Atypical teratoid rhabdoid tumor overview: Difference between revisions
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{{Atypical teratoid rhabdoid tumor}} | {{Atypical teratoid rhabdoid tumor}} | ||
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==Overview== | ==Overview== | ||
Atypical teratoid rhabdoid tumor is an uncommon WHO Grade IV tumor, which in the vast majority of cases occurs in young children less than two years of age. It most frequently presents as a posterior fossa mass. Atypical teratoid rhabdoid tumor is comprised of rhabdoid tumor cells and varying amounts of small undifferentiated primitive neuroectodermal tumor (PNET)-like, mesenchymally, and/or epithelially differentiated tumor cells. Gene involved in the pathogenesis of atypical teratoid rhomboid tumor include ''[[SMARCB1]]'' (''hSNF5/INI-1''), a tumor suppressor gene.<ref name="GinnGajjar2012">{{cite journal|last1=Ginn|first1=Kevin F.|last2=Gajjar|first2=Amar|title=Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions|journal=Frontiers in Oncology|volume=2|year=2012|issn=2234-943X|doi=10.3389/fonc.2012.00114}}</ref> Atypical teratoid rhabdoid tumor may be associated with rhabdoid predisposition syndrome.<ref name="SlavcChocholous2014">{{cite journal|last1=Slavc|first1=Irene|last2=Chocholous|first2=Monika|last3=Leiss|first3=Ulrike|last4=Haberler|first4=Christine|last5=Peyrl|first5=Andreas|last6=Azizi|first6=Amedeo A.|last7=Dieckmann|first7=Karin|last8=Woehrer|first8=Adelheid|last9=Peters|first9=Christina|last10=Widhalm|first10=Georg|last11=Dorfer|first11=Christian|last12=Czech|first12=Thomas|title=Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012|journal=Cancer Medicine|volume=3|issue=1|year=2014|pages=91–100|issn=20457634|doi=10.1002/cam4.161}}</ref> On gross pathology, atypical teratoid rhabdoid tumor is characterized by an encapsulated, grayish, friable mass which is moderately vascular.<ref name="pmid22135607">{{cite journal| author=Chan KH, Mohammed Haspani MS, Tan YC, Kassim F| title=A case report of atypical teratoid/rhabdoid tumour in a 9-year-old girl. | journal=Malays J Med Sci | year= 2011 | volume= 18 | issue= 3 | pages= 82-6 | pmid=22135607 | doi= | pmc=PMC3216225 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22135607 }} </ref> On microscopic histopathological analysis, atypical teratoid rhabdoid tumor is characterized by round blue tumor cells of high cellularity composed of atypical cells with eccentric nuclei, small nucleoli, and abundant amounts of eosinophilic cytoplasm with frequent mitotic figures.<ref name="SlavcChocholous2014">{{cite journal|last1=Slavc|first1=Irene|last2=Chocholous|first2=Monika|last3=Leiss|first3=Ulrike|last4=Haberler|first4=Christine|last5=Peyrl|first5=Andreas|last6=Azizi|first6=Amedeo A.|last7=Dieckmann|first7=Karin|last8=Woehrer|first8=Adelheid|last9=Peters|first9=Christina|last10=Widhalm|first10=Georg|last11=Dorfer|first11=Christian|last12=Czech|first12=Thomas|title=Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012|journal=Cancer Medicine|volume=3|issue=1|year=2014|pages=91–100|issn=20457634|doi=10.1002/cam4.161}}</ref> Atypical teratoid rhabdoid tumor is demonstrated by positivity to [[tumor markers]] such as epithelial membrane antigen, [[vimentin]], and [[actin|smooth muscle actin]].<ref name=ihcatrt1>Markers of Atypical teratoid/rhabdoid tumour. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/atypical-teratoidrhabdoid-tumour. Accessed on December 14, 2015</ref><ref name="pmid16687525">{{cite journal| author=Meyers SP, Khademian ZP, Biegel JA, Chuang SH, Korones DN, Zimmerman RA| title=Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes. | journal=AJNR Am J Neuroradiol | year= 2006 | volume= 27 | issue= 5 | pages= 962-71 | pmid=16687525 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16687525 }} </ref> Atypical teratoid rhabdoid tumor must be differentiated from [[medulloblastoma]], [[pineoblastoma]], spinal primitive neuroectodermal tumors, and [[pineal gland tumor|other pineal gland masses]].<ref name=ddxatrt1>Differential diagnosis of Atypical teratoid/rhabdoid tumour. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/atypical-teratoidrhabdoid-tumour. Accessed on December 14, 2015</ref> Atypical teratoid rhabdoid tumor comprises approximately 1–2% of all pediatric brain tumors; however, in patients less than 3 years of age, this tumor accounts for up to 20% of cases.<ref name="GinnGajjar2012">{{cite journal|last1=Ginn|first1=Kevin F.|last2=Gajjar|first2=Amar|title=Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions|journal=Frontiers in Oncology|volume=2|year=2012|issn=2234-943X|doi=10.3389/fonc.2012.00114}}</ref> The incidence of atypical teratoid rhabdoid tumor is approximately 0.3 per 100,000 individuals in the United States.<ref name=atrtinci1>Atypical teratoid rhabdoid tumor. Wikipedia 2015. https://en.wikipedia.org/wiki/Atypical_teratoid_rhabdoid_tumor. Accessed on December 10, 2015</ref> Males are more commonly affected with atypical teratoid rhabdoid tumor than females. The male to female ratio is approximately 1.9 to 1.<ref name="GinnGajjar2012">{{cite journal|last1=Ginn|first1=Kevin F.|last2=Gajjar|first2=Amar|title=Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions|journal=Frontiers in Oncology|volume=2|year=2012|issn=2234-943X|doi=10.3389/fonc.2012.00114}}</ref> If left untreated, patients with atypical teratoid rhabdoid tumor may progress to develop [[obstructive hydrocephalus]], leptomeningeal dissemination, and ultimately death.<ref name="pmid22135607">{{cite journal| author=Chan KH, Mohammed Haspani MS, Tan YC, Kassim F| title=A case report of atypical teratoid/rhabdoid tumour in a 9-year-old girl. | journal=Malays J Med Sci | year= 2011 | volume= 18 | issue= 3 | pages= 82-6 | pmid=22135607 | doi= | pmc=PMC3216225 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22135607 }} </ref> Common complications of atypical teratoid rhabdoid tumor include [[obstructive hydrocephalus]], leptomeningeal dissemination, and recurrence.<ref name="pmid22135607">{{cite journal| author=Chan KH, Mohammed Haspani MS, Tan YC, Kassim F| title=A case report of atypical teratoid/rhabdoid tumour in a 9-year-old girl. | journal=Malays J Med Sci | year= 2011 | volume= 18 | issue= 3 | pages= 82-6 | pmid=22135607 | doi= | pmc=PMC3216225 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22135607 }} </ref> Prognosis is generally poor, and the 5-year overall survival rate of patients with atypical teratoid rhabdoid tumor is 29 ± 9%.<ref name="GinnGajjar2012">{{cite journal|last1=Ginn|first1=Kevin F.|last2=Gajjar|first2=Amar|title=Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions|journal=Frontiers in Oncology|volume=2|year=2012|issn=2234-943X|doi=10.3389/fonc.2012.00114}}</ref> Symptoms of atypical teratoid rhabdoid tumor include [[headache]], [[vomiting]], [[hydrocephalus|increased head size in infant]], [[ataxia|trouble with balance and coordination]], and [[weight loss]]. Common physical examination findings of atypical teratoid rhabdoid tumor include [[papilledema]], [[ataxia]], [[upper motor neuron lesion]], and [[hemiparesis]].<ref name="pmid22135607">{{cite journal| author=Chan KH, Mohammed Haspani MS, Tan YC, Kassim F| title=A case report of atypical teratoid/rhabdoid tumour in a 9-year-old girl. | journal=Malays J Med Sci | year= 2011 | volume= 18 | issue= 3 | pages= 82-6 | pmid=22135607 | doi= | pmc=PMC3216225 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22135607 }} </ref> On MRI, atypical teratoid rhabdoid tumor is characterized by iso- to slight hyperintensity on T1-weighted imaging and hyperintensity on T2-weighted imaging. There is heterogenous enhancement on contrast administration.<ref name=mriatrt1>MRI brain radiographic features of Atypical teratoid/rhabdoid tumour. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/atypical-teratoidrhabdoid-tumour. Accessed on December 16, 2015</ref> The predominant therapy for atypical teratoid rhabdoid tumor is surgical resection. Adjunctive chemotherapy and radiation is required.<ref name="SlavcChocholous2014">{{cite journal|last1=Slavc|first1=Irene|last2=Chocholous|first2=Monika|last3=Leiss|first3=Ulrike|last4=Haberler|first4=Christine|last5=Peyrl|first5=Andreas|last6=Azizi|first6=Amedeo A.|last7=Dieckmann|first7=Karin|last8=Woehrer|first8=Adelheid|last9=Peters|first9=Christina|last10=Widhalm|first10=Georg|last11=Dorfer|first11=Christian|last12=Czech|first12=Thomas|title=Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012|journal=Cancer Medicine|volume=3|issue=1|year=2014|pages=91–100|issn=20457634|doi=10.1002/cam4.161}}</ref><ref name=chemoatrt1>Treatment and care options of atypical teratoid rhabdoid tumor. Dana-Farber and Boston Children hospital cancer and blood disorder center 2015. http://www.danafarberbostonchildrens.org/conditions/brain-tumor/atypical-teratoid-rhabdoid-tumor.aspx. Accessed on December 21, 2015</ref> | |||
==Historical Perspective== | |||
Atypical teratoid rhabdoid tumor was first reported by Bonnin et al., in 1984.<ref name="GinnGajjar2012">{{cite journal|last1=Ginn|first1=Kevin F.|last2=Gajjar|first2=Amar|title=Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions|journal=Frontiers in Oncology|volume=2|year=2012|issn=2234-943X|doi=10.3389/fonc.2012.00114}}</ref> It was incorporated as a separate entity into the WHO classification of tumors of the nervous system, in 1993. | |||
==Classification== | |||
There is no classification system established for atypical teratoid rhabdoid tumor.<ref name=classatrt1>Classification of atypical teratoid rhabdoid tumor. Dana-Farber Cancer Institute 2015. http://www.dana-farber.org/Health-Library/Childhood-AT/RT---Atypical-Teratoid-Rhabdoid-Tumor.aspx. Accessed on December 14, 2015</ref> | |||
</ref> | ==Pathophysiology== | ||
Atypical teratoid rhabdoid tumor is comprised of rhabdoid tumor cells and varying amounts of small undifferentiated primitive neuroectodermal tumor (PNET)-like, mesenchymally, and/or epithelially differentiated tumor cells.<ref name="SlavcChocholous2014">{{cite journal|last1=Slavc|first1=Irene|last2=Chocholous|first2=Monika|last3=Leiss|first3=Ulrike|last4=Haberler|first4=Christine|last5=Peyrl|first5=Andreas|last6=Azizi|first6=Amedeo A.|last7=Dieckmann|first7=Karin|last8=Woehrer|first8=Adelheid|last9=Peters|first9=Christina|last10=Widhalm|first10=Georg|last11=Dorfer|first11=Christian|last12=Czech|first12=Thomas|title=Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012|journal=Cancer Medicine|volume=3|issue=1|year=2014|pages=91–100|issn=20457634|doi=10.1002/cam4.161}}</ref> Gene involved in the pathogenesis of atypical teratoid rhomboid tumor include ''[[SMARCB1]]'' (''hSNF5/INI-1''), a tumor suppressor gene.<ref name="GinnGajjar2012">{{cite journal|last1=Ginn|first1=Kevin F.|last2=Gajjar|first2=Amar|title=Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions|journal=Frontiers in Oncology|volume=2|year=2012|issn=2234-943X|doi=10.3389/fonc.2012.00114}}</ref> Atypical teratoid rhabdoid tumor may be associated with rhabdoid predisposition syndrome.<ref name="SlavcChocholous2014">{{cite journal|last1=Slavc|first1=Irene|last2=Chocholous|first2=Monika|last3=Leiss|first3=Ulrike|last4=Haberler|first4=Christine|last5=Peyrl|first5=Andreas|last6=Azizi|first6=Amedeo A.|last7=Dieckmann|first7=Karin|last8=Woehrer|first8=Adelheid|last9=Peters|first9=Christina|last10=Widhalm|first10=Georg|last11=Dorfer|first11=Christian|last12=Czech|first12=Thomas|title=Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012|journal=Cancer Medicine|volume=3|issue=1|year=2014|pages=91–100|issn=20457634|doi=10.1002/cam4.161}}</ref> On gross pathology, atypical teratoid rhabdoid tumor is characterized by an encapsulated, grayish, friable mass which is moderately vascular.<ref name="pmid22135607">{{cite journal| author=Chan KH, Mohammed Haspani MS, Tan YC, Kassim F| title=A case report of atypical teratoid/rhabdoid tumour in a 9-year-old girl. | journal=Malays J Med Sci | year= 2011 | volume= 18 | issue= 3 | pages= 82-6 | pmid=22135607 | doi= | pmc=PMC3216225 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22135607 }} </ref> On microscopic histopathological analysis, atypical teratoid rhabdoid tumor is characterized by round blue tumor cells of high cellularity composed of atypical cells with eccentric nuclei, small nucleoli, and abundant amounts of eosinophilic cytoplasm with frequent mitotic figures.<ref name="SlavcChocholous2014">{{cite journal|last1=Slavc|first1=Irene|last2=Chocholous|first2=Monika|last3=Leiss|first3=Ulrike|last4=Haberler|first4=Christine|last5=Peyrl|first5=Andreas|last6=Azizi|first6=Amedeo A.|last7=Dieckmann|first7=Karin|last8=Woehrer|first8=Adelheid|last9=Peters|first9=Christina|last10=Widhalm|first10=Georg|last11=Dorfer|first11=Christian|last12=Czech|first12=Thomas|title=Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012|journal=Cancer Medicine|volume=3|issue=1|year=2014|pages=91–100|issn=20457634|doi=10.1002/cam4.161}}</ref> Atypical teratoid rhabdoid tumor is demonstrated by positivity to [[tumor markers]] such as epithelial membrane antigen, [[vimentin]], and [[actin|smooth muscle actin]].<ref name=ihcatrt1>Markers of Atypical teratoid/rhabdoid tumour. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/atypical-teratoidrhabdoid-tumour. Accessed on December 14, 2015</ref><ref name="pmid16687525">{{cite journal| author=Meyers SP, Khademian ZP, Biegel JA, Chuang SH, Korones DN, Zimmerman RA| title=Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes. | journal=AJNR Am J Neuroradiol | year= 2006 | volume= 27 | issue= 5 | pages= 962-71 | pmid=16687525 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16687525 }} </ref> | |||
==Causes== | |||
There are no established direct causes for atypical teratoid rhabdoid tumor. The development of atypical teratoid rhabdoid tumor is the result of multiple [[mutation|genetic mutations]]. Common genetic mutations involved in the development of atypical teratoid rhabdoid tumor can be found [[Atypical teratoid rhabdoid tumor pathophysiology|'''here''']].<ref name="GinnGajjar2012">{{cite journal|last1=Ginn|first1=Kevin F.|last2=Gajjar|first2=Amar|title=Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions|journal=Frontiers in Oncology|volume=2|year=2012|issn=2234-943X|doi=10.3389/fonc.2012.00114}}</ref> | |||
==Differentiating Atypical Teratoid Rhabdoid Tumor from other Diseases== | |||
Atypical teratoid rhabdoid tumor must be differentiated from [[medulloblastoma]], [[pineoblastoma]], spinal primitive neuroectodermal tumors, and [[pineal gland tumor|other pineal gland masses]].<ref name=ddxatrt1>Differential diagnosis of Atypical teratoid/rhabdoid tumour. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/atypical-teratoidrhabdoid-tumour. Accessed on December 14, 2015</ref> | |||
==Epidemiology and Demographics== | |||
Atypical teratoid rhabdoid tumor comprises approximately 1–2% of all pediatric brain tumors; however, in patients less than 3 years of age, this tumor accounts for up to 20% of cases.<ref name="GinnGajjar2012">{{cite journal|last1=Ginn|first1=Kevin F.|last2=Gajjar|first2=Amar|title=Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions|journal=Frontiers in Oncology|volume=2|year=2012|issn=2234-943X|doi=10.3389/fonc.2012.00114}}</ref> The incidence of atypical teratoid rhabdoid tumor is approximately 0.3 per 100,000 individuals in the United States.<ref name=atrtinci1>Atypical teratoid rhabdoid tumor. Wikipedia 2015. https://en.wikipedia.org/wiki/Atypical_teratoid_rhabdoid_tumor. Accessed on December 10, 2015</ref> Atypical teratoid rhabdoid tumor is a rare disease that tends to affect the children population.<ref name=epidemioageatrt1>Epidemiology of atypical teratoid rhabdoid tumor. Dr Bruno Di Muzio and Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/atypical-teratoidrhabdoid-tumour. Accessed on December 10, 2015</ref><ref name=epioageatrt1>Clinical presentation of atypical teratoid rhabdoid tumor. National Cancer Institute 2015. http://www.cancer.gov/types/brain/hp/child-cns-atrt-treatment-pdq#link/_113_toc. Accessed on December 16, 2015</ref> The median age at diagnosis is approximately 24-30 months.<ref name="SlavcChocholous2014">{{cite journal|last1=Slavc|first1=Irene|last2=Chocholous|first2=Monika|last3=Leiss|first3=Ulrike|last4=Haberler|first4=Christine|last5=Peyrl|first5=Andreas|last6=Azizi|first6=Amedeo A.|last7=Dieckmann|first7=Karin|last8=Woehrer|first8=Adelheid|last9=Peters|first9=Christina|last10=Widhalm|first10=Georg|last11=Dorfer|first11=Christian|last12=Czech|first12=Thomas|title=Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012|journal=Cancer Medicine|volume=3|issue=1|year=2014|pages=91–100|issn=20457634|doi=10.1002/cam4.161}}</ref> Males are more commonly affected with atypical teratoid rhabdoid tumor than females. The male to female ratio is approximately 1.9 to 1.<ref name="GinnGajjar2012">{{cite journal|last1=Ginn|first1=Kevin F.|last2=Gajjar|first2=Amar|title=Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions|journal=Frontiers in Oncology|volume=2|year=2012|issn=2234-943X|doi=10.3389/fonc.2012.00114}}</ref> | |||
==Risk Factors== | |||
There are no established risk factors for atypical teratoid rhabdoid tumor. | |||
==Screening== | |||
There is insufficient evidence to recommend routine screening for atypical teratoid rhabdoid tumor. | |||
==Natural History, Complications and Prognosis== | |||
If left untreated, patients with atypical teratoid rhabdoid tumor may progress to develop [[obstructive hydrocephalus]], leptomeningeal dissemination, and ultimately death.<ref name="pmid22135607">{{cite journal| author=Chan KH, Mohammed Haspani MS, Tan YC, Kassim F| title=A case report of atypical teratoid/rhabdoid tumour in a 9-year-old girl. | journal=Malays J Med Sci | year= 2011 | volume= 18 | issue= 3 | pages= 82-6 | pmid=22135607 | doi= | pmc=PMC3216225 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22135607 }} </ref> Common complications of atypical teratoid rhabdoid tumor include [[obstructive hydrocephalus]], leptomeningeal dissemination, and recurrence.<ref name="pmid22135607">{{cite journal| author=Chan KH, Mohammed Haspani MS, Tan YC, Kassim F| title=A case report of atypical teratoid/rhabdoid tumour in a 9-year-old girl. | journal=Malays J Med Sci | year= 2011 | volume= 18 | issue= 3 | pages= 82-6 | pmid=22135607 | doi= | pmc=PMC3216225 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22135607 }} </ref> Prognosis is generally poor, and the 5-year overall survival rate of patients with atypical teratoid rhabdoid tumor is 29 ± 9%.<ref name="GinnGajjar2012">{{cite journal|last1=Ginn|first1=Kevin F.|last2=Gajjar|first2=Amar|title=Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions|journal=Frontiers in Oncology|volume=2|year=2012|issn=2234-943X|doi=10.3389/fonc.2012.00114}}</ref> | |||
==Diagnosis== | |||
===Diagnostic Criteria=== | |||
The diagnosis of atypical teratoid rhabdoid tumor is based on the current WHO classification of tumors of the central nervous system criteria, which include presence of rhabdoid tumor cells and/or divergent differentiation along [[epithelial]], [[mesenchymal]], [[neuronal]], or [[glial|glial lines]] in addition to the complete loss of [[SMARCB1]] protein expression in tumor cell nuclei, but the expression retained in preexisting cells (e.g., [[endothelial cells]]).<ref name="SlavcChocholous2014">{{cite journal|last1=Slavc|first1=Irene|last2=Chocholous|first2=Monika|last3=Leiss|first3=Ulrike|last4=Haberler|first4=Christine|last5=Peyrl|first5=Andreas|last6=Azizi|first6=Amedeo A.|last7=Dieckmann|first7=Karin|last8=Woehrer|first8=Adelheid|last9=Peters|first9=Christina|last10=Widhalm|first10=Georg|last11=Dorfer|first11=Christian|last12=Czech|first12=Thomas|title=Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012|journal=Cancer Medicine|volume=3|issue=1|year=2014|pages=91–100|issn=20457634|doi=10.1002/cam4.161}}</ref> | |||
===Staging=== | |||
There is no established system for the staging of atypical teratoid rhabdoid tumor. | |||
===History and Symptoms=== | |||
When evaluating a patient for atypical teratoid rhabdoid tumor, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough family and past medical history review. Other specific areas of focus when obtaining the history include the review of associated conditions such as rhabdoid predisposition syndrome.<ref name="SlavcChocholous2014">{{cite journal|last1=Slavc|first1=Irene|last2=Chocholous|first2=Monika|last3=Leiss|first3=Ulrike|last4=Haberler|first4=Christine|last5=Peyrl|first5=Andreas|last6=Azizi|first6=Amedeo A.|last7=Dieckmann|first7=Karin|last8=Woehrer|first8=Adelheid|last9=Peters|first9=Christina|last10=Widhalm|first10=Georg|last11=Dorfer|first11=Christian|last12=Czech|first12=Thomas|title=Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012|journal=Cancer Medicine|volume=3|issue=1|year=2014|pages=91–100|issn=20457634|doi=10.1002/cam4.161}}</ref> Symptoms of atypical teratoid rhabdoid tumor include [[headache]], [[vomiting]], [[hydrocephalus|increased head size in infant]], [[ataxia|trouble with balance and coordination]], and [[weight loss]].<ref name="pmid22135607">{{cite journal| author=Chan KH, Mohammed Haspani MS, Tan YC, Kassim F| title=A case report of atypical teratoid/rhabdoid tumour in a 9-year-old girl. | journal=Malays J Med Sci | year= 2011 | volume= 18 | issue= 3 | pages= 82-6 | pmid=22135607 | doi= | pmc=PMC3216225 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22135607 }} </ref><ref name="UdakaShayan2013">{{cite journal|last1=Udaka|first1=Y. T.|last2=Shayan|first2=K.|last3=Chuang|first3=N. A.|last4=Crawford|first4=J. R.|title=Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child|journal=Case Reports in Oncological Medicine|volume=2013|year=2013|pages=1–4|issn=2090-6706|doi=10.1155/2013/815923}}</ref><ref name=symptomsatrt1>Symptoms of atypical teratoid rhabdoid tumor. Dana-Farber Cancer Institute 2015. http://www.dana-farber.org/Health-Library/Childhood-AT/RT---Atypical-Teratoid-Rhabdoid-Tumor.aspx. Accessed on December 14, 2015</ref> | |||
===Physical Examination=== | |||
Common physical examination findings of atypical teratoid rhabdoid tumor include [[papilledema]], [[ataxia]], [[upper motor neuron lesion]], and [[hemiparesis]].<ref name="pmid22135607">{{cite journal| author=Chan KH, Mohammed Haspani MS, Tan YC, Kassim F| title=A case report of atypical teratoid/rhabdoid tumour in a 9-year-old girl. | journal=Malays J Med Sci | year= 2011 | volume= 18 | issue= 3 | pages= 82-6 | pmid=22135607 | doi= | pmc=PMC3216225 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22135607 }} </ref> | |||
===Laboratory Findings=== | |||
[[CSF analysis]] is done to detect any leptomeningeal dissemination with involvement of the cerebrospinal fluid. Large tumor cells, eccentricity of the nuclei, and prominent nucleoli are consistent findings.<ref name=lab1atrt>{{cite journal|author=Lu L, Wilkinson EJ, Yachnis AT|title=CSF cytology of atypical teratoid/rhabdoid tumor of the brain in a two-year-old girl: a case report|journal=Diagn. Cytopathol.|volume=23|issue=5|pages=329–32|year=2000|pmid=11074628}}</ref> | |||
===CT=== | |||
Head CT scan is helpful in the diagnosis of atypical teratoid rhabdoid tumor. On head CT scan, atypical teratoid rhabdoid tumor is characterized by an isodense, calcified mass with heterogenous enhancement. There may be associated findings of [[obstructive hydrocephalus]].<ref name=ctatrt1>CT brain radiographic features of Atypical teratoid/rhabdoid tumour. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/atypical-teratoidrhabdoid-tumour. Accessed on December 16, 2015</ref> | |||
===MRI=== | |||
Brain MRI is helpful in the diagnosis of atypical teratoid rhabdoid tumor. On MRI, atypical teratoid rhabdoid tumor is characterized by iso- to slight hyperintensity on T1-weighted imaging and hyperintensity on T2-weighted imaging. There is heterogenous enhancement on contrast administration.<ref name=mriatrt1>MRI brain radiographic features of Atypical teratoid/rhabdoid tumour. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/atypical-teratoidrhabdoid-tumour. Accessed on December 16, 2015</ref> | |||
===Ultrasound=== | |||
Ultrasound may be performed to detect atypical teratoid rhabdoid tumor in other parts of the body, especially the kidneys.<ref name=usgatrat1>Diagnosis of atypical teratoid rhabdoid tumor. Dana-Farber Cancer Institute 2015. http://www.dana-farber.org/Health-Library/Childhood-AT/RT---Atypical-Teratoid-Rhabdoid-Tumor.aspx. Accessed on December 16, 2015</ref> | |||
===Other Imaging Findings=== | |||
Other imaging studies for atypical teratoid rhabdoid tumor include [[Nuclear magnetic resonance spectroscopy|magnetic resonance spectroscopy]], which demonstrates elevated [[choline]] and [[N-Acetylaspartate]].<ref name=mriatrt1>MRI brain radiographic features of Atypical teratoid/rhabdoid tumour. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/atypical-teratoidrhabdoid-tumour. Accessed on December 16, 2015</ref> | |||
===Other Diagnostic Studies=== | |||
Other diagnostic studies for atypical teratoid rhabdoid tumor include [[biopsy]] (definitive diagnostic test), fluorescence in situ hybridization (abnormalities of [[22q11.2 deletion syndrome|chromosome 22q11.2]]), and immunohistochemistry (loss of INI-1 staining in the neoplastic cells).<ref name=biopsyatrt1>Diagnostic evaluation of atypical teratoid rhabdoid tumor. National Cancer Institute 2015. http://www.cancer.gov/types/brain/hp/child-cns-atrt-treatment-pdq#link/_113_toc. Accessed on December 16, 2015</ref><ref name="UdakaShayan2013">{{cite journal|last1=Udaka|first1=Y. T.|last2=Shayan|first2=K.|last3=Chuang|first3=N. A.|last4=Crawford|first4=J. R.|title=Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child|journal=Case Reports in Oncological Medicine|volume=2013|year=2013|pages=1–4|issn=2090-6706|doi=10.1155/2013/815923}}</ref> | |||
==Treatment== | |||
===Medical Therapy=== | |||
The predominant therapy for atypical teratoid rhabdoid tumor is surgical resection. Adjunctive chemotherapy and radiation is required.<ref name="SlavcChocholous2014">{{cite journal|last1=Slavc|first1=Irene|last2=Chocholous|first2=Monika|last3=Leiss|first3=Ulrike|last4=Haberler|first4=Christine|last5=Peyrl|first5=Andreas|last6=Azizi|first6=Amedeo A.|last7=Dieckmann|first7=Karin|last8=Woehrer|first8=Adelheid|last9=Peters|first9=Christina|last10=Widhalm|first10=Georg|last11=Dorfer|first11=Christian|last12=Czech|first12=Thomas|title=Atypical teratoid rhabdoid tumor: improved long-term survival with an intensive multimodal therapy and delayed radiotherapy. The Medical University of Vienna Experience 1992-2012|journal=Cancer Medicine|volume=3|issue=1|year=2014|pages=91–100|issn=20457634|doi=10.1002/cam4.161}}</ref><ref name=chemoatrt1>Treatment and care options of atypical teratoid rhabdoid tumor. Dana-Farber and Boston Children hospital cancer and blood disorder center 2015. http://www.danafarberbostonchildrens.org/conditions/brain-tumor/atypical-teratoid-rhabdoid-tumor.aspx. Accessed on December 21, 2015</ref> | |||
===Surgery=== | |||
[[Surgery]] is the mainstay of treatment for atypical teratoid rhabdoid tumor.<ref name="GinnGajjar2012">{{cite journal|last1=Ginn|first1=Kevin F.|last2=Gajjar|first2=Amar|title=Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions|journal=Frontiers in Oncology|volume=2|year=2012|issn=2234-943X|doi=10.3389/fonc.2012.00114}}</ref> Surgery plays a critical role in obtaining [[Tissue (biology)|tissue]] to make an accurate [[diagnosis]]. Stem cell transplantation is indicated in very aggressive atypical teratoid rhabdoid tumors, to receive high doses of chemotherapy.<ref name=chemoatrt1>Treatment and care options of atypical teratoid rhabdoid tumor. Dana-Farber and Boston Children hospital cancer and blood disorder center 2015. http://www.danafarberbostonchildrens.org/conditions/brain-tumor/atypical-teratoid-rhabdoid-tumor.aspx. Accessed on December 21, 2015</ref> | |||
===Primary Prevention=== | |||
There are no primary preventive measures available for atypical teratoid rhabdoid tumor. | |||
==References== | ==References== | ||
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Latest revision as of 15:35, 22 December 2015
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Atypical teratoid rhabdoid tumor Microchapters |
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Differentiating Atypical Teratoid Rhabdoid Tumor from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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Atypical teratoid rhabdoid tumor overview On the Web |
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American Roentgen Ray Society Images of Atypical teratoid rhabdoid tumor overview |
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Directions to Hospitals Treating Atypical teratoid rhabdoid tumor |
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Risk calculators and risk factors for Atypical teratoid rhabdoid tumor overview |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Overview
Atypical teratoid rhabdoid tumor is an uncommon WHO Grade IV tumor, which in the vast majority of cases occurs in young children less than two years of age. It most frequently presents as a posterior fossa mass. Atypical teratoid rhabdoid tumor is comprised of rhabdoid tumor cells and varying amounts of small undifferentiated primitive neuroectodermal tumor (PNET)-like, mesenchymally, and/or epithelially differentiated tumor cells. Gene involved in the pathogenesis of atypical teratoid rhomboid tumor include SMARCB1 (hSNF5/INI-1), a tumor suppressor gene.[1] Atypical teratoid rhabdoid tumor may be associated with rhabdoid predisposition syndrome.[2] On gross pathology, atypical teratoid rhabdoid tumor is characterized by an encapsulated, grayish, friable mass which is moderately vascular.[3] On microscopic histopathological analysis, atypical teratoid rhabdoid tumor is characterized by round blue tumor cells of high cellularity composed of atypical cells with eccentric nuclei, small nucleoli, and abundant amounts of eosinophilic cytoplasm with frequent mitotic figures.[2] Atypical teratoid rhabdoid tumor is demonstrated by positivity to tumor markers such as epithelial membrane antigen, vimentin, and smooth muscle actin.[4][5] Atypical teratoid rhabdoid tumor must be differentiated from medulloblastoma, pineoblastoma, spinal primitive neuroectodermal tumors, and other pineal gland masses.[6] Atypical teratoid rhabdoid tumor comprises approximately 1–2% of all pediatric brain tumors; however, in patients less than 3 years of age, this tumor accounts for up to 20% of cases.[1] The incidence of atypical teratoid rhabdoid tumor is approximately 0.3 per 100,000 individuals in the United States.[7] Males are more commonly affected with atypical teratoid rhabdoid tumor than females. The male to female ratio is approximately 1.9 to 1.[1] If left untreated, patients with atypical teratoid rhabdoid tumor may progress to develop obstructive hydrocephalus, leptomeningeal dissemination, and ultimately death.[3] Common complications of atypical teratoid rhabdoid tumor include obstructive hydrocephalus, leptomeningeal dissemination, and recurrence.[3] Prognosis is generally poor, and the 5-year overall survival rate of patients with atypical teratoid rhabdoid tumor is 29 ± 9%.[1] Symptoms of atypical teratoid rhabdoid tumor include headache, vomiting, increased head size in infant, trouble with balance and coordination, and weight loss. Common physical examination findings of atypical teratoid rhabdoid tumor include papilledema, ataxia, upper motor neuron lesion, and hemiparesis.[3] On MRI, atypical teratoid rhabdoid tumor is characterized by iso- to slight hyperintensity on T1-weighted imaging and hyperintensity on T2-weighted imaging. There is heterogenous enhancement on contrast administration.[8] The predominant therapy for atypical teratoid rhabdoid tumor is surgical resection. Adjunctive chemotherapy and radiation is required.[2][9]
Historical Perspective
Atypical teratoid rhabdoid tumor was first reported by Bonnin et al., in 1984.[1] It was incorporated as a separate entity into the WHO classification of tumors of the nervous system, in 1993.
Classification
There is no classification system established for atypical teratoid rhabdoid tumor.[10]
Pathophysiology
Atypical teratoid rhabdoid tumor is comprised of rhabdoid tumor cells and varying amounts of small undifferentiated primitive neuroectodermal tumor (PNET)-like, mesenchymally, and/or epithelially differentiated tumor cells.[2] Gene involved in the pathogenesis of atypical teratoid rhomboid tumor include SMARCB1 (hSNF5/INI-1), a tumor suppressor gene.[1] Atypical teratoid rhabdoid tumor may be associated with rhabdoid predisposition syndrome.[2] On gross pathology, atypical teratoid rhabdoid tumor is characterized by an encapsulated, grayish, friable mass which is moderately vascular.[3] On microscopic histopathological analysis, atypical teratoid rhabdoid tumor is characterized by round blue tumor cells of high cellularity composed of atypical cells with eccentric nuclei, small nucleoli, and abundant amounts of eosinophilic cytoplasm with frequent mitotic figures.[2] Atypical teratoid rhabdoid tumor is demonstrated by positivity to tumor markers such as epithelial membrane antigen, vimentin, and smooth muscle actin.[4][5]
Causes
There are no established direct causes for atypical teratoid rhabdoid tumor. The development of atypical teratoid rhabdoid tumor is the result of multiple genetic mutations. Common genetic mutations involved in the development of atypical teratoid rhabdoid tumor can be found here.[1]
Differentiating Atypical Teratoid Rhabdoid Tumor from other Diseases
Atypical teratoid rhabdoid tumor must be differentiated from medulloblastoma, pineoblastoma, spinal primitive neuroectodermal tumors, and other pineal gland masses.[6]
Epidemiology and Demographics
Atypical teratoid rhabdoid tumor comprises approximately 1–2% of all pediatric brain tumors; however, in patients less than 3 years of age, this tumor accounts for up to 20% of cases.[1] The incidence of atypical teratoid rhabdoid tumor is approximately 0.3 per 100,000 individuals in the United States.[7] Atypical teratoid rhabdoid tumor is a rare disease that tends to affect the children population.[11][12] The median age at diagnosis is approximately 24-30 months.[2] Males are more commonly affected with atypical teratoid rhabdoid tumor than females. The male to female ratio is approximately 1.9 to 1.[1]
Risk Factors
There are no established risk factors for atypical teratoid rhabdoid tumor.
Screening
There is insufficient evidence to recommend routine screening for atypical teratoid rhabdoid tumor.
Natural History, Complications and Prognosis
If left untreated, patients with atypical teratoid rhabdoid tumor may progress to develop obstructive hydrocephalus, leptomeningeal dissemination, and ultimately death.[3] Common complications of atypical teratoid rhabdoid tumor include obstructive hydrocephalus, leptomeningeal dissemination, and recurrence.[3] Prognosis is generally poor, and the 5-year overall survival rate of patients with atypical teratoid rhabdoid tumor is 29 ± 9%.[1]
Diagnosis
Diagnostic Criteria
The diagnosis of atypical teratoid rhabdoid tumor is based on the current WHO classification of tumors of the central nervous system criteria, which include presence of rhabdoid tumor cells and/or divergent differentiation along epithelial, mesenchymal, neuronal, or glial lines in addition to the complete loss of SMARCB1 protein expression in tumor cell nuclei, but the expression retained in preexisting cells (e.g., endothelial cells).[2]
Staging
There is no established system for the staging of atypical teratoid rhabdoid tumor.
History and Symptoms
When evaluating a patient for atypical teratoid rhabdoid tumor, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough family and past medical history review. Other specific areas of focus when obtaining the history include the review of associated conditions such as rhabdoid predisposition syndrome.[2] Symptoms of atypical teratoid rhabdoid tumor include headache, vomiting, increased head size in infant, trouble with balance and coordination, and weight loss.[3][13][14]
Physical Examination
Common physical examination findings of atypical teratoid rhabdoid tumor include papilledema, ataxia, upper motor neuron lesion, and hemiparesis.[3]
Laboratory Findings
CSF analysis is done to detect any leptomeningeal dissemination with involvement of the cerebrospinal fluid. Large tumor cells, eccentricity of the nuclei, and prominent nucleoli are consistent findings.[15]
CT
Head CT scan is helpful in the diagnosis of atypical teratoid rhabdoid tumor. On head CT scan, atypical teratoid rhabdoid tumor is characterized by an isodense, calcified mass with heterogenous enhancement. There may be associated findings of obstructive hydrocephalus.[16]
MRI
Brain MRI is helpful in the diagnosis of atypical teratoid rhabdoid tumor. On MRI, atypical teratoid rhabdoid tumor is characterized by iso- to slight hyperintensity on T1-weighted imaging and hyperintensity on T2-weighted imaging. There is heterogenous enhancement on contrast administration.[8]
Ultrasound
Ultrasound may be performed to detect atypical teratoid rhabdoid tumor in other parts of the body, especially the kidneys.[17]
Other Imaging Findings
Other imaging studies for atypical teratoid rhabdoid tumor include magnetic resonance spectroscopy, which demonstrates elevated choline and N-Acetylaspartate.[8]
Other Diagnostic Studies
Other diagnostic studies for atypical teratoid rhabdoid tumor include biopsy (definitive diagnostic test), fluorescence in situ hybridization (abnormalities of chromosome 22q11.2), and immunohistochemistry (loss of INI-1 staining in the neoplastic cells).[18][13]
Treatment
Medical Therapy
The predominant therapy for atypical teratoid rhabdoid tumor is surgical resection. Adjunctive chemotherapy and radiation is required.[2][9]
Surgery
Surgery is the mainstay of treatment for atypical teratoid rhabdoid tumor.[1] Surgery plays a critical role in obtaining tissue to make an accurate diagnosis. Stem cell transplantation is indicated in very aggressive atypical teratoid rhabdoid tumors, to receive high doses of chemotherapy.[9]
Primary Prevention
There are no primary preventive measures available for atypical teratoid rhabdoid tumor.
References
- ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 Ginn, Kevin F.; Gajjar, Amar (2012). "Atypical Teratoid Rhabdoid Tumor: Current Therapy and Future Directions". Frontiers in Oncology. 2. doi:10.3389/fonc.2012.00114. ISSN 2234-943X.
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 2.9 Slavc, Irene; Chocholous, Monika; Leiss, Ulrike; Haberler, Christine; Peyrl, Andreas; Azizi, Amedeo A.; Dieckmann, Karin; Woehrer, Adelheid; Peters, Christina; Widhalm, Georg; Dorfer, Christian; Czech, Thomas (2014). "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 Medicine. 3 (1): 91–100. doi:10.1002/cam4.161. ISSN 2045-7634.
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 3.8 Chan KH, Mohammed Haspani MS, Tan YC, Kassim F (2011). "A case report of atypical teratoid/rhabdoid tumour in a 9-year-old girl". Malays J Med Sci. 18 (3): 82–6. PMC 3216225. PMID 22135607.
- ↑ 4.0 4.1 Markers of Atypical teratoid/rhabdoid tumour. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/atypical-teratoidrhabdoid-tumour. Accessed on December 14, 2015
- ↑ 5.0 5.1 Meyers SP, Khademian ZP, Biegel JA, Chuang SH, Korones DN, Zimmerman RA (2006). "Primary intracranial atypical teratoid/rhabdoid tumors of infancy and childhood: MRI features and patient outcomes". AJNR Am J Neuroradiol. 27 (5): 962–71. PMID 16687525.
- ↑ 6.0 6.1 Differential diagnosis of Atypical teratoid/rhabdoid tumour. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/atypical-teratoidrhabdoid-tumour. Accessed on December 14, 2015
- ↑ 7.0 7.1 Atypical teratoid rhabdoid tumor. Wikipedia 2015. https://en.wikipedia.org/wiki/Atypical_teratoid_rhabdoid_tumor. Accessed on December 10, 2015
- ↑ 8.0 8.1 8.2 MRI brain radiographic features of Atypical teratoid/rhabdoid tumour. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/atypical-teratoidrhabdoid-tumour. Accessed on December 16, 2015
- ↑ 9.0 9.1 9.2 Treatment and care options of atypical teratoid rhabdoid tumor. Dana-Farber and Boston Children hospital cancer and blood disorder center 2015. http://www.danafarberbostonchildrens.org/conditions/brain-tumor/atypical-teratoid-rhabdoid-tumor.aspx. Accessed on December 21, 2015
- ↑ Classification of atypical teratoid rhabdoid tumor. Dana-Farber Cancer Institute 2015. http://www.dana-farber.org/Health-Library/Childhood-AT/RT---Atypical-Teratoid-Rhabdoid-Tumor.aspx. Accessed on December 14, 2015
- ↑ Epidemiology of atypical teratoid rhabdoid tumor. Dr Bruno Di Muzio and Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/atypical-teratoidrhabdoid-tumour. Accessed on December 10, 2015
- ↑ Clinical presentation of atypical teratoid rhabdoid tumor. National Cancer Institute 2015. http://www.cancer.gov/types/brain/hp/child-cns-atrt-treatment-pdq#link/_113_toc. Accessed on December 16, 2015
- ↑ 13.0 13.1 Udaka, Y. T.; Shayan, K.; Chuang, N. A.; Crawford, J. R. (2013). "Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child". Case Reports in Oncological Medicine. 2013: 1–4. doi:10.1155/2013/815923. ISSN 2090-6706.
- ↑ Symptoms of atypical teratoid rhabdoid tumor. Dana-Farber Cancer Institute 2015. http://www.dana-farber.org/Health-Library/Childhood-AT/RT---Atypical-Teratoid-Rhabdoid-Tumor.aspx. Accessed on December 14, 2015
- ↑ Lu L, Wilkinson EJ, Yachnis AT (2000). "CSF cytology of atypical teratoid/rhabdoid tumor of the brain in a two-year-old girl: a case report". Diagn. Cytopathol. 23 (5): 329–32. PMID 11074628.
- ↑ CT brain radiographic features of Atypical teratoid/rhabdoid tumour. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/atypical-teratoidrhabdoid-tumour. Accessed on December 16, 2015
- ↑ Diagnosis of atypical teratoid rhabdoid tumor. Dana-Farber Cancer Institute 2015. http://www.dana-farber.org/Health-Library/Childhood-AT/RT---Atypical-Teratoid-Rhabdoid-Tumor.aspx. Accessed on December 16, 2015
- ↑ Diagnostic evaluation of atypical teratoid rhabdoid tumor. National Cancer Institute 2015. http://www.cancer.gov/types/brain/hp/child-cns-atrt-treatment-pdq#link/_113_toc. Accessed on December 16, 2015