Two cases of an atypical teratoid rhabdoid tumour of the CNS and literature review

Czech version

Authors: H. Zítek ¹; D. Sumerauer ²; J. Zámečník ³; P. Vachata ¹; M. Sameš ¹; A. Puchmajerová ⁴
Authors‘ workplace: Neurochirugická klinika UJEP a Masarykovy nemocnice Ústí nad Labem ¹; Klinika dětské hematologie a onkologie 2. LF UK a FN Motol ²; Ústav patologie a molekulární medicíny 2. LF UK a FN Motol ³; Ústav biologie a lékařské genetiky 2. LF UK a FN Motol ⁴
Published in: Cesk Slov Neurol N 2017; 80/113(Online only): 0
Category: No. 5

Overview

Atypical teratoid rhabdoid tumour is a highly malignant tumor of central nervous system predominantly affecting children under 3 years of age. It is a rare yet severe condition.

Diagnostics of this entity might be problematic due to unspecific histological and imiging findings which may mimic characteristics of other pediatric brain tumors. Therefore, exact methods of immunohistochemistry and molecular biology are required for the final diagnosis.

Historically, this condition has been associated with poor prognosis. Fortunately, recent studies have brought more optimistic results, specifically with the use of intensive multimodal therapy containing a combination of surgical resection, chemotherapy including intrathecal administration and focal radiotherapy.

The authors present two cases recently treated at our department as well as a review of scientific literature concerning this issue.

Key words:
atypical teratoid rhabdoid tumor – pediatric embryonal tumor – central nervous systém – primary resection – molecular a immunohistochemical diagnostics – multimodal adjuvant therapy

Sources

1. Buscariollo DL, Park HS, Roberts KB, et al. Survival outcomes in atypical teratoid rhabdoid tumor for patients undergoing radiotherapy in a Surveillance, Epidemiology, and End Results analysis. Cancer 2012;118(17):4212–19. doi: 10.1002/cncr.27373.

2. Schrey D, Carceller Lechón F, Malietzis G, et al. Multimodal therapy in children and adolescents with newly diagnosed atypical teratoid rhabdoid tumor: individual pooled data analysis and review of the literature. J Neurooncol 2016;126(1):81–90. doi: 10.1007/s11060-015-1904-0.

3. Slavc I, Chocholous M, Leiss U, 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 2014;3(1):91–100. doi: 10.1002/cam4.161.

4. DiPatri AJ, Sredni ST, Grahovac G, et al. Atypical teratoid rhabdoid tumors of the posterior fossa in children. Childs Nerv Syst 2015;31(10):1717–28. doi: 10.1007/s00381-015-2844-x.

5. Ostrom QT, Chen Y, M de Blank P, et al. The descriptive epidemiology of atypical teratoid/rhabdoid tumors in the United States, 2001-2010. Neuro Oncol 2014;16(10):1392–9. doi: 10.1093/neuonc/nou090.

6. Lafay-Cousin L, Hawkins C, Carret AS, et al. Central nervous system atypical teratoid rhabdoid tumours: the Canadian Paediatric Brain Tumour Consortium experience. Eur J Cancer 2012;48(3):353–9. doi: 10.1016/j.ejca.2011.09.005.

7. Dufour C, Beaugrand A, Le Deley MC, et al. Clinicopathologic prognostic factors in childhood atypical teratoid and rhabdoid tumor of the central nervous system: a multicenter study. Cancer 2012;118(15):3812–21. doi: 10.1002/cncr.26684.

8. Chi SN, Zimmerman MA, Yao X, et al. Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol 2009;27(3):385–9. doi: 10.1200/JCO.2008.18.7724.

9. von Hoff K, Hinkes B, Dannenmann-Stern E, et al. 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 2011 Dec 1;57(6):978–85. doi: 10.1002/pbc.23236.

10. Pai Panandiker AS, Merchant TE, Beltran C, et al. 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 2012;82(5):1756–63. doi: 10.1016/j.ijrobp.2011.02.059.

Labels

Paediatric neurology Neurosurgery Neurology

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Article was published in

Czech and Slovak Neurology and Neurosurgery

2017 Issue Online only