Favorable outcome of patients affected by rhabdoid tumors due to rhabdoid tumor predisposition syndrome (RTPS).
Pediatr Blood Cancer
; 61(5): 919-21, 2014 May.
Article
en En
| MEDLINE
| ID: mdl-24123847
Rhabdoid tumor predisposition syndrome is usually associated with shorter survival in patients with malignant rhabdoid tumors regardless of anatomical origin. Here we present four children harboring truncating heterozygous SMARCB1/INI1 germline mutations with favorable outcome. All four patients received multi-modality treatment, three according to therapeutic recommendations by the EU-RHAB registry, two without radiotherapy, and mean event-free survival accounts for 7 years. In conclusion, intensive treatment with curative intent is justified for children with rhabdoid tumors even if an underlying rhabdoid predisposition syndrome is demonstrated.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Factores de Transcripción
/
Proteínas Cromosómicas no Histona
/
Tumor Rabdoide
/
Mutación de Línea Germinal
/
Predisposición Genética a la Enfermedad
/
Proteínas de Unión al ADN
Tipo de estudio:
Diagnostic_studies
/
Guideline
Límite:
Child
/
Child, preschool
/
Female
/
Humans
/
Male
/
Newborn
Idioma:
En
Revista:
Pediatr Blood Cancer
Asunto de la revista:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Año:
2014
Tipo del documento:
Article
País de afiliación:
Alemania
Pais de publicación:
Estados Unidos