Atypical teratoid/rhabdoid tumor (AT/RT): Malignant embryonal tumor of CNS in early childhood
Closely related to renal & soft tissue rhabdoid tumors
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Zhang M et al: Radiomic phenotypes distinguish atypical teratoid/rhabdoid tumors from medulloblastoma. AJNR Am J Neuroradiol. 42(9):1702-8, 2021
Tamrazi B et al: Pediatric atypical teratoid/rhabdoid tumors of the brain: identification of metabolic subgroups using in vivo 1H-MR spectroscopy. AJNR Am J Neuroradiol. 40(5):872-7, 2019
Nowak J et al: Magnetic resonance imaging surrogates of molecular subgroups in atypical teratoid/rhabdoid tumor. Neuro Oncol. 20(12):1672-9, 2018
Fischer-Valuck BW et al: Assessment of the treatment approach and survival outcomes in a modern cohort of patients with atypical teratoid rhabdoid tumors using the National Cancer Database. Cancer. 123(4):682-7, 2017
Katz JS et al: Cerebellopontine angle tumors in young children, displaying cranial nerve deficits, and restricted diffusion on diffusion-weighted imaging: a new clinical triad for atypical teratoid/rhabdoid tumors. Childs Nerv Syst. 33(5):833-8, 2017
Frühwald MC et al: Atypical teratoid/rhabdoid tumors-current concepts, advances in biology, and potential future therapies. Neuro Oncol. 18(6):764-78, 2016
Johann PD et al: Atypical teratoid/rhabdoid tumors are comprised of three epigenetic subgroups with distinct enhancer landscapes. Cancer Cell. 29(3):379-93, 2016
DiPatri AJ Jr et al: Atypical teratoid rhabdoid tumors of the posterior fossa in children. Childs Nerv Syst. 31(10):1717-28, 2015
Ho DM et al: Integrated genomics has identified a new AT/RT-like yet INI1-positive brain tumor subtype among primary pediatric embryonal tumors. BMC Med Genomics. 8:32, 2015
Lau CS et al: Atypical teratoid rhabdoid tumors: a population-based clinical outcomes study involving 174 patients from the Surveillance, Epidemiology, and End Results database (1973-2010). Cancer Manag Res. 7:301-9, 2015
Torchia J et al: Molecular subgroups of atypical teratoid rhabdoid tumours in children: an integrated genomic and clinicopathological analysis. Lancet Oncol. 16(5):569-82, 2015
Ostrom QT et al: CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2007-2011. Neuro Oncol. 16 Suppl 4:iv1-63, 2014
Slavc I et al: Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012. Cancer Med. 3(1):91-100, 2014
Au Yong KJ et al: How specific is the MRI appearance of supratentorial atypical teratoid rhabdoid tumors? Pediatr Radiol. 43(3):347-54, 2013
Bruggers CS et al: Magnetic resonance imaging spectroscopy in pediatric atypical teratoid rhabdoid tumors of the brain. J Pediatr Hematol Oncol. 36(6):341-5, 2013
Perreault S et al: Relapse patterns in pediatric embryonal central nervous system tumors. J Neurooncol. 115(2):209-15, 2013
Koral K et al: Imaging characteristics of atypical teratoid-rhabdoid tumor in children compared with medulloblastoma. AJR Am J Roentgenol. 190(3):809-14, 2008
Warmuth-Metz M et al: CT and MR imaging in atypical teratoid/rhabdoid tumors of the central nervous system. Neuroradiology. 50(5):447-52, 2008
Meyers SP et al: Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes. AJNR Am J Neuroradiol. 27(5):962-71, 2006
Parmar H et al: Imaging findings in primary intracranial atypical teratoid/rhabdoid tumors. Pediatr Radiol. 36(2):126-32, 2006
Fenton LZ et al: Atypical teratoid/rhabdoid tumor of the central nervous system in children: an atypical series and review. Pediatr Radiol. 33(8):554-8, 2003
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References
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Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Synonyms
Malignant rhabdoid tumor of brain
Intracranial rhabdoid tumor
Definitions
Atypical teratoid/rhabdoid tumor (AT/RT): Malignant embryonal tumor of CNS in early childhood
Closely related to renal & soft tissue rhabdoid tumors
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Zhang M et al: Radiomic phenotypes distinguish atypical teratoid/rhabdoid tumors from medulloblastoma. AJNR Am J Neuroradiol. 42(9):1702-8, 2021
Tamrazi B et al: Pediatric atypical teratoid/rhabdoid tumors of the brain: identification of metabolic subgroups using in vivo 1H-MR spectroscopy. AJNR Am J Neuroradiol. 40(5):872-7, 2019
Nowak J et al: Magnetic resonance imaging surrogates of molecular subgroups in atypical teratoid/rhabdoid tumor. Neuro Oncol. 20(12):1672-9, 2018
Fischer-Valuck BW et al: Assessment of the treatment approach and survival outcomes in a modern cohort of patients with atypical teratoid rhabdoid tumors using the National Cancer Database. Cancer. 123(4):682-7, 2017
Katz JS et al: Cerebellopontine angle tumors in young children, displaying cranial nerve deficits, and restricted diffusion on diffusion-weighted imaging: a new clinical triad for atypical teratoid/rhabdoid tumors. Childs Nerv Syst. 33(5):833-8, 2017
Frühwald MC et al: Atypical teratoid/rhabdoid tumors-current concepts, advances in biology, and potential future therapies. Neuro Oncol. 18(6):764-78, 2016
Johann PD et al: Atypical teratoid/rhabdoid tumors are comprised of three epigenetic subgroups with distinct enhancer landscapes. Cancer Cell. 29(3):379-93, 2016
DiPatri AJ Jr et al: Atypical teratoid rhabdoid tumors of the posterior fossa in children. Childs Nerv Syst. 31(10):1717-28, 2015
Ho DM et al: Integrated genomics has identified a new AT/RT-like yet INI1-positive brain tumor subtype among primary pediatric embryonal tumors. BMC Med Genomics. 8:32, 2015
Lau CS et al: Atypical teratoid rhabdoid tumors: a population-based clinical outcomes study involving 174 patients from the Surveillance, Epidemiology, and End Results database (1973-2010). Cancer Manag Res. 7:301-9, 2015
Torchia J et al: Molecular subgroups of atypical teratoid rhabdoid tumours in children: an integrated genomic and clinicopathological analysis. Lancet Oncol. 16(5):569-82, 2015
Ostrom QT et al: CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2007-2011. Neuro Oncol. 16 Suppl 4:iv1-63, 2014
Slavc I et al: Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012. Cancer Med. 3(1):91-100, 2014
Au Yong KJ et al: How specific is the MRI appearance of supratentorial atypical teratoid rhabdoid tumors? Pediatr Radiol. 43(3):347-54, 2013
Bruggers CS et al: Magnetic resonance imaging spectroscopy in pediatric atypical teratoid rhabdoid tumors of the brain. J Pediatr Hematol Oncol. 36(6):341-5, 2013
Perreault S et al: Relapse patterns in pediatric embryonal central nervous system tumors. J Neurooncol. 115(2):209-15, 2013
Koral K et al: Imaging characteristics of atypical teratoid-rhabdoid tumor in children compared with medulloblastoma. AJR Am J Roentgenol. 190(3):809-14, 2008
Warmuth-Metz M et al: CT and MR imaging in atypical teratoid/rhabdoid tumors of the central nervous system. Neuroradiology. 50(5):447-52, 2008
Meyers SP et al: Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes. AJNR Am J Neuroradiol. 27(5):962-71, 2006
Parmar H et al: Imaging findings in primary intracranial atypical teratoid/rhabdoid tumors. Pediatr Radiol. 36(2):126-32, 2006
Fenton LZ et al: Atypical teratoid/rhabdoid tumor of the central nervous system in children: an atypical series and review. Pediatr Radiol. 33(8):554-8, 2003
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