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Study Summary
No. COGAREN03B2:.......Diagnostic; Kidney; Laboratory; Pediatrics......Najat Daw-Bitar......Pediatrics
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Study Summary Title
Study Summary
Number:
COGAREN03B2
Study Title:Renal Tumors Classification, Biology, and Banking Study
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Physician New Patient Referral
Name:Najat Daw-BitarPatients Call:800-392-1611 (in U.S.A.) 713-792-6161 (outside U.S.A.)
Dept:PediatricsReferring MD
Call:
800-392-1611 (in U.S.A.) 713-792-6161 (outside U.S.A.)
Phone:713-792-6620
Contact us about clinical trials
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General Information
Disease Group:Diagnostic
Kidney
Laboratory
Pediatrics
Supported By:NCI through COG
Phase of Study:N/AReturn
Visit:
Patients being treated on a COG treatment trial will return for a blood sample
collection to send to COG reference labs at the end of therapy and one year
after the end of therapy.
Treatment
Agents:
NoneHome Care:Not applicable.
Treatment Loc:Both at MDACC & outside MDACC at one or more Collaborating Sites or Institutions
Estimated
Length of Stay
in Houston:
Length of stay depends on the extent and outcome of biopsy or surgery.
Relapse(s) may require additional biopsy or surgery.
Description/
Intervention:
The goal of this study is to study what percentage of rhabdoid tumors have
mutations of the INI1 gene, and to compare the behavior of malignant rhabdoid
tumors with and without mutations of the INI1 gene.

Researchers would like to study the blood samples of all parents of pediatric
patients diagnosed with malignant rhabdoid tumor who are enrolled on
COGAREN03B2. The study COGAREN03B2 will remain open even if there are no
treatment studies, so it is not known how many parents will take part in this
optional study.
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Study Objectives / Outcomes
Primary Objectives
1) To classify patients with renal tumors by histological categorization, surgico-pathological 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.
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.

Secondary Objectives
1) To monitor outcome for those patients who are not eligible for a subsequent therapeutic study including patients initially classified as Low Risk, defined as those with favorable histology Wilms tumor Stage I (> 2 years old or tumor weight > 550 g) or Stage II whose final risk classification is Low Risk and whose tumors do not have LOH 1p and 16q.
2) To describe whether the pulmonary tumor burden correlates with outcome in Stage IV patients.
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.
4) To compare the sensitivity and specificity of pre-operative abdominal CT and MRI for the identification
and differentiation of nephrogenic rests and Wilms tumor in children with multiple renal lesions.
5) To correlate the method of conception (natural versus assisted reproductive technology) with the
development of Wilms tumor.
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.
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Study Status Information
Study Activation / Registration Date:08/30/2007
IRB Review and Approval Date:08/30/2007
Study Type:Laboratory
Recruitment Status:Closed
Projected Accrual:2,200
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Enrollment Eligibility
If you do not meet the enrollment eligibility, there may be other treatment options for you. Please Contact the Referral Office for more information.

Inclusion Criteria:1) Patients must be < 30 years old at the time of diagnosis.

2) Patients who plan to enroll on a renal tumor therapeutic study must also meet the submission requirements for the relevant therapeutic study.

3) 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.

4) Patients with following tumors are eligible for this study: 1) Nephroblastoma (Wilms Tumor), favorable histology, anaplasia (Diffuse, Focal); 2) Nephrogenic rests and Nephroblastomatosis; 3) Cystic Nephroma and Cystic Partially Differentiated Nephroblastoma; 4) Metanephric Tumors (Metanephric Adenoma; Metanephric Adenofibroma; Metanephric Stromal Tumor);

5) #4 continued: 5) Mesoblastic Nephroma (Cellular, Classic, Mixed); 6) Clear Cell Sarcoma; 7) Rhabdoid tumor; 8) Renal Epithelioid Tumors of Childhood (Papillary renal cell carcinoma; Renal medullary carcinoma; Renal tumors associated with Xp11.2 translocations; Oncocytic renal neoplasms following neuroblastoma); 9) Angiolipoma; or, 10) Ossifying renal tumor of infancy.

6) Patients with an extrarenal nephroblastoma are also eligible.

7) Extrarenal tumors: Patients with the first occurrence of the following tumors are eligible: 1) Extrarenal nephroblastoma; 2) Malignant rhabdoid tumor occurring anywhere outside the Central Nervous System.

8) For patients with unilateral favorable histology Wilms tumor, tumor tissue from the biopsy and/or surgical procedure must be available for central review and diagnostic studies (LOH). This must be the first occurrence of Wilms tumor and patients must not have had any therapy prior to the biopsy and/or surgical procedure for their disease.

9) For patients with bilateral disease diagnosed by abdominal CT scan and confirmed by central review, biopsy is not required. However, if biopsy is done, tissue must be submitted as for other renal tumors.

10) For patients with Wilms tumor-predisposing syndromes and unilateral disease diagnosed by abdominal CT scan and confirmed by central review, biopsy is also not required. However, if biopsy is done, tissue must be submitted as for other renal tumors.

11) Patients with extra-renal Wilms tumor must have tumor tissue available for central review.

12) Patients with extra-CNS malignant rhabdoid tumor must have tumor tissue available for central review.

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

14) Timing considerations: Patients must begin protocol therapy on renal tumor therapeutic studies by Day 14 after surgery or biopsy. It is suggested that pathology slides, operative notes, and radiology studies are submitted as soon as possible after nephrectomy/biopsy and by Day 7 to allow adequate time for central review to be completed. Samples may be submitted before an institutional diagnosis is rendered.

15) Patients may begin protocol therapy before central review is completed if medically indicated (e.g., significant symptoms from large tumor burden). However, enrollment on renal tumor therapeutic studies must occur within 7 days after beginning treatment or by Day 14 following surgery, whichever occurs first. If treatment begins before central review is completed, samples and studies must be submitted immediately to allow central review to occur.

16) Patients with renal tumors who are to be on the biology/banking study only must have their samples and studies submitted within 30 days of diagnostic surgery

17) All patients and/or their parents or legal guardians must sign a written informed consent.

18) All institutional, FDA, and NCI requirements for human studies must be met.

Exclusion Criteria:1) Patients >/= 30 years old at the time of diagnosis.

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Links
Registration Number: NCT00898365
Study Information on Clinical Trials Registry (clinicaltrials.gov)

Other Links:
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Results


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