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Ataxia-telangiectasia and wilms tumor: reduced treatment but early relapse.
Pérez-Villena, Ana; Cormenzana, María; de Prada, Inmaculada; Pérez-Martínez, Antonio; Aleo, Esther.
Afiliación
  • Pérez-Villena A; Divisions of Paediatric Haematology and Oncology, Hospital Infantil Universitario Niño Jesús, Madrid, Spain.
J Pediatr Hematol Oncol ; 35(4): 308-10, 2013 May.
Article en En | MEDLINE | ID: mdl-23612382
Ataxia-telangiectasia (A-T) is an autosomal recessive disease characterized by progressive cerebellar ataxia, oculocutaneous telangiectasia, immunodeficiency, a high incidence of lymphoreticular tumors, and an increased sensitivity to chemoradiotherapy-induced DNA damage. The appropriate cancer therapy remains unknown because of high toxicity rates with full-dose conventional protocols. We present a patient with A-T and nephroblastoma, who received an adapted treatment regimen. To our knowledge this is the second report on nephroblastoma in a patient with A-T but the first with confirmed premortem studies. Although the patient tolerated the chemotherapy regimen well, the patient relapsed and died a year after initial diagnosis.
Asunto(s)

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Ataxia Telangiectasia / Tumor de Wilms / Neoplasias Renales Tipo de estudio: Guideline Límite: Child, preschool / Female / Humans Idioma: En Revista: J Pediatr Hematol Oncol Asunto de la revista: HEMATOLOGIA / NEOPLASIAS / PEDIATRIA Año: 2013 Tipo del documento: Article País de afiliación: España Pais de publicación: Estados Unidos

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Ataxia Telangiectasia / Tumor de Wilms / Neoplasias Renales Tipo de estudio: Guideline Límite: Child, preschool / Female / Humans Idioma: En Revista: J Pediatr Hematol Oncol Asunto de la revista: HEMATOLOGIA / NEOPLASIAS / PEDIATRIA Año: 2013 Tipo del documento: Article País de afiliación: España Pais de publicación: Estados Unidos