Biomarkers in Tissue Samples From Patients With High-Risk Wilms Tumor

OBJECTIVES:

I. To assess genomic gains and losses in high risk renal tumors, including up to 80
favorable histology Wilms tumors that relapse (RFHWT), 50 anaplastic Wilms tumors (UHWT), 15
clear cell sarcomas of the kidney (CCSK), and 40 rhabdoid tumors (RT) using a high density
genetic platform to survey for recurrent copy number variations and allelic imbalances. II.
To define transcription patterns within 80 RFHWT, 50 UHWT, 15 CCSK, and 40 RT using a high
throughput platform for global gene expression. III. To define DNA methylation patterns
within 80 RFHWT, 50 UHWT, 15 CCSK, and 40 RT using a high throughput platform. IV. To
identify genetic mutations involved in the pathogenesis of Wilms tumor, and in the
development of relapse and anaplasia through the study of 80 RFHWT, 50 UHWT, 15 CCSK, and 40
RT using next generation sequencing tools.

V. To facilitate the integration of the above databases and allow meaningful access by
investigators through the infrastructure provided by TARGET, including its data portal and
associated caBIG tool.

OUTLINE: This is a multicenter study.

Archived tumor tissue samples are analyzed for DNA copy number determination, gene
expression, DNA methylation, and genomic re-sequencing by array-based methods, including PCR
analysis, methylation-specific reverse transcriptase-PCR (RT-PCR), and quantitative RT-PCR.

Inclusion Criteria:

- Diagnosis of high-risk Wilms tumor meeting ≥ 1 of the following criteria:

- Relapsed disease

- Anaplastic disease

- Clear cell sarcomas of the kidney

- Rhabdoid tumors

- Registered on NWTS-4, NWTS-5 (now COG-Q9401), or participation in AREN03B2 protocols
with clinical follow-up > 3 years

- Banked frozen tumor samples and paired normal DNA available with clinical data
points, including the following:

- Age, race, and gender

- Stage and reason for stage

- Tumor weight

- Associated precursor lesions (rests)

- Histologic subtype

- Site and time of recurrence

- Days of follow-up

- Time and reasons for death (e.g., tumor, toxicity, infection, or other)