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  • Chemotherapy and Radiation Therapy in Treating Young Patients With Newly Diagnosed, Previously Untreated, High-Risk Medulloblastoma/PNET (ACNS0332)
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Summary

This collection contains data from the Children’s Oncology Group (COG) Clinical Trial NCT00392327, "Chemotherapy and Radiation Therapy in Treating Young Patients With Newly Diagnosed, Previously Untreated, High-Risk Medulloblastoma/PNET". The Study Chair was James M. Olson, M.D., Ph.D. It was sponsored by NCI and performed by the Children's Oncology Group under study number ACNS0332. This randomized phase III trial studies different chemotherapy and radiation therapy regimens to compare how well they work in treating young patients with newly diagnosed, previously untreated, high-risk medulloblastoma.  Select patient-level clinical data from this trial is available via the following link: https://nctn-data-archive.nci.nih.gov/node/838.

Trial Description

Children with histologically diagnosed high-risk medulloblastoma, supratentorial primitive neuro-ectodermal tumor of the CNS (CNS-PNET), and pineoblastoma (PBL) have had poor survival despite intensive treatment.  The Children’s Oncology Group (COG) study ACNS0332 was designed to test two approaches for treatment intensification for these patients: addition of carboplatin during irradiation and/or addition of isotretinoin to the adjuvant regimen. Carboplatin has demonstrated preclinical and clinical efficacy in these tumors and is well tolerated in children, whereas isotretinoin crosses the blood-brain barrier efficiently and is effective against preclinical models of medulloblastoma (MB).  Molecular profiling later revealed tumor heterogeneity that was not detectable at protocol inception. Enrollment of patients with CNS-PNET/PBL was subsequently discontinued, and outcomes for this part of the study reported.  Eighty-five participants with institutionally diagnosed CNS-PNETs/PBLs were enrolled. Of 60 patients with sufficient tissue, 31 had tumors that were non-pineal in location. Twenty-two patients (71%) of those 31 were diagnosed with tumor types not intended for trial inclusion, including 18 high-grade (HGGs), two atypical teratoid rhbdoid tumors, and 2 ependymomas. Outcomes across tumor types were strikingly different. Neither carboplatin, nor isotretinoin significantly altered outcomes for all patients. Survival for patients with HGG was similar to that of historic studies that avoid craniospinal irradiation and intensive chemotherapy.  For patients with CNS-PNET/PBL, prognosis is considerably better than previously assumed when molecularly confirmed HGGs are removed. Identification of molecular HGGs may spare affected children from unhelpful intensive treatment. This trial highlights the challenges of a histology-based diagnosis for pediatric brain tumors and indicates that molecular profiling should become a standard component of initial diagnosis.

Pre-operative and post-operative MRI scans of the brain with and without contrast and spinal MRI with and without contrast were required.  All scans underwent central review.

Trial Outcomes

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

Hwang, E. I., Kool, M., Burger, P. C., Capper, D., Chavez, L., Brabetz, S., Williams-Hughes, C., Billups, C., Heier, L., Jaju, A., Michalski, J., Li, Y., Leary, S., Zhou, T., von Deimling, A., Jones, D. T. W., Fouladi, M., Pollack, I. F., Gajjar, A., … Olson, J. M. (2018). Extensive Molecular and Clinical Heterogeneity in Patients With Histologically Diagnosed CNS-PNET Treated as a Single Entity: A Report From the Children’s Oncology Group Randomized ACNS0332 Trial. Journal of Clinical Oncology, 36(34), 3388–3395. https://doi.org/10.1200/jco.2017.76.4720. Epub ahead of print. PMID: 30332335.


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 NCT00392327 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.

Data TypeDownload all or Query/Filter
Images (DICOM, 93.0 GB)
Clinical Data (CSV, External)

Click the Versions tab for more info about data releases.

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

Detailed Description

Image Statistics


Modalities

MR,CT

Number of Patients

85

Number of Studies

688

Number of Series

8617

Number of Images

376003

Images Size (GB)93

Citations & Data Usage Policy

This is a limited access data set. Upon receiving access you may only use it for the purposes outlined in your request to the the NCTN Data Archive. You are not allowed to redistribute the data or use it for other purposes. See TCIA's Data Usage Policies and Restrictions for additional details. Questions may be directed to help@cancerimagingarchive.net Users of this data must abide by the TCIA Data Usage Policy and the Creative Commons Attribution 4.0 International License under which it has been published. Attribution should include references to the following citations:

Data Citation

Hwang, E. I., Kool, M., Burger, P. C., Capper, D., Chavez, L., Brabetz, S., Williams-Hughes, C., Billups, C., Heier, L., Jaju, A., Michalski, J., Li, Y., Leary, S., Zhou, T., von Deimling, A., Jones, D. T. W., Fouladi, M., Pollack, I. F., Gajjar, A., … Olson, J. M. (2021). Chemotherapy and Radiation Therapy in Treating Young Patients With Newly Diagnosed, Previously Untreated, High-Risk Medulloblastoma/PNET (ACNS0332) [Data set]. The Cancer Imaging Archive. https://doi.org/10.7937/TCIA.582B-XZ89

Publication Citation

Hwang, E. I., Kool, M., Burger, P. C., Capper, D., Chavez, L., Brabetz, S., Williams-Hughes, C., Billups, C., Heier, L., Jaju, A., Michalski, J., Li, Y., Leary, S., Zhou, T., von Deimling, A., Jones, D. T. W., Fouladi, M., Pollack, I. F., Gajjar, A., … Olson, J. M. (2018). Extensive Molecular and Clinical Heterogeneity in Patients With Histologically Diagnosed CNS-PNET Treated as a Single Entity: A Report From the Children’s Oncology Group Randomized ACNS0332 Trial. Journal of Clinical Oncology, 36(34), 3388–3395. https://doi.org/10.1200/jco.2017.76.4720. Epub ahead of print. PMID: 30332335.

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. Journal of Digital Imaging, 26(6), 1045–1057. 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 2021/05/04

Data TypeDownload all or Query/Filter
Images (DICOM, 93.0 GB)
Clinical Data (CSV, External)







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