Skip Navigation LinksHome > May 2013 - Volume 35 - Issue 4 > Ataxia-Telangiectasia and Wilms Tumor: Reduced Treatment Bu...
Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e31828fccdf
Clinical and Laboratory Observations

Ataxia-Telangiectasia and Wilms Tumor: Reduced Treatment But Early Relapse

Pérez-Villena, Ana MD*; Cormenzana, María MD*; Prada, Inmaculada de MD; Pérez-Martínez, Antonio MD, PhD*; Aleo, Esther MD, PhD*

Collapse Box

Abstract

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.

© 2013 Lippincott Williams & Wilkins, Inc.

Login

Article Tools

Share

Search for Similar Articles
You may search for similar articles that contain these same keywords or you may modify the keyword list to augment your search.

Connect With Us

Twitter
twitter.com/JPHOonline

For additional oncology content, visit LWW Oncology Journals on Facebook.