Children's National

Conditions

• 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

Eligibility

Eligible Ages

Under 29 Years

Eligible Genders

All

Accepts Healthy Volunteers

No

Criteria

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

Detailed Description

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. (As of Amendment 8, this aim is limited to patients with institutionally classified Stage I to IV focal or diffuse anaplasia identified at initial biopsy or primary nephrectomy OR at delayed nephrectomy/second biopsy.) II. 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: I. To monitor outcome for those patients who are not eligible for a subsequent therapeutic study. II. To describe whether the pulmonary tumor burden correlates with outcome in Stage IV patients.(Completed as of Amendment 7) III. 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. (Completed as of Amendment 7) IV. To compare the sensitivity and specificity of preoperative abdominal CT and MRI for the identification and differentiation of nephrogenic rests and Wilms tumor in children with multiple renal lesions. (Completed as of Amendment 7) V. To correlate the method of conception (natural versus 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. (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.