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  • Combination Chemotherapy With or Without Radiation Therapy in Treating Young Patients With Newly Diagnosed Stage III or Stage IV Wilms Tumor (AREN0533)

Summary

This collection contains data from the Children’s Oncology Group (COG) Clinical Trial NCT00379340, “Combination Chemotherapy With or Without Radiation Therapy in Treating Young Patients With Newly Diagnosed Stage III or Stage IV Wilms Tumor, " Study Chair: David Dix, MD. It was sponsored by NCI and performed by the Children's Oncology Group under study number AREN0533. This phase III trial is studying how well combination chemotherapy with or without radiation therapy works in treating young patients with newly diagnosed stage III or stage IV favorable histology Wilms tumor. Select patient-level clinical data from this trial is available via the following link: https://nctn-data-archive.nci.nih.gov/node/737.

Trial Description

Patients with stage IV favorable histology Wilms tumor (FHWT), the majority of whom have pulmonary metastases, have inferior outcomes compared with those with localized disease. Their treatment is also complicated by a risk of late effects, including cardiac dysfunction, lung toxicity, musculoskeletal and soft tissue defects, and second malignancies. The AREN0533 study applied two separate strategies for risk stratification for patients with Stage III and IV favorable histology Wilms tumor. The first is the identification of patients with pulmonary nodules who can be spared bilateral pulmonary irradiation. Patients with Stage IV favorable histology Wilms tumor have a 4-year event-free survival (EFS) of 75% with chemotherapy and irradiation to sites of metastatic disease (most frequently in the lungs). European investigators are able to spare 75% of their patients with pulmonary nodules from irradiation based on the initial response to chemotherapy. The response of the lung metastases to 6 weeks of chemotherapy consisting of vincristine, dactinomycin, and doxorubicin (Regimen DD-4A) was used to determine if radiation of lung nodules is needed. Patients who had complete disappearance of their lung metastases (or who had tissue confirmation that the nodules do not contain viable tumor) at the Week 6 evaluation were considered rapid responders and continued with DD-4A. Patients who did not have complete resolution of pulmonary nodules by Chest CT underwent pulmonary irradiation and were switched to Regimen M (DD-4A variation with dactinomycin and doxorubicin given on the same day plus cyclophosphamide and etoposide). Central radiology review of the chest CTs were performed on all Stage IV patients with lung metastases at study enrollment and at Week 6. The second risk stratification variable was the allelic loss of 1p and 16q. Patients with Stage III and IV favorable histology Wilms tumor with loss of heterozygosity (LOH) of both 1p and 16q have a 4-yr EFS of 65%. Patients with LOH of 1p and 16q were assigned to Regimen M in an attempt to improve the 4-year EFS of this group of patients to 84%.

Trial Outcomes

Results of the trial have been reported in the following publications:

  • Dix DB, Seibel NL, Chi YY, Khanna G, Gratias E, Anderson JR, Mullen EA, Geller JI, Kalapurakal JA, Paulino AC, Perlman EJ, Ehrlich PF, Malogolowkin M, Gastier-Foster JM, Wagner E, Grundy PE, Fernandez CV, Dome JS. Treatment of Stage IV Favorable Histology Wilms Tumor With Lung Metastases: A Report From the Children's Oncology Group AREN0533 Study. J Clin Oncol. 2018 Jun 1;36(16):1564-1570. doi: 10.1200/JCO.2017.77.1931. Epub 2018 Apr 16. PMID: 29659330; PMCID: PMC6075846.
  • Dix, D. B., Fernandez, C. V., Chi, Y.-Y., Mullen, E. A., Geller, J. I., Gratias, E. J., Khanna, G., Kalapurakal, J. A., Perlman, E. J., Seibel, N. L., Ehrlich, P. F., Malogolowkin, M., Anderson, J., Gastier-Foster, J., Shamberger, R. C., Kim, Y., Grundy, P. E., & Dome, J. S. (2019). Augmentation of Therapy for Combined Loss of Heterozygosity 1p and 16q in Favorable Histology Wilms Tumor: A Children’s Oncology Group AREN0532 and AREN0533 Study Report. In Journal of Clinical Oncology (Vol. 37, Issue 30, pp. 2769–2777). American Society of Clinical Oncology (ASCO). https://doi.org/10.1200/jco.18.01972

Data Access

This is a limited access data set. To request access please register an account on the NCTN Data Archive.  After logging in, use the "Request Data" link in the left side menu.  Follow the on screen instructions, and enter NCT00379340 when asked which trial you want to request.  In step 2 of the Create Request form, be sure to select “Imaging Data Requested”. Please contact NCINCTNDataArchive@mail.nih.gov for any questions about access requests.

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Images (DICOM, 185 GB)



   

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Clinical data (link)

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Please contact help@cancerimagingarchive.net  with any questions regarding usage.

Detailed Description

Image Statistics


Modalities

CT, CR, MR, US

Number of Patients

294

Number of Studies

845

Number of Series

4761

Number of Images

364082

Images Size (GB)185

De-identification of DICOM dates

De-identification of dates for this dataset uses the DICOM Part 3.15 Annex E standard “Retain Longitudinal With Modified Dates Option” which allows dates to be retained as long as they are modified from the original date.  TCIA implements this using a technique which de-identifies the dates while preserving the longitudinal relationship between them.  Original dates will be first normalized to 01 January, 1960 and then offset relative to the date of registration for each patient.  This normalized date system was chosen in order to make it obvious that the dates are not real, and to make it easy to quickly determine how much time has passed between the date of registration and the patients' related imaging studies. 

For example, if the real date of a patient's registration was 03/27/2018 and the original imaging Study Date was 03/29/2018 then the "Days from registration" would be +2 and the anonymized TCIA Study Date would become 01/03/1960.

Insertion of computed "REGISTRATION"/Days offset from registration" value

In addition to modifying the actual date fields in the DICOM header, the "days from registration" values are calculated and stored in the DICOM tag (0012,0052) Longitudinal Temporal Offset from Event with the associated tag (0012,0053) Longitudinal Temporal Event Type set to “REGISTRATION”. 

Note: If these DICOM tags are not present, DICOM tag (0012,0050) Clinical Trial Time Point ID with the associated tag (0012,0051) Clinical Trial Time Point Description provides this same information.  This inconsistency is due to a change in how dates were handled in the first NCTN trials that were published on TCIA.


Citations & Data Usage Policy

This is a limited access data set. Upon receiving access, you must abide by the terms of your NCTN/NCORP Data Archive’s Data Use Agreement (DUA). You are not allowed to redistribute the data or use it for other purposes. Attribution should include references to the following citations:

Data Citation

Dix, D. B., Seibel, N. L., Chi, Y.-Y., Khanna, G., Gratias, E., Anderson, J. R., Mullen, E. A., Geller, J. I., Kalapurakal, J. A., Paulino, A. C., Perlman, E. J., Ehrlich, P. F., Malogolowkin, M., Gastier-Foster, J. M., Wagner, E., Grundy, P. E., Fernandez, C. V., & Dome, J. S. (2022). Combination Chemotherapy With or Without Radiation Therapy in Treating Young Patients With Newly Diagnosed Stage III or Stage IV Wilms Tumor (AREN0533) (Version 1) [Data set]. The Cancer Imaging Archive. https://doi.org/10.7937/SJEZ-CJ78

Publication Citation

Dix, D. B., Seibel, N. L., Chi, Y.-Y., Khanna, G., Gratias, E., Anderson, J. R., Mullen, E. A., Geller, J. I., Kalapurakal, J. A., Paulino, A. C., Perlman, E. J., Ehrlich, P. F., Malogolowkin, M., Gastier-Foster, J. M., Wagner, E., Grundy, P. E., Fernandez, C. V., & Dome, J. S. (2018). Treatment of Stage IV Favorable Histology Wilms Tumor With Lung Metastases: A Report From the Children’s Oncology Group AREN0533 Study. In Journal of Clinical Oncology (Vol. 36, Issue 16, pp. 1564–1570). American Society of Clinical Oncology (ASCO). https://doi.org/10.1200/jco.2017.77.1931. Epub 2018 Apr 16. PMID: 29659330; PMCID: PMC6075846.

Publication Citation

Dix, D. B., Fernandez, C. V., Chi, Y.-Y., Mullen, E. A., Geller, J. I., Gratias, E. J., Khanna, G., Kalapurakal, J. A., Perlman, E. J., Seibel, N. L., Ehrlich, P. F., Malogolowkin, M., Anderson, J., Gastier-Foster, J., Shamberger, R. C., Kim, Y., Grundy, P. E., & Dome, J. S. (2019). Augmentation of Therapy for Combined Loss of Heterozygosity 1p and 16q in Favorable Histology Wilms Tumor: A Children’s Oncology Group AREN0532 and AREN0533 Study Report. In Journal of Clinical Oncology (Vol. 37, Issue 30, pp. 2769–2777). American Society of Clinical Oncology (ASCO). https://doi.org/10.1200/jco.18.01972.

TCIA Citation

Clark, K., Vendt, B., Smith, K., Freymann, J., Kirby, J., Koppel, P., Moore, S., Phillips, S., Maffitt, D., Pringle, M., Tarbox, L., & Prior, F. (2013). The Cancer Imaging Archive (TCIA): Maintaining and Operating a Public Information Repository. In Journal of Digital Imaging (Vol. 26, Issue 6, pp. 1045–1057). Springer Science and Business Media LLC. https://doi.org/10.1007/s10278-013-9622-7

Other Publications Using This Data

TCIA maintains a list of publications which leverage TCIA data. If you have a manuscript you'd like to add please contact the TCIA Helpdesk.

Version 1 (Current): Updated 2022/01/20

Data TypeDownload all or Query/Filter
Images (DICOM, 185 GB)
Clinical Data (link)



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