Treatment of Newly Diagnosed Patient's With Wilm's Tumor Requiring Abdominal Radiation Delivered With Proton Beam Irradiation

Inclusion Criteria:

• Patients are eligible to be enrolled on this trial at the time of initial (orpresumptive) diagnosis of Wilms tumor, at the time of surgery or at the time ofradiation treatment.

• Patients that are determined to be stage I or II will be eligible for the surgicaland biologic aspects of this trial but will otherwise be followed per institutionalstandards and be "off therapy" at the time of stage determination (followed only forsurvival).

• Patients identified to have an anaplastic or other unfavorable tissue component (non-Wilms histology) to their tumor may have tissue assessed for the biologyobjective but will be removed from the therapeutic portion of the study at the timeof identification of this pathologic finding.

Performance Level • The Karnofsky performance status must be ≥50 for patients >16 years of age and the Lansky performance status must be ≥ 50 for patients ≤ 16 years of age.

Prior Therapy

• Only prior therapy with the initial chemotherapy regimen defined in section 5.1 andAppendix III are allowed for patients that are treated with neoadjuvantchemotherapy. These patients may change to an alternate regime based on response orbiologic features and noted in section 5.1. Prior biopsy or surgery is allowed.

• Prior radiation therapy is not allowed if directed at the abdomen. Emergent RT toother sites of disease is acceptable.

Organ Function Requirements:

• None - As per institutional standard of care. Diagnosis / Stage

• Presumed diagnosis of Wilms tumor (continued management on trial will depend onstage and histology)

• Resected upfront stage III, IV or V favorable histology Wilms tumor. Stage IIIincludes any one or multiples of the following:

• Lymph nodes within the abdomen or pelvis are involved by tumor. (Lymph nodeinvolvement in the thorax, or other extra-abdominal sites is a criterion forStage IV)

• The tumor has penetrated through the peritoneal surface

• Tumor implants are found on the peritoneal surface

• Gross or microscopic tumor remains post-operatively (e.g., tumor cells arefound at the margin of surgical resection on microscopic examination - in thepost chemotherapy setting only specific types of viable tumor at the surgicalmargin are considered stage III AND require adjuvant radiation - see surgery /pathology / radiation section for additional details)

• The tumor is not completely resectable because of local infiltration into vitalstructures

• Tumor spillage occurring either before or during surgery

• A trans abdominal biopsy (regardless of type- tru-cut, open or fine needleaspiration) was performed prior to resection or chemotherapy. Retroperitonealbiopsies DO NOT require radiotherapeutic management like stage III

• Tumor is removed in greater than one piece (e.g. tumor cells are found in aseparately excised adrenal gland; a tumor thrombus within the renal vein isremoved separately from the nephrectomy specimen). Extension of the primarytumor within vena cava into thoracic vena cava and heart is considered StageIII, rather than Stage IV even though outside the abdomen.

• Stage IV - Hematogenous metastases (lung, liver, bone, brain, etc.), or lymph nodemetastases outside the abdomino-pelvic region are present. (The presence of tumorwithin the adrenal gland is not interpreted as metastasis and staging depends on allother staging parameters present). Patients with lung involvement and localabdominal stage I or II are eligible to remain on trial for whole lung irradiationobjective.

• Stage V - Bilateral renal involvement by tumor is present at diagnosis. An attemptshould be made to stage each side according to the above criteria on the basis ofthe extent of disease

• Patients that have or will receive neoadjuvant chemotherapy for unresectedunilateral or bilateral Wilms tumor without a biopsy are not assumed to be requireadjuvant radiation on this study. That determination will be based on surgicalfindings and the approach to treatment detailed in the protocol.

Timing

• Patients undergoing upfront surgery must start RT, if indicated, within 28 days ofdefinitive surgery with a goal of starting RT by day 21.

• Patients receiving neoadjuvant chemotherapy will be enrolled either at diagnosis (ifat St. Jude) or at the time of RT.

Other Criteria

• Female patients of childbearing potential (age ≥10 years old or post-menarche) must have a negative pregnancy test prior to enrollment.

Exclusion Criteria:

• Inability or unwillingness to provide written informed consent

• Prior radiation therapy to a site to be treated with proton / photon radiation aspart of this trial. Emergent radiation is allowed.