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  • Vincristine, Dactinomycin, and Doxorubicin With or Without Radiation Therapy or Observation Only in Treating Younger Patients Who Are Undergoing Surgery for Newly Diagnosed Stage I, Stage II, or Stage III Wilms' Tumor (AREN0532)

Summary

This collection contains data from the Children’s Oncology Group (COG) Clinical Trial NCT00352534, “Vincristine, Dactinomycin, and Doxorubicin With or Without Radiation Therapy or Observation Only in Treating Younger Patients Who Are Undergoing Surgery for Newly Diagnosed Stage I, Stage II, or Stage III Wilms' Tumor". Principal Investigator: Conrad Fernandez, MD in Halifax, NS.  It was sponsored by NCI and performed by the Children's Oncology Group under study number AREN0532. This phase III trial is studying vincristine, dactinomycin, and doxorubicin with or without radiation therapy or observation only to see how well they work in treating patients undergoing surgery for newly diagnosed stage I, stage II, or stage III Wilms' tumor. Select patient-level clinical data from this trial is available via the following link: https://nctn-data-archive.nci.nih.gov/node/689.

Trial Description           

COG AREN0532 is a treatment study of kidney tumors which have not spread to other parts of the body. 544 very low and standard risk favorable histology Wilms Tumor patients entered the trial. At accrual all patients were Stage I-III and had not received any prior therapy. Dates of therapy were from 2006 to 2013.

The National Wilms Tumor Study (NWTS) approach to treating stage III favorable-histology Wilms tumor (FHWT) is Regimen DD4A (vincristine, dactinomycin, and doxorubicin) and radiation therapy. Further risk stratification is desired to improve outcomes and reduce late effects. In a 2018 JCO paper, the trial evaluated clinical and biologic variables for patients with stage III FHWT without combined loss of heterozygosity (LOH) at chromosomes 1p and 16q. This paper included 535 patients with stage III disease. Relapse after stage III treatment is associated with an overall survivsal (OS) of only 50% despite intensive salvage chemotherapy and/or autologous bone marrow transplantation. It is thus highly desirable to identify patients who need augmentation of initial therapy with the hope of preventing relapse.

A novel finding in this study was the remarkably strong predictive value of combining LOH and lymph node status. The relapse rate was exceptionally low among patients with tumors that were LOH - and lymph node – negative. However, this trial demonstrated that those with combined lymph node involvement and LOH 1p or 16q had a significantly worse 4-year EFS outcome of 74%. There is a trend toward a poorer 4-year OS in this comparison; however, it is not statistically different.

Approximately two thirds of patients had delayed-nephrectomy tissue submitted for central pathology review. Most patients with blastemal-type Wilms tumor but none of seven patients with low-risk/ completely necrotic Wilms tumor experienced relapse, consistent withthe findings of the International Society of Pediatric Oncology (SIOP) that histologic response to preoperative chemotherapy plays an important role in predicting outcome.

The results of this trial described the overall good outcome of patients with stage III FHWT using DD4A with radiation therapy and identified an association of combined lymph node and LOH status, as well as postchemotherapy, delayed nephrectomy histology, with EFS.

Data from the 2018 J Clin Oncol. paper, cited below: A total of 535 patients with stage III disease were studied. Median follow-up was 5.2 years (range, 0.2 to 9.5). Four-year event-free survival (EFS) and overall survival estimates were 88% (95% CI,85% to 91%) and 97% (95% CI, 95% to 99%), respectively. A total of 58 of 66 relapses occurred in the first 2 years, predominantly pulmonary (n = 36). Eighteen patients died, 14 secondary to disease.

A better EFS was associated with negative lymph node status (P less than .01) and absence of LOH 1p or 16q (P less than .01), but not with gross residual disease or peritoneal implants. In contrast, the 4-year EFS was only 74% in patients with combined positive lymph node status and LOH 1p or 16q. A total of 123 patients (23%) had delayed nephrectomy. Submitted delayed nephrectomy histology showed anaplasia (n = 8; excluded from survival analysis); low risk/completely necrotic (n = 7; zero relapses), intermediate risk (n = 63; six relapses), and high-risk/blastemal type (n=7; five relapses).

Trial Outcomes

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

  • Fernandez CV, Mullen EA, Chi YY, Ehrlich PF, Perlman EJ, Kalapurakal JA, Khanna G, Paulino AC, Hamilton TE, Gow KW, Tochner Z, Hoffer FA, Withycombe JS, Shamberger RC, Kim Y, Geller JI, Anderson JR, Grundy PE, Dome JS. Outcome and Prognostic Factors in Stage III Favorable-Histology Wilms Tumor: A Report From the Children's Oncology Group Study AREN0532. J Clin Oncol. 2018 Jan 20;36(3):254-261. doi: 10.1200/JCO.2017.73.7999. Epub 2017 Dec 6. Erratum in: J Clin Oncol. 2019 Oct 10;37(29):2710. PMID: 29211618; PMCID: PMC5773840. https://doi.org/10.1200/jco.2017.73.7999
  • Dix DB, Fernandez CV, Chi YY, Mullen EA, Geller JI, Gratias EJ, Khanna G, Kalapurakal JA, Perlman EJ, Seibel NL, Ehrlich PF, Malogolowkin M, Anderson J, Gastier-Foster J, Shamberger RC, Kim Y, Grundy PE, Dome JS; AREN0532 and AREN0533 study committees. 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. J Clin Oncol. 2019 Oct 20;37(30):2769-2777. doi: 10.1200/JCO.18.01972. Epub 2019 Aug 26. PMID: 31449468; PMCID: PMC7001789. 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 NCT00352534 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, 236 GB)


   

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Clinical data (link)

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Detailed Description

Image Statistics


Modalities

CR, CT, MR, PT, RTIMAGE, US

Number of Patients

544

Number of Studies

1021

Number of Series

5903

Number of Images

472056
Images Size (GB)236

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

Fernandez, C. V., Mullen, E. A., Chi, Y.-Y., Ehrlich, P. F., Perlman, E. J., Kalapurakal, J. A., Khanna, G., Paulino, A. C., Hamilton, T. E., Gow, K. W., Tochner, Z., Hoffer, F. A., Withycombe, J. S., Shamberger, R. C., Kim, Y., Geller, J. I., Anderson, J. R., Grundy, P. E., & Dome, J. S. (2022). Vincristine, Dactinomycin, and Doxorubicin With or Without Radiation Therapy or Observation Only in Treating Younger Patients Who Are Undergoing Surgery for Newly Diagnosed Stage I, Stage II, or Stage III Wilms’ Tumor (AREN0532) (Version 1) [Data set]. The Cancer Imaging Archive. https://doi.org/10.7937/6PJ1-M859

Publication Citation

Fernandez, C. V., Mullen, E. A., Chi, Y.-Y., Ehrlich, P. F., Perlman, E. J., Kalapurakal, J. A., Khanna, G., Paulino, A. C., Hamilton, T. E., Gow, K. W., Tochner, Z., Hoffer, F. A., Withycombe, J. S., Shamberger, R. C., Kim, Y., Geller, J. I., Anderson, J. R., Grundy, P. E., & Dome, J. S. (2018). Outcome and Prognostic Factors in Stage III Favorable-Histology Wilms Tumor: A Report From the Children’s Oncology Group Study AREN0532. In Journal of Clinical Oncology (Vol. 36, Issue 3, pp. 254–261). American Society of Clinical Oncology (ASCO). https://doi.org/10.1200/jco.2017.73.7999

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

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Version 1 (Current): Updated 2022/03/31

Data TypeDownload all or Query/Filter

Images (DICOM, 236 GB)


   

(Download requires the NBIA Data Retriever)

Clinical data (link)



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