Study of Kidney Tumors in Younger Patients

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