Atypical Teratoid / Rhaboid Tumour (AT/RT)

Overview


Tumour Group: Pediatric Brain Tumours
WHO Grade: Grade IV
Prevalence/Incidence: AT/RTs are rare, accounting for 1 to 2% of pediatric brain tumours. However, due to the number of cases in children under the age of three, AT/RT are estimated to account for at least 20% of central nervous system (CNS) tumours in infants.
Typical Age Range: These tumours typically occur in children ages 3 and younger.

Contents
Description of Tumour
Symptoms
Treatment / Standard of Care
Prognosis
References

Description of Tumour


AT/RT is a very aggressive brain tumour seen in infants and young children. The tumour is characterized through a mutation of the tumour suppressor gene INI1. Genetic predisposition (germline mutation) can be found in up to 35% of patients. Therefore genetic testing for patients and parents is warranted.

About half of AT/RTs are found in the cerebellum or brainstem. However, they may appear anywhere in the brain or spinal cord. An AT/RT can appear as a large, bulky mass.

Symptoms


Common symptoms include, but are not necessarily limited to:

  • Change in activity level
  • Increased head size (in infants)
  • Lack of coordination
  • Loss of balance
  • Morning headache or headache that goes away after vomiting
  • Nausea and vomiting
  • Trouble walking and regression of milestones
  • Unusual sleepiness

Treatment / Standard of Care


Currently there are no standard-of-care treatment protocols available for AT/RT. Treatment generally involves several approaches including surgery to remove the tumour, followed by chemotherapy and/or high-dose chemotherapy, and stem cell transplant. Frequently focal radiation is also considered as part of the treatment plan.

Prognosis  


A prognosis is an estimate of the likely progress of a disease after a diagnosis, based on an average patient group. Since every person is different, please take time to talk with your health care team about how this information applies to you.

By clicking on 'Expand,' a statistic on the prognosis for atypical teratoid / rhabdoid tumours will be shown.

Expand for Prognosis Information

For brain tumour patients, a prognosis depends on several factors, which can include age and other health issues, the size of the tumour, its molecular profile, the type of tumour, how much can be removed and its response to treatments such as chemotherapy and radiation therapy.

Because most patients with AT/RT are less than three years of age, the overall prognosis for AT/RT is very poor. However, with recent advances in more innovative and intensive treatment approaches, the outcome appears to be improving.

References


Atypical teratoid/rhabdoid tumor: short clinical description and insight into possible mechanism of the disease. Bikowska B, Grajkowska W, Jóźwiak J. Eur J Neurol. 2011 Jun;18(6):813-8.

Atypical teratoid rhabdoid tumor: current therapy and future directions. Ginn KF, Gajjar A. Front Oncol. 2012;2:114.

"Atypical Teratoid Rhaboid Tumour (AT / RT)." www.abta.org. American Brain Tumour Association, n.d. Web. 11 July 2013.

"Atypical Teratoid Rhabdoid Tumour." Wikipedia. Wikimedia Foundation, 07 Mar. 2013. Web. 11 July 2013.

Central nervous system atypical teratoid rhabdoid tumours: the Canadian Paediatric Brain Tumour Consortium experience. Lafay-Cousin L, Hawkins C, Carret AS, Johnston D, Zelcer S, Wilson B, Jabado N, Scheinemann K, Eisenstat D, Fryer C, Fleming A, Mpofu C, Larouche V, Strother D, Bouffet E, Huang A. Eur J Cancer. 2012 Feb;48(3):353-9.

Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. Chi SN, Zimmerman MA, Yao X, Cohen KJ, Burger P, Biegel JA, Rorke-Adams LB, Fisher MJ, Janss A, Mazewski C, Goldman S, Manley PE, Bowers DC, Bendel A, Rubin J, Turner CD, Marcus KJ, Goumnerova L, Ullrich NJ, Kieran MW.J Clin Oncol. 2009 Jan 20;27(3):385-9.

Louis, David N. WHO Classification of Tumours of the Central Nervous System. Lyon: International Agency for Research on Cancer, 2007. Print

Pediatric Brain Tumour Handbook 6th Edition. Brain Tumour Foundation of Canada. 2012.

Image credited to Marvin 101 (Own work) [GFDL (http://www.gnu.org/copyleft/fdl.html) or CC-BY-SA-3.0-2.5-2.0-1.0 (http://creativecommons.org/licenses/by-sa/3.0)], via Wikimedia Commons
 

 You can also download this information as a Information Sheet on Atypical Teratoid/Rhaboid Tumour (pdf).

 

For Calgary researcher Dr. Aru Narendran, knowing first-hand what the brain tumour journey can mean for families motivates him to spend his days in a lab. There he and his team search for a cure for atypical teratoid / rhabdoid tumour (AT/RT). Learn more about Dr. Narendran's commitment to finding a cure >>

 

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