ACNS0332 | Chemotherapy and Radiation Therapy in Treating Young Patients With Newly Diagnosed, Previously Untreated, High-Risk Medulloblastoma/PNET
DOI: 10.7937/TCIA.582B-XZ89 | Data Citation Required | 432 Views | Image Collection
| Location | Species | Subjects | Data Types | Cancer Types | Size | Status | Updated | |
|---|---|---|---|---|---|---|---|---|
| Head | Human | 85 | CT, MR | Medulloblastoma | Clinical | Limited, Complete | 2021/05/04 |
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.
Version 1: Updated 2021/05/04
| Title | Data Type | Format | Access Points | Subjects | License | |||
|---|---|---|---|---|---|---|---|---|
| Images | CT, MR | DICOM | Download requires NBIA Data Retriever |
85 | 688 | 8,617 | 376,003 | NCTN/NCORP Data Archive License (Without Collaborative Agreement) |
Additional Resources for this Dataset
The National Cancer Institute (NCI) has created a centralized, controlled-access database, called the NCTN/NCORP Data Archive, for storing and sharing datasets generated from clinical trials of the National Clinical Trials Network (NCTN) and the NCI Community Oncology Research Program (NCORP). Clinical data from the participants in this trial can be found at:
- NCTN/NCORP Data Archive (Clinical Data)
Citations & Data Usage Policy
Data Citation Required: Users must abide by the TCIA Data Usage Policy and Restrictions. Attribution must include the following citation, including the Digital Object Identifier:
Data Citation |
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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 |
Detailed Description
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.
Related Publications
Publications by the Dataset Authors
The authors recommended the following as the best source of additional information about this dataset:
Publication Citation |
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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., Packer, R.J., Pfister, S.M., and 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. PMID: 30332335. |
No other publications were recommended by dataset authors.
Research Community Publications
TCIA maintains a list of publications that leveraged this dataset. If you have a manuscript you’d like to add please contact TCIA’s Helpdesk.