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Vol. 27. Num. 2.March - April 2016
Pages 51-102
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Vol. 27. Num. 2.March - April 2016
Pages 51-102
Case Report
DOI: 10.1016/j.neucir.2015.01.003
Prognosis of atypical teratoid rhabdoid tumors (AT/RT) treated with multimodal therapy protocols. Report of our series
Pronóstico de los tumores teratoides rabdoides atípicos (TT/RA) tratados con protocolos de terapia multimodal. Descripción de nuestra serie
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Alfonso Valencia-Moyaa, Laura González-Garcíab,
Corresponding author
gonzalezgarcialaura@hotmail.com

Corresponding author.
, Bienvenido Ros-Lópezb, Tomás Acha-Garcíac, Bernardo Weil-Larad, Pablo Obando-Pachecoc, Miguel Ángel Arráez-Sánchezb
a Neurosurgery Department, Gómez Ulla Hospital, Madrid, Spain
b Neurosurgery Department, Carlos Haya University Hospital, Málaga, Spain
c Oncology Department, Carlos Haya University Hospital, Málaga, Spain
d Pathology Department, Carlos Haya University Hospital, Málaga, Spain
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Table 1. Review of the literature.
Abstract
Objective

Atypical teratoid rhabdoid tumors (AT/RT) of the central nervous system are rare, very aggressive embryological tumors, typically diagnosed in young patients and having a low survival rate after diagnosis. The aim of this study was to emphasize, based on the latest results in the literature, the need for protocols for multidisciplinary treatment in these patients.

Material and methods

We report our series of 3 cases treated, diagnosed and followed up between 2009 and 2014. They were treated with multimodal therapy protocols (Rhabdoid SIOP-2007 and European Rhabdoid Registry EU-RHAB-2010). In addition, we carried out a literature review.

Results

Two of our 3 cases (supratentorial and spinal tumors) did not show any progression of the disease after long follow-up, in contrast with most of the cases available in the literature. The second patient had a shorter survival.

Conclusions

Patient age at the time of diagnosis, supratentorial location of the mass and fewer complications with adjuvant treatments seem to be factors yielding good prognosis for AT/RT tumors. In agreement with the latest international protocols, multidisciplinary treatment is the ideal treatment, consisting of radiotherapy and chemotherapy after complete tumor resection.

Keywords:
AT/RT
Atypical teratoid rhabdoid tumor
Brain tumor
Pediatric patients
Multidisciplinary treatment
Abbreviations:
AT/RT
DOX
DWI
EGFR
EFS
EMA
GFAP
ICE
SMA
VCA
VCD
Resumen
Objetivo

Los tumores teratoides rabdoides atípicos (TT/RA) del sistema nervioso central son tumores embrionarios muy agresivos, de baja incidencia, típicamente diagnosticados en pacientes jóvenes, con una baja supervivencia tras el diagnóstico. El objetivo de este estudio es resaltar la necesidad del tratamiento multidisciplinar protocolizado de estos pacientes sobre la base de los resultados más actuales de la literatura.

Material y métodos

Presentamos nuestra serie de 3 casos de TT/RA diagnosticados, tratados y seguidos entre 2009 y 2014. Nuestros pacientes se trataron siguiendo los protocolos de terapia multimodal (Rhabdoid SIOP-2007, European Rhabdoid Registry EU-RHAB-2010). De forma adicional, se realizó una revisión de la literatura.

Resultados

Dos de nuestros pacientes (lesiones supratentorial y espinal) no presentaron progresión de la enfermedad años tras el diagnóstico, en comparación con lo descrito en la literatura. Por otra parte, el segundo paciente presentó una supervivencia menor.

Conclusiones

Pueden considerarse factores de buen pronóstico: la edad del paciente en el momento del diagnóstico, las lesiones supratentoriales, y las escasas complicaciones de los tratamientos adyuvantes. El consenso actual en cuanto al tratamiento idóneo consiste en cirugía, seguida de quimioterapia y radioterapia.

Palabras clave:
TT/RA
Tumor teratoide rabdoide atípico
Tumor cerebral
Pacientes pediátricos
Tratamiento multidisciplinar

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