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| Home > Publications database > Relevance of Molecular Groups in Children with Newly Diagnosed Atypical Teratoid Rhabdoid Tumor: Results from Prospective St. Jude Multi-Institutional Trials. > print |
| Home > Publications database > Relevance of Molecular Groups in Children with Newly Diagnosed Atypical Teratoid Rhabdoid Tumor: Results from Prospective St. Jude Multi-Institutional Trials. > print |
| 001 | 168085 | ||
| 005 | 20240229133557.0 | ||
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| 100 | 1 | _ | |a Upadhyaya, Santhosh A |0 0000-0001-6101-149X |b 0 |
| 245 | _ | _ | |a Relevance of Molecular Groups in Children with Newly Diagnosed Atypical Teratoid Rhabdoid Tumor: Results from Prospective St. Jude Multi-Institutional Trials. |
| 260 | _ | _ | |a Philadelphia, Pa. [u.a.] |c 2021 |b AACR |
| 336 | 7 | _ | |a article |2 DRIVER |
| 336 | 7 | _ | |a Output Types/Journal article |2 DataCite |
| 336 | 7 | _ | |a Journal Article |b journal |m journal |0 PUB:(DE-HGF)16 |s 1625054136_11425 |2 PUB:(DE-HGF) |
| 336 | 7 | _ | |a ARTICLE |2 BibTeX |
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| 500 | _ | _ | |a 27(10):2879-2889 |
| 520 | _ | _ | |a Report relevance of molecular groups to clinico-pathologic features, germline SMARCB1/SMARCA4 alterations (GLA), and survival of children with atypical teratoid rhabdoid tumor (ATRT) treated in two multi-institutional clinical trials.Seventy-four participants with newly diagnosed ATRT were treated in two trials: infants (SJYC07: age<3 years; n=52) and children (SJMB03: age 3-21 years; n=22), using surgery, conventional chemotherapy (infants), or dose-dense chemotherapy with autologous stem cell rescue (children), and age- and risk-adapted radiation therapy [focal (infants) and craniospinal (CSI) (children)]. Molecular groups ATRT-MYC (MYC), ATRT-SHH (SHH), and ATRT-TYR (TYR) were determined from tumor DNA methylation profiles.Twenty-four participants (32%) were alive at time of analysis at a median follow-up of 8.4 years (range, 3.1-14.1 years). Methylation profiling classified 64 ATRTs as TYR (n=21), SHH (n=30), and MYC (n=13), SHH group being associated with metastatic disease. Among infants, TYR group had the best overall survival (OS) (P=0.02). However, outcomes did not differ by molecular groups among infants with non-metastatic (M0) disease. Children with M0 disease and <1.5 cm2 residual tumor had a 5-year progression-free survival (PFS) of 72.7{plus minus}12.7% and OS of 81.8{plus minus}11%. Infants with M0 disease had a 5-year PFS of 39.1{plus minus}11.5% and OS of 51.8{plus minus}12%. Those with metastases fared poorly [5-year OS 25{plus minus}12.5% (children) and 0% (infants)]. SMARCB1 GLAs were not associated with PFS.Among infants, those with ATRT-TYR had the best OS. ATRT-SHH was associated with metastases and consequently with inferior outcomes. Children with non-metastatic ATRT benefit from post-operative CSI and adjuvant chemotherapy. |
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| 700 | 1 | _ | |a Robinson, Giles |0 0000-0001-7441-9486 |b 1 |
| 700 | 1 | _ | |a Onar-Thomas, Arzu |b 2 |
| 700 | 1 | _ | |a Orr, Brent A |0 0000-0003-0997-4728 |b 3 |
| 700 | 1 | _ | |a Johann, Pascal |b 4 |
| 700 | 1 | _ | |a Wu, Gang |b 5 |
| 700 | 1 | _ | |a Billups, Catherine A |b 6 |
| 700 | 1 | _ | |a Tatevossian, Ruth G |0 0000-0002-1653-3026 |b 7 |
| 700 | 1 | _ | |a Dhanda, Sandeep Kumar |0 0000-0003-1381-7434 |b 8 |
| 700 | 1 | _ | |a Srinivasan, Ashok |b 9 |
| 700 | 1 | _ | |a Broniscer, Alberto |b 10 |
| 700 | 1 | _ | |a Qaddoumi, Ibrahim |b 11 |
| 700 | 1 | _ | |a Vinitsky, Anna |b 12 |
| 700 | 1 | _ | |a Armstrong, Gregory T |b 13 |
| 700 | 1 | _ | |a Bendel, Anne |0 0000-0002-2062-0974 |b 14 |
| 700 | 1 | _ | |a Hassall, Timothy Eg |0 0000-0002-7883-3710 |b 15 |
| 700 | 1 | _ | |a Partap, Sonia |0 0000-0001-5615-7689 |b 16 |
| 700 | 1 | _ | |a Fisher, Paul G |b 17 |
| 700 | 1 | _ | |a Crawford, John R |0 0000-0002-4100-7001 |b 18 |
| 700 | 1 | _ | |a Chintagumpala, Murali M |b 19 |
| 700 | 1 | _ | |a Bouffet, Eric |0 0000-0002-6832-6539 |b 20 |
| 700 | 1 | _ | |a Gururangan, Sridharan |b 21 |
| 700 | 1 | _ | |a Mostafavi, Roya |b 22 |
| 700 | 1 | _ | |a Sanders, Robert P |b 23 |
| 700 | 1 | _ | |a Klimo, Paul |b 24 |
| 700 | 1 | _ | |a Patay, Zoltan |0 0000-0003-1479-9864 |b 25 |
| 700 | 1 | _ | |a Indelicato, Daniel J |b 26 |
| 700 | 1 | _ | |a Nichols, Kim E |0 0000-0002-5581-6555 |b 27 |
| 700 | 1 | _ | |a Boop, Frederick A |b 28 |
| 700 | 1 | _ | |a Merchant, Thomas E |0 0000-0002-0412-6255 |b 29 |
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| 700 | 1 | _ | |a Ellison, David W |0 0000-0003-1239-7757 |b 31 |
| 700 | 1 | _ | |a Gajjar, Amar |0 0000-0001-5019-0699 |b 32 |
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