DESCRIPTION: (Adapted from the Applicant's Abstract): This proposal is to
continue the follow-up of children and young adults who were treated
successfully for Wilms tumor on protocol studies of the National Wilms Tumor
Study Group (NWTSG). Patients will be contacted annually and those who
become lost will be actively traced. Information on health status and key
events will be obtained from periodic physical exams and medical histories,
with medical records used to confirm reproductive outcomes and key
diagnoses. The goals are to help elucidate the genetic events involved in
the pathogenesis of Wilms tumor, to estimate the risk of adverse pregnancy
outcomes and congenital defects in offspring, and to determine the risks of
second malignant neoplasms, congestive heart failure and renal failure in
survivors.Adjuvant treatment for Wilms tumor involves the combination of
several chemotherapeutic agents with or without radiation therapy. This
facilitates comparison of rates of key events among randomized subgroups of
patients that differ with respect to the dose and duration of use of
radiation and specific drugs. Data on the occurrence of Wilms tumor in
family members and offspring will facilitate testing of specific genetic
models and the estimation of recurrence risks for use by clinical
geneticists in counselling affected families. Although initially believed
to exemplify Knudson's two stage mutational model in a manner similar to
retinoblastoma, the genetics of Wilms tumor is now recognized to be more
complex and heterogeneous. The proposed epidemiologic studies will provide
insights regarding carcinogenic mechanisms that can be used to orient and
interpret the laboratory studies. Collaboration with groups of molecular
biologists, providing them national access to informative subgroups of
patients, will speed their discoveries.
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