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{{DiseaseDisorder infobox |
__NOTOC__
  Name          = Atypical teratoid rhabdoid tumor |
  ICD10          = {{ICD10|C|70||c|69}}-{{ICD10|C|72||c|69}} |
  ICD9          = {{ICD9|191}}-{{ICD9|192}}  |
  Image          = ATRT-MRI.jpg|
  Caption        = [[Magnetic resonance imaging|MRI]] of an AT/RT|
  ICDO          = 9508/3 |
  OMIM          = 609322|
  DiseasesDB    = 30780|
  MedlinePlus    = 000768 |
  MeshID        = D016543  |
}}
{{Atypical teratoid rhabdoid tumor}}
{{Atypical teratoid rhabdoid tumor}}
{{CMG}}
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''


{{CMG}}{{AE}}{{SR}}{{Sab}}


==Overview==
{{SK}} ATRT; AT/RT; Atypical teratoid rhabdoid tumour; Atypical teratoid/Rhabdoid tumor; Atypical teratoid/Rhabdoid tumour; Primary brain tumor; Brain tumour
'''Atypical teratoid rhabdoid tumor''' ('''AT/RT''') is a rare [[tumor]] usually diagnosed in childhood.  Although usually a [[brain tumor]], AT/RT can occur anywhere in the [[central nervous system]] (CNS) including the [[spinal cord]].  About 60% will be in the [[posterior cranial fossa]] (particularly the [[cerebellum]]).  One review estimated 52% posterior fossa (PF), 39% sPNET ([[supratentorial]] [[primitive neuroectodermal tumor| primitive neuroectodermal tumors]]), 5% [[pineal gland|pineal]], 2% [[Spinal tumor|spinal]], and 2% multi-focal.<ref name="Kieran">{{cite journal
  | author =  Kieran MW
  | title =  An Update on Germ Cell Tumors, Atypical Teratoid/Rhaboid Tumors, and Choroid Plexus Tumors Rare Tumors 3: Brain Tumors---Germ Cell Tumors, Atypical Teratoid/Rhabdoid Tumors, and Choroid Plexus Tumors
  | journal =  American Society of Clinical Oncology
  | volume = Education Book
  | year =  2006
  | url =  http://www.asco.org/portal/site/ASCO/menuitem.64cfbd0f85cb37b2eda2be0aee37a01d/?vgnextoid=09f8201eb61a7010VgnVCM100000ed730ad1RCRD&vmview=vm_session_presentations_view&confID=40&sessionID=295
  | accessdate = 2007-05-20 }}
</ref>


==Genetics==
==[[Atypical teratoid rhabdoid tumor overview|Overview]]==


== [[Atypical teratoid rhabdoid tumor historical perspective|Historical Perspective]] ==


== [[Atypical teratoid rhabdoid tumor classification|Classification]]==


==Diagnosis==
== [[Atypical teratoid rhabdoid tumor pathophysiology|Pathophysiology]] ==


The standard work-up for AT/RT includes:
== [[Atypical teratoid rhabdoid tumor causes|Causes]] ==


* MRI [[Magnetic resonance imaging]] of the brain and spine
== [[Atypical teratoid rhabdoid tumor differential diagnosis|Differentiating Atypical Teratoid Rhabdoid Tumor from other Diseases]] ==
* LP ([[Lumbar puncture]]) to look for M1 disease
* CT ([[Computed tomography]]) of chest and abdomen to check for a tumor
* BMA ([[Bone marrow examination|Bone Marrow Asperant]]) to check for bone tumors.  Often a doctor will want perform a [[Hematopoietic stem cell transplantation|stem cell transplant]]
* BX [[Bone marrow examination|Bone marrow biopsy]]
* [[Bone scan]]


==Treatment==
== [[Atypical teratoid rhabdoid tumor epidemiology and demographics|Epidemiology and Demographics]] ==
==Prognosis==


== [[Atypical teratoid rhabdoid tumor risk factors|Risk Factors]] ==


==References==
== [[Atypical teratoid rhabdoid tumor screening|Screening]] ==
{{reflist|2}}
 
== [[Atypical teratoid rhabdoid tumor natural history, complications and prognosis|Natural History, Complications and Prognosis]] ==
 
== Diagnosis ==
 
[[Atypical teratoid rhabdoid tumor diagnostic criteria|Diagnostic Criteria]] | [[Atypical teratoid rhabdoid tumor staging|Staging]] | [[Atypical teratoid rhabdoid tumor history and symptoms|History and Symptoms]] | [[Atypical teratoid rhabdoid tumor physical examination|Physical Examination]] | [[Atypical teratoid rhabdoid tumor laboratory findings|Laboratory Findings]] | [[Atypical teratoid rhabdoid tumor CT|CT]] | [[Atypical teratoid rhabdoid tumor MRI|MRI]] | [[Atypical teratoid rhabdoid tumor echocardiography or ultrasound|Ultrasound]] | [[Atypical teratoid rhabdoid tumor other imaging findings|Other Imaging Findings]] | [[Atypical teratoid rhabdoid tumor other diagnostic studies|Other Diagnostic Studies]]
 
== Treatment ==
 
[[Atypical teratoid rhabdoid tumor medical therapy|Medical Therapy]] | [[Atypical teratoid rhabdoid tumor surgery|Surgery]] | [[Atypical teratoid rhabdoid tumor prevention|Prevention]] | [[Atypical teratoid rhabdoid tumor cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Atypical teratoid rhabdoid tumor future or investigational therapies|Future or Investigational Therapies]]
 
== Case Studies ==
 
[[Atypical teratoid rhabdoid tumor case study one|Case #1]]


==External links==
* [http://cms.clevelandclinic.org/childrenshospital/body.cfm?id=471 The Rhabdoid Registry]
* {{cite journal
  | authors =  Hilden JM, Meerbaum S, Burger P, et al
  | title =  Central Nervous System Atypical Teratoid/Rhabdoid Tumor: Results of Therapy in Children Enrolled in a Registry
  | journal = Journal of Clinical Oncology
  | volume =  22
  | issue =  14
  | pages =  2877–84
  | date =  [[2004-07-15]]
  | url =  http://jco.ascopubs.org/cgi/content/full/22/14/2877?ck=nck
  | doi = 10.1200/JCO.2004.07.073
  | accessdate = 2007-05-23}}
*{{cite journal
| authors = Beigel J, Kalpana G, Knudsen E, et al.
| year = 2002
| title = The role of INI and the SWI/SNF complex in the development of rhabdoid tumors:  Meeting Summary from the workshop on childhood atypical teratoid rhabdoid tumors
| journal =  Cancer Research 2002
| issue = 63:
| pages = 323–28
| url = http://cancerres.aacrjournals.org/cgi/content/abstract/62/1/323
}}
*{{cite journal
  | author = Huret J, Sevenet N
  | title = Rhabdoid predisposition syndrome
  | year = 2002
  | journal = Atlas of Genetics and Cytogenetics in Oncology and Haematology
  | url  =  http://www.infobiogen.fr/services/chromcancer/Kprones/rhabdKpronID10051.html
  }}
{{Nervous tissue tumors}}
{{Nervous tissue tumors}}


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[[pl:Atypowy nowotwór teratoidny (rabdoidny)]]
[[pl:Atypowy nowotwór teratoidny (rabdoidny)]]


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[[Category:Medicine]]Atypical teratoid rhabdoid tumor (patient information)

Latest revision as of 19:37, 31 July 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]Sabawoon Mirwais, M.B.B.S, M.D.[3]

Synonyms and keywords: ATRT; AT/RT; Atypical teratoid rhabdoid tumour; Atypical teratoid/Rhabdoid tumor; Atypical teratoid/Rhabdoid tumour; Primary brain tumor; Brain tumour

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Atypical Teratoid Rhabdoid Tumor from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria | Staging | History and Symptoms | Physical Examination | Laboratory Findings | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

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Case Studies

Case #1

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