This research trial studies kidney tumors in younger patients. Collecting and storing samples
of tumor tissue, blood, and urine from patients with cancer to study in the laboratory may
help doctors learn more about changes that occur in deoxyribonucleic acid (DNA) and identify
biomarkers related to cancer.
PRIMARY OBJECTIVES:
I. 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 define eligibility for a series of therapeutic studies. (Loss of
heterozygosity [LOH] testing discontinued as of April 2014)
II. 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:
I. 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.
II. Describe whether the pulmonary tumor burden correlates with outcome in stage IV patients.
(Completed as of Amendment 7)
III. 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.
(Completed as of Amendment 7)
IV. Compare the sensitivity and specificity of pre-operative abdominal CT scan and magnetic
resonance imaging (MRI) for the identification and differentiation of nephrogenic rests and
Wilms' tumor in children with multiple renal lesions. (Completed as of Amendment 7)
V. Correlate the method of conception (natural vs assisted reproductive technology) with the
development of Wilms' tumor. (Completed as of Amendment 7)
VI. To evaluate the frequency of integrase interactor 1 (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. (INI1 testing discontinued as of April 2014) (Completed as of Amendment 7)
OUTLINE:
Tumor tissue, blood, and urine samples are collected for research studies, including
immunohistochemistry. CT scans and MRIs are also performed. Loss of heterozygosity analyses
(chromosome 1p and 16q) are performed by extraction of DNA. DNA polymorphisms are assayed by
polymerase chain reaction using standard methodology. Leftover specimens are archived for
future studies. (LOH and INI1 testing discontinued as of April 2014)
Patients are followed up periodically for 5 years.
Inclusion Criteria:
- 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 adenofibroma,
metanephric stromal tumor)
- Mesoblastic nephroma (cellular, classic, mixed)
- Clear cell sarcoma
- Rhabdoid tumor (any malignant rhabdoid tumor occurring outside the central
nervous system [CNS])
- Renal epithelioid tumors of childhood (papillary renal cell carcinoma, medullary
renal cell carcinoma, renal tumors associated with Xp11.2 translocations,
oncocytic renal neoplasms after neuroblastoma)
- Angiolipoma
- Ossifying renal tumor of infancy
- Patients with the first occurrence of the following tumors are also eligible:
- Extrarenal nephroblastoma or extrarenal neprogenic rests
- Malignant rhabdoid tumor occurring anywhere outside the central nervous system
- 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, (with exception of bilateral, bilaterally predisposed, multicentric,
or unilateral tumor in solitary kidney planning to enroll without biopsy***), the
following submissions are required:
- A complete set of recut hematoxylin and eosin (H & E) slides (including from
sampled lymph nodes, if patient had upfront nephrectomy)
- * Tissue must be from diagnosis, prior to any renal tumor directed
chemotherapy or radiation (only exception is for presumed favorable
histology Wilms tumor [FHWT] patients discovered to have diffuse anaplastic
Wilms tumor [DAWT] at delayed nephrectomy and plan to enroll at delayed
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.
- Tissue must be from diagnosis, prior to any renal tumor directed
chemotherapy or radiation (only exception is for presumed FHWT patients
discovered to have DAWT at delayed nephrectomy and plan to enroll at delayed
nephrectomy)
- 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)
- Patients with bilateral, bilaterally predisposed, multicentric, or
unilateral tumor in solitary kidney planning to enroll without biopsy via
imaging only - these patients will not have central review or have a risk
assignment issued, but may contribute to specimen banking for future
research. 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. The Specimen Transmittal Form and Pre
Treatment Pathology Checklist are also needed.
- Please note: if the above required items are not received within 120 days of
study enrollment, the patient will be considered off study
- All patients and/or their parents or legal guardians must sign a written informed
consent
- All institutional, Food and Drug Administration (FDA), and National Cancer Institute
(NCI) requirements for human studies must be met
