IRB Study Number CC226
Status Recruiting
Institute Taussig Cancer Institute
Description
1.1 Primary Objectives
1.1.1 To classify patients with renal tumors by histological categorization, surgicopathological stage, presence of metastases, age at diagnosis, tumor weight and loss of heterozygosity for chromosomes 1p and 16q, to thereby define eligibility for a series of therapeutic studies.
1.1.2 To maintain a biological samples bank to make specimens available to scientists to evaluate additional potential biological prognostic variables and for the conduct of other research by scientists.
1.2 Secondary Objectives
1.2.1 To monitor outcome for those patients who are not eligible for a subsequent therapeutic study.
1.2.2 To describe whether the pulmonary tumor burden correlates with outcome in Stage IV patients.
1.2.3 To describe the sensitivity and specificity of abdominal computed tomography (CT) by comparison with surgical and pathologic findings for identification of local tumor spread beyond the renal capsule to adjacent muscle and organs, lymph node involvement at the renal hilum and in the retroperitoneum, preoperative tumor rupture and metastases to the liver.
1.2.4 To compare the sensitivity and specificity of preoperative abdominal CT and MRI for the identification and differentiation of nephrogenic rests and Wilms tumor in children with multiple renal lesions.
1.2.5 To correlate the method of conception (natural versus assisted reproductive technology) with the development of Wilms tumor.
1.2.6 To evaluate the frequency of INI1 mutations in renal and extrarenal malignant rhabdoid tumor of the kidney and to determine the incidence of germline and inherited versus somatic mutations to facilitate clinical correlations on the companion study AREN0321.
Inclusion Criteria
3.2.1 Diagnosis
3.2.1.1 Renal tumors
Patients with the first occurrence of any tumor of the kidney identified on CT scan or MRI are eligible for enrollment on the banking component of this study.
As of Amendment 8, only patients with an institutional diagnosis of Stage I-IV focal or diffuse anaplasia will be eligible for central review and return of an Initial Risk Assignment and/or subsequent central pathology review. All other patients will not undergo a central review of pathology, imaging, or surgical reports, and Initial Risk Assignment will not be entered. These patients will be eligible only for the banking portion of the study.
Eligible tumors include (but are not limited to):
Nephroblastic Tumors
Nephroblastoma (Wilms Tumor)
• Favorable histology
• Anaplasia (Diffuse, Focal)
Nephrogenic rests and Nephroblastomatosis
Cystic Nephroma and Cystic Partially Differentiated Nephroblastoma
Metanephric Tumors
• Metanephric Adenoma
• Metanephric Adenofibroma
• Metanephric Stromal Tumor
Mesoblastic Nephroma
Cellular, Classic, Mixed
Clear Cell Sarcoma
Rhabdoid Tumor
Renal Epithelioid Tumors of Childhood
Papillary renal cell carcinoma
Renal medullary carcinoma
Renal tumors associated with Xp11.2 translocations
Oncocytic renal neoplasms following neuroblastoma
Angiolipoma
Ossifying renal tumor of infancy
3.2.1.2 Extrarenal tumors
Patients with the first occurrence of the following tumors are also eligible:
• Extrarenal nephroblastoma or extrarenal nephrogenic rests
• Malignant rhabdoid tumor occurring anywhere outside the Central Nervous System
3.2.2 Required Submissions
Required specimens, reports, forms, and copies of imaging studies must be available or will become available for submission and the institution must intend on submitting them as described in the protocol procedures.
For ALL patients, the following submissions are required:
• A complete set of recut H & E slides (including from sampled lymph nodes, if patient had upfront nephrectomy)* #
• Representative formalin-fixed paraffin-embedded tissue block or if a block is unavailable, 10 unstained slides from a representative block of tumor, if available.*
• Institutional pathology report, Specimen Transmittal Form, and Pre-Treatment Pathology Checklist
• Copies of images and institutional reports of CT and/or MRI abdomen and pelvis, and Pre-Treatment Imaging Checklist
• Copies of images and institutional report of chest CT for all malignant tumors
• Institutional surgical report(s) and Pre-Treatment Surgical Checklist
• CRFs: Staging Checklist and Metastatic Disease Form (if metastatic disease is noted on imaging)
* Tissue must be from diagnosis, prior to any renal tumor directed chemotherapy or radiation (only exception is for presumed FHWT patients discovered to have FAWT or DAWT at delayed nephrectomy and plan to enroll at delayed nephrectomy or second biopsy, see Section 3.1.5.1.1).
If there are concerns with limited availability of diagnostic slides, please contact the study chair or study pathologist.
For patients with bilateral, bilaterally predisposed, multicentric, or unilateral tumor in solitary kidney, required slides and tissue may be submitted for banking at the time of their first post-chemotherapy surgical procedure.
Please note: if the above required items are not received within 120 days of study enrollment, the patient will be considered off study per Section 7.1 criteria.
3.2.3 Age
Patients must be < 30 years old at the time of diagnosis.
3.2.4 Regulatory
All patients and/or their parents or legal guardians must sign a written informed consent.
All institutional, FDA, and NCI requirements for human studies must be met.
Exclusion Criteria
Exclusion Criteria Not Available
