AREN03B2: Renal Tumors Classification, Biology and Banking

Study ID
STU 092010-004

Cancer Related

Healthy Volunteers

Study Sites

  • Children’s Medical Center (Dallas, Plano, Southlake)


Principal Investigator
Jonathan Wickiser, M.D.


Collect tissue and peripheral blood samples of patients with kidney tumors for immediate use to analyze biological factors. obtain imaging studies (CT, MRi, ultrasound) during routine care to find out the location and exact size of the tumor. Furthermore, to deposit in a tissue bank the leftover tissue and/or blood samples and urine samples collected. Samples will be collected at the time of initial diagnosis, during treatment, at the time of surgical procedure, at the completion of therapy, one year following therapy completion, and at relapse/progression. These samples will be obtained when other samples are obtained for standard care. These samples can be used for approved projects by laboratory-based investigators. The tissue, blood and urine submitted for review will be labeled with the participant's name, medical record number, date of birth, date of procedure, and the unique identifiers provided by CoG. any cells or Dna given to other scientists for future research tests will not be labeled with the participant's name or any other information that could be linked to him/her in any way. information about the participant's disease is linked to the samples in the cell bank, but no personal information will be disclosed to anyone doing research on the samples. only the main offices of the Children's oncology Group will know the name or other information linked to the individual number on the sample of cells. CoG plans to keep track of the participant's medical condition for about ten years following enrollment on this study.

Summary of Blood Sample Submission
Following enrollment (for all patients with favorable histology Wilms tumor): 5 -10 mLs (1 x 2 teaspoons)
Prior to surgery: 6 mLs (1 teaspoon)
at the end of therapy: 5 -10 mLs (1 x 2 teaspoons)
one year after the end of therapy: 5 -10 mLs (1 x 2 teaspoons)

optional ini1 Testing and Collection of Parental Blood Sample-
Samples of malignant rhabdoid tumor will be analyzed for the presence of ini1 mutations if the patient's family agrees to this testing. once a patient is diagnosed with malignant rhabdoid tumor, the optional informed consent for ini1 testing will be reviewed with the family. Consent for ini1 gene testing is offered to all patients with malignant rhabdoid tumor through this study.

ini1 mutation analysis will be conducted in the laboratory of Dr. Jaclyn Biegel (Children's Hospital of Philadelphia). if the parents agree to provide blood for ini1 testing, 4 teaspoonfuls of blood will be shipped to a CoG laboratory. With permission, the tumor tissue and blood that was collected when the child enrolled on aRen03B2 will be tested for the ini1 mutation. The blood will be used to see if the child's normal cells have the ini1 mutation only if the ini1 mutation is found in the tumor. if the mutation is found in the blood sample, the blood sample from each parent will be tested to see if either of them has the mutation. The results of the ini1 testing will not affect the child's treatment. However, if the parent or the child is found to carry the ini1 mutation in normal cells, this knowledge may be useful in telling whether other family members are at risk for rhabdoid tumor. The parents are given the choice of whether they wish to be notified of the ini1 test results. if the parents decide to be notified, the results will be given to the physician and genetic counseling will be arranged.

Participant Eligibility

1) Renal tumors- Patients with the first occurrence of any tumor of the kidney identified on CT scan or MRI are eligible for this study. Histologic diagnosis is not required prior to enrollment but is required for all patients once on 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 Adenofribroma

* Metanephric Stromal Tumor

Mesoblastic Nephroma:
Cellular, Classic, Mixed

Clear Cell Sarcoma

Rhabdoid Tumor - Any malignant rhabdoid tumor occurring outside the Central Nervous System

Renal Epithelioid Tumors of Childhood:
Papillary renal cell carcinoma
Renal medullary carcinoma
Renal tumors associated with Xp11.2 translocations
Oncocytic renal neoplasms following neuroblastoma


Ossifying renal tumor of infancy

Extrarenal tumors- Patients with the first occurrence of the following tumors are also eligible: extrarenal nephroblastoma, malignant rhabdoid tumor occurring anywhere outside the Central Nervous System.

2) Required specimens, reports, and copies of imaging studies must be available for submission or must become available during the required timeframe.

For ALL patients (with exception of bilateral, bilaterally predisposed or unilateral tumor in solitary kidney planning to enroll without biopsy), the following submissions are required:

* A complete set of recut H & E slides,

* Representative formalin-fixed paraffin-embedded tissue block or if a block is unavailable, 10 unstained slides from a representative block of tumor.

* Institutional pathology report, transmittal form and pathology checklist.

* Copies of images and institutional reports of CT and/or MRI abdomen and pelvis

* Copies of images and institutional report of CT chest for all malignant tumors

* Institutional surgical report(s)

* For patients with clinical features and required imaging findings consistent with the eligibility for the bilateral study, AREN0534 (or successor study), confirmed by central review, biopsy is not required. However, if biopsy is done, tissue must be submitted as for other renal tumors, and initial risk assignment will require pathology and surgical rapid central reviews. Transmittal form and pathology checklist are also needed.

* Patients with extrarenal Wilms tumor must have tumor tissue available for central review.

* Patients

3) Age

* Patients must be < 30 years old at the time of diagnosis.

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.