Atypical teratoid rhabdoid tumor: long-term survival after chemoradiotherapy



Atypical teratoid rhabdoid tumors (ATRTs) arise from the central nervous system largely in the pediatric population. They portend a very poor prognosis with few long-term survivors. We describe a series of five cases at our institution.


We conducted a retrospective chart review and clinical follow-up.


Three patients underwent chemoradiation after surgical resection; the two patients whose caretakers declined this therapy passed away soon after diagnosis. Chemoradiation included intravenous and intrathecal chemotherapy as well as intensity-modulated radiotherapy after resection. Of the patients receiving chemoradiation, two patients had infratentorial tumors, two had gross residual tumor after resection, and two were under the age of 3 years. The three patients receiving trimodality therapy remain clinically and symptomatically disease-free with follow-up times of 44, 46, and 55 months. Two of the patients have mild neuropsychiatric sequelae after therapy.


Long-term, high-volume trials of ATRT are currently not published. We offer experience in successful long-term survival of this tumor treated with chemoradiotherapy.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8


  1. 1.

    Woehrer A, Slavc I, Waldhoer T, Heinzl H, Zielonke N, Czech T et al (2010) Austrian brain tumor registry. Cancer 116:5725–5732

    PubMed  Article  Google Scholar 

  2. 2.

    Rorke LB, Packer RJ, Biegel JA (1996) Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: definition of an entity. J Neurosurg 85:56–65

    CAS  PubMed  Article  Google Scholar 

  3. 3.

    Buscariollo DL, Park HS, Roberts KB, Yu JB (2012) Survival outcomes in atypical teratoid rhabdoid tumor for patients undergoing radiotherapy in a surveillance, epidemiology, and end results analysis. Cancer 118:4212–4219

    PubMed  Article  Google Scholar 

  4. 4.

    Chen YW, Wong TT, Ho DM, Huang PI, Chang KP, Shiau CY et al (2006) Impact of radiotherapy for pediatric CNS atypical teratoid/rhabdoid tumor (single institute experience). Int J Radiat Oncol Biol Phys 15:1038–1043

    Article  Google Scholar 

  5. 5.

    Finkelstein-Shechter T, Gassas A, Mabbott D, Huang A, Bartels U, Tabori U et al (2010) Atypical teratoid or rhabdoid tumors: improved outcome with high-dose chemotherapy. J Pediatr Hematol Oncol 32:e182–e186

    PubMed  Article  Google Scholar 

  6. 6.

    Lafay-Cousin L, Hawkins C, Carret AS, Johnston D, Zelcer S, Wilson B et al (2012) Central nervous system atypical teratoid rhabdoid tumours: the Canadian Paediatric Brain Tumour Consortium experience. Eur J Cancer 48:353–359

    CAS  PubMed  Article  Google Scholar 

  7. 7.

    Tekautz TM, Fuller CE, Blaney S, Fouladi M, Broniscer A, Merchant TE et al (2005) Atypical teratoid/rhabdoid tumors (ATRT): improved survival in children 3 years of age and older with radiation therapy and high-dose alkylator-based chemotherapy. J Clin Oncol 23:1491–1499

    CAS  PubMed  Article  Google Scholar 

  8. 8.

    Chi SN, Zimmerman MA, Yao X, Cohen KJ, Burger P, Biegel JA et al (2009) Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol 27:385–389

    PubMed Central  PubMed  Article  Google Scholar 

  9. 9.

    Bouvier C, De Paula AM, Fernandez C, Quilichini B, Scavarda D, Gentet JC et al (2008) Atypical teratoid/rhabdoid tumour: 7-year event-free survival with gross total resection and radiotherapy in a 7-year-old boy. Childs Nerv Syst 24:143–147

    CAS  PubMed  Article  Google Scholar 

  10. 10.

    von Hoff K, Hinkes B, Dannenmann-Stern E, von Bueren AO, Warmuth-Metz M, Soerensen N et al (2011) Frequency, risk-factors and survival of children with atypical teratoid rhabdoid tumors (AT/RT) of the CNS diagnosed between 1988 and 2004, and registered to the German HIT database. Pediatr Blood Cancer 57:978–985

    Article  Google Scholar 

  11. 11.

    Athale UH, Duckworth J, Odame I, Barr R (2009) Childhood atypical teratoid rhabdoid tumor of the central nervous system: a meta-analysis of observational studies. J Pediatr Hematol Oncol 31:651–663

    PubMed  Article  Google Scholar 

  12. 12.

    Slavc I, Chocholous M, Leiss U, Haberler C, Peyrl A, Azizi AA et al (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 Med 3:91–100

    PubMed Central  PubMed  Article  Google Scholar 

  13. 13.

    Takahashi-Fujigasaki J, Matumoto M, Kan I, Oka H, Yasue M (2012) Atypical teratoid/rhabdoid tumor with 26-year overall survival: case report. J Neurosurg Pediatr 9:400–405

    PubMed  Article  Google Scholar 

  14. 14.

    Pai Panandiker AS, Merchant TE, Beltran C, Wu S, Sharma S, Boop FA et al (2012) Sequencing of local therapy affects the pattern of treatment failure and survival in children with atypical teratoid rhabdoid tumors of the central nervous system. Int J Radiat Oncol Biol Phys 82:1756–1763

    PubMed Central  PubMed  Article  Google Scholar 

  15. 15.

    Hirth A, Pedersen PH, Wester K, Mork S, Helgestad J (2003) Cerebral atypical teratoid/rhabdoid tumor of infancy: long-term survival after multimodal treatment, also including triple intrathecal chemotherapy and gamma knife radiosurgery—case report. Pediatr Hematol Oncol 20:327–332

    CAS  PubMed  Article  Google Scholar 

  16. 16.

    De Amorim Bernstein K, Sethi R, Trofimov A, Zeng C, Fullerton B, Yeap BY (2013) Early clinical outcomes using proton radiation for children with central nervous system atypical teratoid rhabdoid tumors. Int J Radiat Oncol Biol Phys 86:114–120

    PubMed  Article  Google Scholar 

Download references



Ethical approval

For this type of study, formal consent is not required. This article does not contain any studies with human participants or animals performed by any of the authors.

Conflict of interest

The authors all declare that conflicts of interest do not exist.

Author information



Corresponding author

Correspondence to Chi Lin.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Verma, V., Johnson, C.P., Bennion, N.R. et al. Atypical teratoid rhabdoid tumor: long-term survival after chemoradiotherapy. Childs Nerv Syst 31, 1393–1399 (2015).

Download citation


  • Atypical teratoid rhabdoid tumor
  • Pediatric brain tumors
  • Radiotherapy
  • Chemotherapy