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

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

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

Prognosis of atypical teratoid rhabdoid tumors (AT/RT) treated with multimodal therapy protocols. Report of our series.

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.

Congenital Peripheral Vestibular Syndrome in a Domestic Ferret (Mustela putorius furo).

A 3-month-old intact female ferret (Mustela putorius furo) was presented with a 2-month history of ataxia. On physical examination, the ferret had difficulty standing upright. During the neurologic examination, the patient had a left head tilt and positional strabismus, circled to the left, and was ataxic. Results of the complete blood count were consistent with a mild normocytic normochromic anemia. Initial treatment was supportive. Magnetic resonance imaging was performed and revealed an asymmetry of the inner ears. A brainstem auditory evoked response test was also performed. History, clinical signs, and diagnostic test results indicated that the ferret was suffering from congenital peripheral vestibular syndrome and left-sided deafness. Congenital disease should be considered in the differential diagnosis of young ferrets with peripheral vestibular syndrome. Supportive care and physiotherapy can improve balance and motor function, leading to an acceptable quality of life.