for people ages up to 29 years (full criteria)
study started



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.

Official Title

Renal Tumors Classification, Biology, and Banking Study



  1. 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.


  1. Monitor outcome for those patients who are not eligible for a subsequent therapeutic study.

II. Describe whether the pulmonary tumor burden correlates with outcome in stage IV patients.

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.

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.

  1. Correlate the method of conception (natural vs assisted reproductive technology) with the development of Wilms' tumor.

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)


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.


Adult Cystic Nephroma Anaplastic Kidney Wilms Tumor Angiolipoma Cellular Congenital Mesoblastic Nephroma Classic Congenital Mesoblastic Nephroma Clear Cell Sarcoma of the Kidney Congenital Mesoblastic Nephroma Cystic Partially Differentiated Kidney Nephroblastoma Diffuse Hyperplastic Perilobar Nephroblastomatosis Extrarenal Rhabdoid Tumor Kidney Medullary Carcinoma Kidney Neoplasm Kidney Oncocytoma Kidney Wilms Tumor Metanephric Adenofibroma Metanephric Adenoma Metanephric Stromal Tumor Metanephric Tumor Mixed Congenital Mesoblastic Nephroma Ossifying Renal Tumor of Infancy Papillary Renal Cell Carcinoma Renal Cell Carcinoma Renal Cell Carcinoma Associated With Xp11.2 Translocations/TFE3 Gene Fusions Rhabdoid Tumor of the Kidney Wilms Tumor Carcinoma Carcinoma, Renal Cell Adenoma Rhabdoid Tumor Kidney Neoplasms Carcinoma, Medullary Sarcoma, Clear Cell Nephroma, Mesoblastic Adenofibroma Neoplasms Cytology Specimen Collection Procedure Laboratory Biomarker Analysis Ancillary-correlative (renal tumor classification, biology)


For people ages up to 29 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, 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 hematoxylin and eosin (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)
  • Tissue must be from diagnosis, prior to any chemotherapy or radiation
  • 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 with extra-CNS malignant rhabdoid tumor must have tumor tissue available for central review
  • 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


  • Mattel Children's Hospital UCLA
    Los Angeles California 90095 United States
  • Children's Hospital and Research Center at Oakland
    Oakland California 94609-1809 United States
  • University of California Davis Comprehensive Cancer Center
    Sacramento California 95817 United States
  • UCSF Medical Center-Parnassus
    San Francisco California 94143 United States
  • UCSF Medical Center-Mission Bay
    San Francisco California 94158 United States
  • Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center
    Torrance California 90502 United States
  • Cedars Sinai Medical Center
    Los Angeles California 90048 United States
  • Rady Children's Hospital - San Diego
    San Diego California 92123 United States
  • Children's Hospital Los Angeles
    Los Angeles California 90027 United States
  • Children's Hospital of Orange County
    Orange California 92868 United States


currently not accepting new patients, but might later
Start Date
Children's Oncology Group
Study Type
Last Updated