid tumors: meeting summary from the workshop on childhood atypical teratoid/rhabdoid tumors
    Jaclyn A Biegel
    Department of Pediatrics, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Cancer Res 62:323-8. 2002
    ..A detailed summary of the discussions regarding diagnostic studies and the roles for radiation therapy, chemotherapy, and bone marrow or stem cell transplant in the treatment of rhabdoid tumors will be published separately...
  35. pmc Germline nonsense mutation and somatic inactivation of SMARCA4/BRG1 in a family with rhabdoid tumor predisposition syndrome
    Reinhard Schneppenheim
    Department of Pediatric Hematology and Oncology, University Medical Center Hamburg Eppendorf, Martinistrasse 52, Hamburg, Germany
    Am J Hum Genet 86:279-84. 2010
    ..Familial cases, described as rhabdoid tumor predisposition syndrome (RTPS), have been linked to heterozygous SMARCB1 germline mutations...
  36. ncbi Induction of autophagy in malignant rhabdoid tumor cells by the histone deacetylase inhibitor FK228 through AIF translocation
    Motonobu Watanabe
    Department of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan
    Int J Cancer 124:55-67. 2009
    ..Using immunoelectron microscopy, we confirmed AIF translocation into the nucleus of FK228-induced autophagic cells in vivo. Thus, FK228 is a novel candidate for an antitumor agent for MRT cells...
  37. ncbi Aurora A is a repressed effector target of the chromatin remodeling protein INI1/hSNF5 required for rhabdoid tumor cell survival
    Seungjae Lee
    Department of Genetics, Albert Einstein College of Medicine, New York University, New York, NY, USA
    Cancer Res 71:3225-35. 2011
    ..We propose that a high degree of Aurora A expression may play a role in aggressive behavior of RTs and that targeting expression or activity of this gene is a novel therapeutic strategy for these tumors...
  38. ncbi Immunohistochemical analysis of hSNF5/INI1 in pediatric CNS neoplasms
    Alexander R Judkins
    Department of Pathology, Children s Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Am J Surg Pathol 28:644-50. 2004
    Atypical teratoid/rhabdoid tumor (AT/RT) may be misdiagnosed as primitive neuroectodermal tumor/medulloblastoma (PNET) and occasionally as other tumors...
  39. ncbi GOK: a gene at 11p15 involved in rhabdomyosarcoma and rhabdoid tumor development
    S Sabbioni
    Department of Experimental and Diagnostic Medicine and Interdepartment Center for Biotechnology, University of Ferrara, Italy
    Cancer Res 57:4493-7. 1997
    ..5, was studied in breast cancer, rhabdomyosarcoma, and rhabdoid tumor cell lines...
  40. ncbi Primary rhabdoid cancer of the ileum: a case report and review of the literature
    Laszlo Toth
    Department of Pathology Molnár, University of Debrecen, Medical and Health Sciences Center MHCC, Debrecen, Hungary
    Pathol Res Pract 206:110-5. 2010
    ..of a rhabdoid cancer, described as an infrequent variant of Wilms' tumor, several cases of extrarenal rhabdoid tumor have been reported in the literature...
  41. ncbi Etodolac, a selective cyclooxygenase-2 inhibitor, induces apoptosis by activating caspases in human malignant rhabdoid tumor cells (FRTK-1)
    Michiyuki Hakozaki
    First Department of Pathology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima 960 1295, Japan
    Oncol Rep 17:169-73. 2007
    Malignant rhabdoid tumor (MRT) is a rare and highly aggressive tumor presenting in the kidney and soft tissue in childhood. However, effective treatment for MRT has not been established...
  42. ncbi Soft-tissue extrarenal rhabdoid tumor with a unique long-term survival
    Aurelie Fabre
    Histopathology Department, St Mary s Hospital, London, UK
    Ultrastruct Pathol 28:49-52. 2004
    ..The authors record the case a soft-tissue rhabdoid tumor in a 12-year-old boy with a unique long-term survival in excess of 16 years...
  43. ncbi Prognostic significance of dysadherin expression in epithelioid sarcoma and its diagnostic utility in distinguishing epithelioid sarcoma from malignant rhabdoid tumor
    Teiyu Izumi
    Department of Anatomic Pathology, Pathological Sciences, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Mod Pathol 19:820-31. 2006
    ..We studied the clinicopathological features in 72 cases of epithelioid sarcoma and in six cases of malignant rhabdoid tumor, and also assessed the immunohistochemical expression of dysadherin, E-cadherin and MIB-1 in epithelioid ..
  44. ncbi Snf5 tumor suppressor couples chromatin remodeling, checkpoint control, and chromosomal stability
    Anthony N Imbalzano
    University of Massachusetts Medical School, Department of Cell Biology, Worcester, Massachusetts 01655, USA
    Cancer Cell 7:294-5. 2005
    ..2005) reports that human SNF5 also signals via the p16INK4a-Rb-E2F pathway to regulate chromosomal stability, suggesting a new function for this chromatin remodeling protein in tumor suppression...
  45. pmc Atypical teratoid/rhabdoid tumor arising from the third cranial nerve
    Charles C Wykoff
    Bascom Palmer Eye Institute, University of Miami Miller School of Medicine and Miami Children s Hospital, Miami, Florida 33136, USA
    J Neuroophthalmol 28:207-11. 2008
    ..Rapid enlargement of the lesion over 1 month led to subtotal neurosurgical resection of an atypical teratoid/rhabdoid tumor (AT/RT), a rare, highly aggressive malignancy of infancy closely related histologically to medulloblastoma and ..
  46. pmc Potent inhibition of rhabdoid tumor cells by combination of flavopiridol and 4OH-tamoxifen
    Velasco Cimica
    Department of Genetics, Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, New York 10461, USA
    BMC Cancer 10:634. 2010
    ....
  47. pmc Clinicopathologic comparison of familial versus sporadic atypical teratoid/rhabdoid tumors (AT/RT) of the central nervous system
    Carol S Bruggers
    Division of Hematology Oncology, Department of Pediatrics, University of Utah School of Medicine and Primary Children s Medical Center, Salt Lake City, Utah, USA
    Pediatr Blood Cancer 56:1026-31. 2011
    ..Little data regarding familial AT/RT clinical course exist. This study described and compared familial (F) versus sporadic (S) AT/RT and elucidated SMARCB1 mutations and inheritance patterns...
  48. ncbi Mutation analysis of human cytokeratin 8 gene in malignant rhabdoid tumor: a possible association with intracytoplasmic inclusion body formation
    Hideki Shiratsuchi
    Department of Anatomic Pathology, Kyushu University, Graduate School of Medical Sciences, Fukuoka, Japan
    Mod Pathol 15:146-53. 2002
    The rhabdoid cell, which is typically observed in malignant rhabdoid tumor (MRT) and other malignant neoplasms, has an eosinophilic cytoplasm containing a spheroid perinuclear inclusion body...
  49. ncbi Atypical teratoid/rhabdoid tumor arising in a ganglioglioma: genetic characterization
    B K Kleinschmidt-Demasters
    Department of Pathology, Anschutz Medical Campus, University of Colorado, Denver, CO 80045, USA
    Am J Surg Pathol 35:1894-901. 2011
    Atypical teratoid/rhabdoid tumor (AT/RT) is an uncommon, aggressive, embryonal pediatric brain tumor that almost always develops de novo and does not arise within, or evolve from, other brain tumor types...
  50. ncbi Suppression of tumorigenicity of rhabdoid tumor derived G401 cells by the multivalent HB-19 pseudopeptide that targets surface nucleolin
    Bernard Krust
    CNRS Universite Paris Descartes, Unité Régulation de la Transcription de Maladies Génétique, 45 Rue des Saints Peres, Paris Cedex 06, France
    Biochimie 93:426-33. 2011
    ..Here we show that treatment of rhabdoid tumor derived G401 cells with a nucleolin antagonist, the HB-19 pseudopeptide, could restore contact inhibition, ..
  51. ncbi Malignant rhabdoid tumor of adrenal gland
    Nilgun Yaris
    Department of Paediatric Oncology, Karadeniz Technical University, Faculty of Medicine, Trabzon, Turkey
    Med Pediatr Oncol 39:128-31. 2002
  52. ncbi Differential expression of miRNAs in rhabdomyosarcoma and malignant rhabdoid tumor
    Sorin Armeanu-Ebinger
    Department of Pediatric Surgery and Urology, University Children s Hospital, Hoppe Seyler Str 3, 72076 Tubingen, Germany
    Exp Cell Res 318:2567-77. 2012
    Alveolar rhabdomyosarcoma (RMA) and malignant rhabdoid tumor (MRT) have a frequent metastatic spread and a poor prognosis. Aberrant miRNA expression is often found in metastatic tumors...
  53. pmc High-dose chemotherapy and autologous stem cell rescue for atypical teratoid/rhabdoid tumor of the central nervous system
    Theodore Nicolaides
    Department of Pediatrics, University of California San Francisco School of Medicine, 505 Parnassus Avenue, M649, Box 0106, San Francisco, CA 94143, USA
    J Neurooncol 98:117-23. 2010
    ..Two of nine patients treated for AT/RT at our institution with high dose chemotherapy and autologous bone marrow transplant are long-term survivors, suggesting that a subset of patients can be cured with this approach...
  54. ncbi Epigenetic modification after inhibition of IGF-1R signaling in human central nervous system atypical teratoid rhabdoid tumor (AT/RT)
    Kyu Won Shim
    Pediatric Neurosurgery Research Lab, Developmental Biology Program, Division of Pediatric Neurosurgery, Children s Hospital of Chicago Research Center and Department of Neurosurgery, Northwestern University Feinberg School of Medicine, Chicago, IL 60614, USA
    Childs Nerv Syst 29:1245-51. 2013
    ..This study investigated epigenetic modifications in human central nervous system atypical teratoid rhabdoid tumors (AT/RTs), in response to inhibition of insulin-like growth factor receptor 1 (IGF-1R)...
  55. ncbi Renal and extrarenal congenital rhabdoid tumor: diagnosis by fine-needle aspiration biopsy and FISH
    Ricardo Drut
    Department of Pathology, Hospital de Ninos Superiora Sor Maria Ludovica, La Plata, Argentina
    Diagn Cytopathol 27:32-4. 2002
    We report on 2 patients with congenital malignant rhabdoid tumor, one located to the kidney and the other to the soft parts of the cheek...
  56. ncbi Treatment of metastatic rhabdoid tumor of the kidney
    Lars Wagner
    Department of Hematology Oncology, St Jude Children s Research Hospital, Memphis, Tennessee 38105 2794, USA
    J Pediatr Hematol Oncol 24:385-8. 2002
    Metastatic rhabdoid tumor of the kidney (RTK) is a highly lethal malignancy; only one survivor with stage 4 disease has been reported...
  57. ncbi Antitumor activity of gefitinib in malignant rhabdoid tumor cells in vitro and in vivo
    Yasumichi Kuwahara
    Department of Pediatrics, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kamigyo ku, Kyoto 602 8566, Japan
    Clin Cancer Res 10:5940-8. 2004
    Malignant rhabdoid tumor (MRT) is a rare and highly aggressive neoplasm of young children. Current treatments have had only limited success. Epidermal growth factor receptor (EGFR) was found recently to be expressed on MRT cell lines...
  58. ncbi Malignant rhabdoid tumor presenting as a hemangioma
    A E Albregts
    Department of Dermatology, University of Texas, Houston Medical School, USA
    Pediatr Dermatol 13:468-71. 1996
    Malignant rhabdoid tumor is a rare and highly aggressive malignancy of unknown etiology. We report a primary cutaneous rhabdoid tumor on the upper back of a newborn...
  59. ncbi Sarcomatoid carcinoma of the gallbladder with a rhabdoid tumor component
    Mi Jung Kim
    Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
    Arch Pathol Lab Med 127:e406-8. 2003
    We report the case of a sarcomatoid carcinoma with a rhabdoid tumor component originating in the gallbladder, along with immunohistochemical and electron microscopic findings...
  60. ncbi Age, stage, and radiotherapy, but not primary tumor site, affects the outcome of patients with malignant rhabdoid tumors
    Iyad Sultan
    Department of Pediatric Oncology, King Hussein Cancer Centre KHCC, Amman, Jordan
    Pediatr Blood Cancer 54:35-40. 2010
    ..Malignant rhabdoid tumors (MRTs) are aggressive and often fatal; the Surveillance, Epidemiology, and End Results (SEER) database offers an opportunity to study this rare malignancy...
  61. ncbi Identification and metastatic potential of tumor-initiating cells in malignant rhabdoid tumor of the kidney
    Satohiko Yanagisawa
    Department of Pediatric Surgery, Jichi Medical University, Shimotsuke, Tochigi, Japan
    Clin Cancer Res 15:3014-22. 2009
    Malignant rhabdoid tumor of the kidney (MRTK) is a rare and highly aggressive malignancy of infanthood...
  62. ncbi 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
    Sharon L Gardner
    Department of Pediatrics, New York University, New York, New York 10016, USA
    Pediatr Blood Cancer 51:235-40. 2008
    ..Historically, surgery and standard dose chemotherapy have resulted in a median survival of 8.5 months from diagnosis...
  63. pmc Haploinsufficiency of Snf5 (integrase interactor 1) predisposes to malignant rhabdoid tumors in mice
    C W Roberts
    Division of Hematology Oncology, Children s Hospital and Dana Farber Cancer Institute, Department of Pediatrics, Harvard Medical School and Howard Hughes Medical Institute, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 97:13796-800. 2000
    Malignant rhabdoid tumor (MRT) is an aggressive, highly lethal cancer of young children. Tumors occur in various locations, including kidney, brain, and soft tissues...
  64. ncbi An extrarenal malignant rhabdoid tumor suspected to originate from the mesentery in an adult: report of a case
    Masaharu Ohgaki
    Department of Surgery, Fukuchiyama City Hospital, 231 Atsunaka cho, Fukuchiyama, Kyoto 602 8505, Japan
    Surg Today 33:556-9. 2003
    A malignant rhabdoid tumor is very rare and its prognosis is extremely poor. It was first described as a distinctive and highly malignant neoplasm of the infant kidney...
  65. ncbi Adult rhabdoid renal cell carcinoma
    Beverley Shannon
    Tissugen Pty Ltd, Perth, Western Australia
    Arch Pathol Lab Med 126:1506-10. 2002
    Pediatric rhabdoid tumor of the kidney is regarded as a distinct neoplasm, whereas rhabdoid differentiation in adult renal cell carcinoma is usually found in association with conventional (clear cell) tumor, from which it is thought to ..
  66. pmc Histone deacetylase inhibition decreases proliferation and potentiates the effect of ionizing radiation in atypical teratoid/rhabdoid tumor cells
    Jeffrey A Knipstein
    Department of Pediatrics, Section of Pediatric Hematology Oncology BMT, University of Colorado Denver, Aurora, CO, USA
    Neuro Oncol 14:175-83. 2012
    Atypical teratoid/rhabdoid tumor (ATRT) is a highly malignant central nervous system neoplasm that primarily occurs in children less than 3 years of age...
  67. pmc Epigenetic inactivation of the tumor suppressor BIN1 drives proliferation of SNF5-deficient tumors
    Elizabeth S McKenna
    Department of Pediatric Oncology, Dana Farber Cancer Institute, Boston, MA USA
    Cell Cycle 11:1956-65. 2012
    ..Identification of BIN1 as a SNF5 target gene reveals a novel tumor suppressive regulatory mechanism whose disruption can drive cancer formation...
  68. ncbi Atypical teratoid rhabdoid tumors (ATRTs): the British Columbia's Children's Hospital's experience, 1986-2006
    Adam J Fleming
    Division of Hematology, Oncology and Bone Marrow Transplantation, British Columbia Children s Hospital, Vancouver, British Columbia, Canada
    Brain Pathol 22:625-35. 2012
    ..Epigenetically, the MGMT promoter is usually unmethylated in ATRT, suggesting that potential temozolomide-based chemotherapy may be of limited efficacy...
  69. ncbi Congenital orbital and disseminated extrarenal malignant rhabdoid tumor
    Chloe Gottlieb
    McMaster University School of Medicine, Hamilton, Ontario, Canada
    Ophthal Plast Reconstr Surg 21:76-9. 2005
    ..Histologic reports, slides, and clinical photographs were reviewed. A diagnosis of malignant rhabdoid tumor was made...
  70. ncbi Metastatic malignant rhabdoid tumor of the liver treated with tandem high-dose therapy and autologous peripheral blood stem cell rescue
    Howard M Katzenstein
    Med Pediatr Oncol 40:199-201. 2003
  71. ncbi Malignant extrarenal rhabdoid tumor of the bladder: 9-year survival after chemotherapy and partial cystectomy
    Joseph H Chang
    Department of Urology, Kaiser Permanente Medical Center, Los Angeles Woodland Hills, California, USA
    J Urol 171:820-1. 2004
  72. ncbi Fetal and neonatal rhabdoid tumor
    Hart Isaacs
    Department of Pathology, Rady Children s Hospital San Diego, San Diego, CA 92123, USA
    J Pediatr Surg 45:619-26. 2010
    Few studies have focused on the behavior of rhabdoid tumor (RT) in the fetus and neonate. The purpose of this review is to show that perinatal RTs are associated with unusual findings and a poor prognosis.
  73. ncbi Clinicopathologic prognostic factors in childhood atypical teratoid and rhabdoid tumor of the central nervous system: a multicenter study
    Christelle Dufour
    Department of Pediatric and Adolescent Oncology, Gustave Roussy Institute, 114 rue Edouard Vaillant, Villejuif, France
    Cancer 118:3812-21. 2012
    ..The objective of this study was to describe the clinical and pathologic features and to identify prognostic factors in patients with atypical teratoid/rhabdoid tumors (AT/RT) of the central nervous system (CNS)...
  74. pmc Absence of oncogenic canonical pathway mutations in aggressive pediatric rhabdoid tumors
    Mark W Kieran
    Director, Pediatric Medical Neuro Oncology, Dana Farber Cancer Institute and Boston Children s Hospital, Pediatric Hematology Oncology, Boston, MA 02215, USA
    Pediatr Blood Cancer 59:1155-7. 2012
    Rhabdoid tumors (also called atypical teratoid/rhabdoid tumor (AT/RT) in the brain), are highly malignant, poor prognosis lesions arising in the kidneys, soft tissues, and central nervous system...
  75. ncbi Expression of neural stem cell markers in malignant rhabdoid tumor cell lines
    Kazuto Okuno
    Department of Pediatrics, Shiga University of Medical Science, Otsu, Shiga, Japan
    Oncol Rep 23:485-92. 2010
    Malignant rhabdoid tumor (MRT) is considered to display multi-phenotypic characteristics but the true origin of this tumor remains unknown. In recent years, the concept of the cancer stem cell (CSC) has drawn great attention...
  76. ncbi Clinical and molecular features in patients with atypical teratoid rhabdoid tumor or malignant rhabdoid tumor
    Uwe Kordes
    Department of Pediatric Hematology and Oncology, University Medical Center Hamburg Eppendorf, Hamburg, Germany
    Genes Chromosomes Cancer 49:176-81. 2010
    The SMARCB1 gene status in 50 patients with atypical teratoid rhabdoid tumor and/or malignant rhabdoid tumor recruited to a German registry was prospectively analyzed with FISH and PCR...
  77. ncbi Molecular analysis of pediatric brain tumors
    Jaclyn A Biegel
    Division of Human Genetics, Department of Pediatrics, University of Pennsylvania School of Medicine, The Children s Hospital of Philadelphia, 3615 Civic Center Boulevard, Philadelphia, PA 19104, USA
    Curr Oncol Rep 6:445-52. 2004
    ..The finding of an INI1 alteration in an atypical teratoid/rhabdoid tumor or malignant neoplasm with overlapping histologic features will be required for entry onto disease-specific ..
  78. ncbi Paratesticular congenital malignant rhabdoid tumor diagnosed by fine-needle aspiration cytology. a case report
    Javier Salamanca
    Department of Pathology, 12 de Octubre University Hospital, Madrid, Spain
    Diagn Cytopathol 30:46-50. 2004
    We report the FNA features of a congenital malignant extrarenal rhabdoid tumor (MERT) located in the right paratesticular area of a newborn full-term boy (39 wk gestation), with disseminated metastases in the liver and right parietal ..
  79. ncbi Incidence of atypical teratoid/rhabdoid tumors in children: a population-based study by the Austrian Brain Tumor Registry, 1996-2006
    Adelheid Woehrer
    Institute of Neurology, Medical University of Vienna, Vienna, Austria
    Cancer 116:5725-32. 2010
    ..They occur predominantly in young children, with an estimated prevalence of 1% to 2% among all pediatric CNS tumors. However, population-based data on the incidence of these tumors are not yet available...
  80. ncbi Primary extrarenal rhabdoid tumor of the ovary. A case report
    Charles A Leath
    Department of Obstetrics and Gynecology, University of Alabama at Birmingham, 340 Old Hillman Building, 619 19th Street South, Birmingham, AL 35249 7333, USA
    J Reprod Med 48:283-6. 2003
    ..Although extrarenal rhabdoid tumors have been documented at multiple extrarenal sites, to our knowledge no primary ovarian cases have been reported...
  81. pmc Tandem high-dose chemotherapy and autologous stem cell transplantation in young children with atypical teratoid/rhabdoid tumor of the central nervous system
    Eun Sil Park
    Department of Pediatrics, Institute of Health Science, Gyeongsang National University School of Medicine, Jinju, Korea
    J Korean Med Sci 27:135-40. 2012
    ..3% ± 17.3%. Our tandem HDCT/autoSCT is feasible; however, early administration of RT prior to tandem HDCT/autoSCT should be considered to improve the outcome after tandem HDCT/autoSCT...
  82. ncbi Cerebral composite atypical teratoid/rhabdoid tumor and yolk sac tumor in the frontal lobe of an infant. Case report
    Masaru Honda
    Department of Neurosurgery, National Nagasaki Medical Center, Omura, Japan
    Neurol Med Chir (Tokyo) 45:318-21. 2005
    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...
  83. ncbi Systematic analysis of the antiproliferative effects of novel and standard anticancer agents in rhabdoid tumor cell lines
    Henning Lünenbürger
    Department of Pediatric Hematology and Oncology, University Children s Hospital Munster, Munster, Germany
    Anticancer Drugs 21:514-22. 2010
    ..3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyl-2H-tetrazolium bromide (MTT) cell proliferation assay on three rhabdoid tumor cell lines, A204, G401, and BT16, derived from different anatomical sites...
  84. ncbi Proximal-type epithelioid sarcoma of the vulva: relationship to malignant extrarenal rhabdoid tumor
    Ashok Tholpady
    Department of Pathology, University of Virginia Medical Center, Charlottesville, VA 22908 0214, USA
    Int J Gynecol Pathol 29:600-4. 2010
    ..We report a case of this entity and discuss its relationship to the epithelial-type "malignant rhabdoid tumor" (MRT) of the soft tissue...
  85. ncbi Congenital extrarenal non-central nervous system malignant rhabdoid tumor
    Mitra Sajedi
    Southern Alberta Children s Cancer Program, Alberta Children s Hospital and Tom Baker Cancer Center, Calgary, Alberta, Canada
    J Pediatr Hematol Oncol 24:316-20. 2002
    Malignant rhabdoid tumor (MRT) is a rare tumor occurring mostly in kidneys and central nervous system (CNS). Its prognosis is not good. Occasionally, MRTs are diagnosed at or immediately after birth...
  86. ncbi Cytopathology and ultrastructure of primary rhabdoid tumor of lung
    I Rubenchik
    Department of Pathology, Toronto Hospital, Ontario, Canada
    Ultrastruct Pathol 20:355-60. 1996
    ..presents the light microscopy, immunocytochemistry, and ultrastructure of the first unequivocal extrarenal rhabdoid tumor occurring in lung...
  87. ncbi Epigenetic repression of RASSF1A but not CASP8 in supratentorial PNET (sPNET) and atypical teratoid/rhabdoid tumors (AT/RT) of childhood
    J Mühlisch
    Department of Pediatric Hematology and Oncology, University Children s Hospital Muenster, Germany
    Oncogene 25:1111-7. 2006
    ..Further studies of epigenetic changes in these rare tumors are warranted as their biology remains obscure and treatment efforts have been rather unsuccessfull...
  88. ncbi Malignant rhabdoid tumor beside benign skin mesenchymal neoplasm with myofibromatous features
    M Garcia-Bustinduy
    Department of Dermatology, Hospital Universitario de Canarias, University of La Laguna, Tenerife, Spain
    J Cutan Pathol 26:509-15. 1999
    ..The pathological findings suggest a mesenchymal origin (hemangiopericytic or myofibroblastic type) for both tumors. The patient was surgically treated, but she died nine months later...
  89. ncbi Activation of Akt/mTOR pathway in a patient with atypical teratoid/rhabdoid tumor
    Jarosław Jóźwiak
    Department of Histology and Embryology, Center for Biostructure Research, Medical University of Warsaw, ul Chalubinskiego 5, 02 004 Warsaw, Poland
    Folia Neuropathol 48:185-9. 2010
    A typical teratoid/rhabdoid tumor (AT/RT) is a highly malignant childhood brain tumor. Most AT/RTs are shown to contain chromosome 22 mutation in the region of hSNF5/INI1 gene, whose protein product participates in chromatin remodeling...
  90. ncbi Malignant extrarenal rhabdoid tumor of the vulva in an adult
    H Narendra
    Department of Surgical Oncology, Kasturba Medical College, Manipal University, Manipal, India
    J Cancer Res Ther 6:82-5. 2010
    ..Malignant rhabdoid tumor (MRT) of the kidney is a distinctive clinicopathological entity that is recognized as a highly aggressive ..
  91. ncbi Atypical teratoid/rhabdoid tumor of the spine in an adult: case report and review of the literature
    Elena L Zarovnaya
    Department of Pathology, Dartmouth Hitchcock Medical Center, One Medical Center Drive, Lebanon, NH 03756, USA
    J Neurooncol 84:49-55. 2007
    ..An unrestricted autopsy was performed. To our knowledge, this is the first case of a spinal atypical teratoid/rhabdoid tumor in an adult fully documented with molecular, immunohistochemical, cytogenetic and autopsy findings.
  92. ncbi Malignant rhabdoid tumor in a pregnant adult female: literature review of central nervous system rhabdoid tumors
    Michelle L Erickson
    Department of Pathology, Yale University School of Medicine, New Haven, CT 06520 8023, USA
    J Neurooncol 74:311-9. 2005
    ..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 ..
  93. ncbi Malignant rhabdoid tumor of the vulva: case report with cytological, immunohistochemical, ultrastructural and DNA ploidy studies and a review of the literature
    T Igarashi
    Department of Obstetrics and Gynecology, Tohoku University School of Medicine, Sendai, Japan
    Pathol Int 48:887-91. 1998
    A case of malignant rhabdoid tumor of the vulva in a 25-year-old female was examined. The patient presented with a subcutaneous nodule in the left labium majus...
  94. ncbi Primary rhabdoid tumor of the brain in an adult
    Turkan Rezanko
    Department of Pathology, Ataturk Training and Research Hospital, Izmir, Turkey
    Neuropathology 26:57-61. 2006
    b>Rhabdoid tumor (RT) is an uncommon childhood neoplasm that typically arises within the kidney. It is characterized by an aggressive clinical course...
  95. ncbi Malignant rhabdoid tumor of the vulva: a case report and review of the literature
    G Lupi
    Gynecology Oncology Department, Istituto Nazionale per lo Studio e la Cura dei Tumori, Milan, Italy
    Tumori 82:93-5. 1996
    Malignant rhabdoid tumor (MRT) is an uncommon aggressive neoplasm which usually occurs in the kidney of children, but it has also been found in extrarenal sites. MRT arising in the vulva is extremely rare...
  96. ncbi Treatment of stage IV malignant rhabdoid tumor of the kidney (MRTK) with ICE and VDCy: a case report
    Masaki Yamamoto
    Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Japan
    J Pediatr Hematol Oncol 28:286-9. 2006
    The prognosis of stage IV malignant rhabdoid tumor of the kidney (MRTK) has been extremely poor...
  97. pmc Atypical teratoid/rhabdoid tumor evolving from an optic pathway ganglioglioma: case study
    Jeffrey C Allen
    Department of Neurology, New York University School of Medicine, New York, NY 10016, USA
    Neuro Oncol 8:79-82. 2006
    We report an atypical teratoid/rhabdoid tumor arising in a ganglioglioma from an 11-year-old male who had been treated over a nine-year period...
  98. ncbi Atypical teratoid rhabdoid tumor (AT/RT) in adults: review of four cases
    Addisalem T Makuria
    Department of Pathology, Georgetown University Hospital, 3900 Reservoir Road N W, Washington, DC 20007, USA
    J Neurooncol 88:321-30. 2008
    ..Although the prognosis is dismal in pediatric population, long term survival is possible in adult AT/RT cases after surgery and adjuvant radiotherapy and chemotherapy...
  99. ncbi Impact of radiotherapy for pediatric CNS atypical teratoid/rhabdoid tumor (single institute experience)
    Yi Wei Chen
    Cancer Center, Neurological Institute, Taipei, Taiwan
    Int J Radiat Oncol Biol Phys 64:1038-43. 2006
    To assess outcomes and prognostic factors in radiotherapy of pediatric central nervous system atypical teratoid/rhabdoid tumor (AT/RT).
  100. ncbi Malignant rhabdoid tumor of the clitoris in an elderly patient: report of a case
    D Haidopoulos
    1st Department of Obstetrics and Gynecology, University of Athens, Alexandra Hospital, Greece
    Eur J Gynaecol Oncol 23:447-9. 2002
    ..We report a case of malignant rhabdoid tumor of the clitoris occurring in an elderly patient...
  101. ncbi Trastuzumab activates allogeneic or autologous antibody-dependent cellular cytotoxicity against malignant rhabdoid tumor cells and interleukin-2 augments the cytotoxicity
    Yoshiki Katsumi
    Department of Pediatrics, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto, Japan
    Clin Cancer Res 14:1192-9. 2008
    Malignant rhabdoid tumor (MRT) is an early childhood cancer with poor prognosis...

Research Grants24

  1. Role of hsnf5/BAF47 Loss in Human Cancer Development
    Bernard E Weissman; Fiscal Year: 2012
    ..in the development of an aggressive childhood cancer of the kidney, brain and soft tissues called malignant rhabdoid tumor (MRT)...
  2. GENETICS OF PEDIATRIC RHABDOID TUMORS
    Jaclyn A Biegel; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): Rhabdoid tumor is a clinically aggressive malignancy that generally presents in the first four years of life...
  3. NATIONAL WILMS TUMOR STUDY -5
    Daniel Green; Fiscal Year: 2003
    ..evaluate the activity of the combination of etoposide, carboplatinum and cyclophosphamide in children with rhabdoid tumor of the kidney...
  4. TUMOR SUPPRESSORS AND IMPRINTING AT CHROMOSOME 11P155
    Michael Higgins; Fiscal Year: 2000
    ..Three BWS rearrangement breakpoints and a rhabdoid tumor breakpoint have been shown to disrupt the Long QT (KVLQT1) gene...
  5. RHABDOID TUMOR GENE
    Deborah Schofield; Fiscal Year: 2000
    ..The research proposal centers around an attempt to define the rhabdoid tumor, a poorly understood and controversial childhood neoplasm which is generally associated with a dismal ..
  6. ISOLATION OF THE NEUROBLASTOMA PREDISPOSITION GENE
    John Maris; Fiscal Year: 2006
    ..Ultimately, these experiments should lead to the identification of a common pathway to neuroblastoma tumorigenesis that will be an outstanding target for rationally designed therapeutics. ..
  7. PEDIATRIC BRAIN TUMOR RESEARCH CENTER
    Susan Blaney; Fiscal Year: 2003
    ..It offers leadership in the development of new agents, in new uses of sophisticated radiotherapy, and in the application of new approaches to brain tumor therapy. ..
  8. SWI/SNF Chromatin Remodeling Loss and Human Cancer
    Bernard Weissman; Fiscal Year: 2007
    ..abstract_text> ..
  9. MOLECULAR ANALYSIS OF FANCONI'S ANEMIA C PROTEIN
    Sharon Plon; Fiscal Year: 2003
    ....
  10. Xenogen's IVIS 100 Imaging System
    Mark Kieran; Fiscal Year: 2004
    ..This luciferase-based technology affords a superior method to achieve this aim. ..
  11. Significance of Genetic Alterations in Neuroblastoma
    John Maris; Fiscal Year: 2006
    ..It is expected that these will be of prognostic importance and serve as specific targets for developmental therapeutics. ..
  12. Serum Markers of Angiogenesis in von Hippel-Lindau Dise*
    Gail Tomlinson; Fiscal Year: 2005
    ..The establishment of a VHL registry in North Texas will also place us in an excellent position to develop clinical protocols aimed towards early detection of lesions in VHL patients. ..
  13. ALTERNATIVE DNA DAMAGE CHECKPOINT PATHWAYS IN EUKARYOTES
    Sharon Plon; Fiscal Year: 2002
    ..These latter results will demonstrate whether activation of an alternative checkpoint pathway might be used therapeutically for patients with AT or to alter the resistance of tumors to radiation and other DNA-damaging agents. ..
  14. HARVARD PEDIATRIC BRAIN TUMOR CENTER
    Mark Kieran; Fiscal Year: 2003
    ..David Louis). In summary, we will bring the full thrust of a Comprehensive Cancer Center and the full services of a pediatric center to enhance the Consortium and our mutual missions. ..
  15. SWI/SNF complex loss facilitates gene silencing during NSCLC development
    Bernard E Weissman; Fiscal Year: 2010
    ..abstract_text> ..
  16. ISOLATION OF A SECOND WILMS TUMOR SUPPRESSOR GENE
    Bernard Weissman; Fiscal Year: 2001
    ....
  17. Pirfenidone in Children with PNs in NF1
    Roger Packer; Fiscal Year: 2006
    ..Abstract Not Provided ..
  18. Do Physicians Understand Uncertain Variants and Other Genetic Test Results?
    Sharon Plon; Fiscal Year: 2009
    ..With the increasing availability of complex testing modalities, e.g. DNA and RNA gene chips, for a variety of both rare and common diseases, appropriate reporting and physician education must accompany the development of these tests. ..
  19. Regulation- Medulloblastoma Growth by alpha-v integrins
    Tobey Macdonald; Fiscal Year: 2006
    ..The long-range goal of this work is to broaden our understanding of medulloblastoma growth, so that novel biologic therapies may be developed into new treatments for medulloblastoma, the most common malignant brain tumor in children. ..
  20. CHILDRENS NATIONAL MEDICAL CENTER
    Roger Packer; Fiscal Year: 2003
    ....
  21. Gene Transfer to Facilitate Dose-Intensification in the Treatment of Pediatric B*
    Lars Wagner; Fiscal Year: 2007
    ..We hypothesize this strategy will be well tolerated and allow for meaningful dose escalation through chemoprotection afforded by gene transfer into hematopoietic stem cells. [unreadable] [unreadable] [unreadable]..
  22. TELOMERASE AS A PROGNOSTIC INDICATOR FOR WILMS TUMOR
    Jeffrey Dome; Fiscal Year: 2002
    ..Telomerase levels will be quantified by assaying enzyme activity, expression of TERT, and expression of hTR, the RNA template component of the enzyme. ..
  23. Lapatinib in childhood CNS malignancies
    Maryam Fouladi; Fiscal Year: 2006
    ..These data will be used in the rational design of future phase I combination or frontline studies to improve the outcome in children with these poor prognosis tumors. [unreadable] [unreadable]..
  24. Analysis of the Novel Tumor Suppressor Gene SNF5/INI1
    Charles Roberts; Fiscal Year: 2006
    Malignant rhabdoid tumor (MRT) is an aggressive, highly lethal cancer that strikes young children. Tumors occur in various locations including kidney, brain, and soft tissues...