Malignant triton tumor in a patient with Li-Fraumeni syndrome and a novel TP53 mutation.
Pediatr Blood Cancer
; 49(7): 1000-4, 2007 Dec.
Article
en En
| MEDLINE
| ID: mdl-16333835
We report a 3-year-old boy with a malignant triton tumor (MTT) involving the left masticator space with local invasion and regional lymph node metastasis. Family history and detection of a novel germline TP53 mutation confirmed his diagnosis of Li Fraumeni syndrome (LFS). MTT has not been previously described in association with LFS. This case along with a comprehensive review of the literature, illustrate the importance of both somatic and germline TP53 mutations in the pathogenesis MTT. The tumor could not be resected and he was successfully treated with intensive induction chemotherapy, irradiation, and high-dose chemotherapy with autologous stem cell transplantation.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Neoplasias Nasales
/
Proteína p53 Supresora de Tumor
/
Síndrome de Li-Fraumeni
/
Neoplasias de la Vaina del Nervio
/
Mutación de Línea Germinal
Tipo de estudio:
Diagnostic_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Límite:
Child, preschool
/
Humans
/
Male
Idioma:
En
Revista:
Pediatr Blood Cancer
Asunto de la revista:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Año:
2007
Tipo del documento:
Article
País de afiliación:
Estados Unidos
Pais de publicación:
Estados Unidos