scholarly journals ATRT-26. META-ANALYSIS OF TREATMENT MODALITIES IN METASTATIC ATYPICAL TERATOID/RHABDOID TUMORS IN CHILDREN

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii281-iii281
Author(s):  
Reena M Underiner ◽  
Mostafa Eltobgy ◽  
Joseph R Stanek ◽  
Jonathan L Finlay ◽  
Mohamed S AbdelBaki
Keyword(s):  
Surgical Resection ◽  
Meta Analysis ◽  
Univariate Analysis ◽  
Multivariable Analysis ◽  
Substantial Effect ◽  
Treatment Modalities ◽  
Risk Of Death ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

Abstract BACKGROUND Metastatic atypical teratoid/rhabdoid tumors (AT/RT) are aggressive central nervous system tumors that present during infancy and are associated with dismal outcomes. Patients receive multimodal treatment including surgical resection, systemic chemotherapy and one or more of intrathecal chemotherapy (IT), marrow-ablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) and radiation therapy (XRT). While data regarding treatment modalities for AT/RT patients exist, no comprehensive data have been published regarding the metastatic patient population. METHODS We performed a meta-analysis of 1,578 articles published through September 2018, including 44 studies with a total of 123 subjects. Additionally, seven patients were incorporated through chart review of patients treated at Nationwide Children’s Hospital. RESULTS Analysis of 130 patients revealed a 3-year overall survival (OS) of 25%. Age at diagnosis had a significant impact on survival (p=0.0355); 3-year OS for infants < 18 months was 21%; 18–36 months was 26%; and > 36 months was 36%. Location of the primary tumor, metastatic stage and extent of surgical resection did not have significant impact on OS. On univariate analysis, XRT (p<0.0001), IT (p=0.01) and AuHCR (p<0.0001) were found to significantly improve survival. The most substantial effect was noted in patients who received AuHCR (3-year OS of 60% versus 9% in those who did not). On multivariable analysis XRT (p=0.0006), IT (p=0.0124) and AuHCR (p<0.0001) were independently associated with reduced risk of death.

scholarly journals CMET-15. META-ANALYSIS OF TREATMENT MODALITIES IN METASTATIC ATYPICAL TERATOID/RHABDOID TUMORS IN CHILDREN

2017 ◽  
Vol 19 (suppl_6) ◽  
pp. vi42-vi42
Author(s):  
Reena Underiner ◽  
Mostafa Eltobgy ◽  
Joseph R Stanek ◽  
Jonathan L Finlay ◽  
Mohamed S AbdelBaki
Keyword(s):  
Meta Analysis ◽  
Treatment Modalities ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

Meta-Analysis of Treatment Modalities in Metastatic Atypical Teratoid/Rhabdoid Tumors in Children

2020 ◽  
Vol 108 ◽  
pp. 106-112 ◽  
Author(s):  
Reena M. Underiner ◽  
Mostafa Eltobgy ◽  
Joseph R. Stanek ◽  
Jonathan L. Finlay ◽  
Mohamed S. AbdelBaki
Keyword(s):  
Meta Analysis ◽  
Treatment Modalities ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

scholarly journals ATRT-36. META-ANALYSIS OF TREATMENT MODALITIES IN METASTATIC ATYPICAL TERATOID/RHABDOID TUMORS IN CHILDREN

2018 ◽  
Vol 20 (suppl_2) ◽  
pp. i35-i35
Author(s):  
Reena Underiner ◽  
Mostafa Eltobgy ◽  
Joseph Stanek ◽  
Jean Michaud ◽  
Wafik Zaky ◽  
...  
Keyword(s):  
Meta Analysis ◽  
Treatment Modalities ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

scholarly journals Molecular subgrouping of atypical teratoid/rhabdoid tumors—a reinvestigation and current consensus

2019 ◽  
Vol 22 (5) ◽  
pp. 613-624 ◽  
Author(s):  
Ben Ho ◽  
Pascal D Johann ◽  
Yura Grabovska ◽  
Mamy Jean De Dieu Andrianteranagna ◽  
Fupan Yao ◽  
...  
Keyword(s):  
Common Ground ◽  
Meta Analysis ◽  
Future Research ◽  
Molecular Subgroups ◽  
Genetic Event ◽  
Pathway Activation ◽  
Atypical Teratoid ◽  
Clinicopathological Significance ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

Abstract Background Atypical teratoid/rhabdoid tumors (ATRTs) are known to exhibit molecular and clinical heterogeneity even though SMARCB1 inactivation is the sole recurrent genetic event present in nearly all cases. Indeed, recent studies demonstrated 3 molecular subgroups of ATRTs that are genetically, epigenetically, and clinically distinct. As these studies included different numbers of tumors, various subgrouping techniques, and naming, an international working group sought to align previous findings and to reach a consensus on nomenclature and clinicopathological significance of ATRT subgroups. Methods We integrated various methods to perform a meta-analysis on published and unpublished DNA methylation and gene expression datasets of ATRTs and associated clinicopathological data. Results In concordance with previous studies, the analyses identified 3 main molecular subgroups of ATRTs, for which a consensus was reached to name them ATRT-TYR, ATRT-SHH, and ATRT-MYC. The ATRT-SHH subgroup exhibited further heterogeneity, segregating further into 2 subtypes associated with a predominant supratentorial (ATRT-SHH-1) or infratentorial (ATRT-SHH-2) location. For each ATRT subgroup we provide an overview of its main molecular and clinical characteristics, including SMARCB1 alterations and pathway activation. Conclusions The introduction of a common classification, characterization, and nomenclature of ATRT subgroups will facilitate future research and serve as a common ground for subgrouping patient samples and ATRT models, which will aid in refining subgroup-based therapies for ATRT patients.

scholarly journals Outcomes with respect to extent of surgical resection for pediatric atypical teratoid rhabdoid tumors

2019 ◽  
Vol 36 (4) ◽  
pp. 713-719
Author(s):  
Alexandra Richards ◽  
Ronak Ved ◽  
Christopher Murphy ◽  
Dawn Hennigan ◽  
John-Paul Kilday ◽  
...  
Keyword(s):  
Overall Survival ◽  
Surgical Resection ◽  
Total Resection ◽  
Term Survival ◽  
Common Location ◽  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors ◽  
Extent Of Surgical Resection

Abstract Purpose To evaluate overall survival for atypical teratoid rhabdoid tumors (ATRTs) in relation to extent of surgical resection. Methods The neurosurgical tumor databases from three UK Pediatric centers (University Hospital of Wales, Alder Hey and Royal Manchester Children’s Hospital) were analyzed. Patients with a diagnosis of ATRT were identified between 2000 and 2018. Data was collected regarding demographics, extent of resection, complications, and overall survival. Results Twenty-four patients diagnosed with ATRT underwent thirty-eight operations. The age range was 20 days to 147 months (median 17.5 months). The most common location for the tumor was the posterior fossa (nine patients; 38%). Six patients (25%) underwent a complete total resection (CTR), seven (29%) underwent a near total resection (NTR), eight (33.3%) underwent a subtotal resection (STR), and three patients (12.5%) had biopsy only. Two-thirds of patients who underwent a CTR are still alive, as of March 2019, compared to 29% in the NTR and 12.5% in the STR groups. Out of the thirty-eight operations, there were a total of twenty-two complications, of which the most common was pseudomeningocele (27%). The extent of surgical resection (p = 0.021), age at surgery (p = 0.00015), and the presence of metastases at diagnosis (0.015) significantly affected overall survival. Conclusions Although these patients are a highly vulnerable group, maximal resection is recommended where possible, for the best chance of long-term survival. However, near total resections are likely beneficial when compared with subtotal resections and biopsy alone. Maximal surgical resection should be combined with adjuvant therapies for the best long-term outcomes.

scholarly journals ATRT-05. RESULTS OF MULTICENTER TRIAL CONCERNING THE TREATMENT OF CHILDREN WITH ATYPICAL TERATOID/RHABDOID TUMORS (ATRT) OF THE CENTRAL NERVOUS SYSTEM

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii276-iii276
Author(s):  
Olga Zheludkova ◽  
Lyudmila Olkhova ◽  
Yuri Kushel’ ◽  
Armen Melikyan ◽  
Marina Ryzhova ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Progression Free Survival ◽  
Multicenter Trial ◽  
Intrathecal Methotrexate ◽  
Subtotal Resection ◽  
Tumor Biopsy ◽  
Atypical Teratoid ◽  
The Central Nervous System ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors

Abstract We analyzed 105 patients under 18 years. The median age was 21 months. There were 54 boys and 51 girls. The supratentorial tumors were in 53 patients, infratentorial in 48, and in spinal cord in 4. 60 had stage M0,29-М+and 16-Mx. All the patients got surgical treatment:total tumor removal in 34,subtotal in 37,partial in 30,and biopsy in 4;75 patients got chemoradiotherapy to ATRT-2006;6-CWS;13-EU-RHAB;5-HIT-SKK;individual schemes in 6. RESULTS: 47 are alive,1 was LFU, and 57 died. PFS was 32%±0.05; the five-year OS 40%±0.05. The median survival-30 months, the median progression-free survival-12 months, and the median of follow-up-23 months. PFS was significantly better in patients more than 12 months compared to patients younger than 12 months:40 and 12%;p=0.00161.After total resection PFS was higher compared to subtotal resection, partial resection, and tumor biopsy:48,38,0,and 0%(p=0.025). After chemoradiotherapy, PFS was higher compared to patients without radiotherapy: 49and 0%(р=0.0000000).PFS for stage M0 was higher compared to stage M+and stage Mx:41,15,and 27%,respectively(р=0.00032).PFS was better for the tumors in the spinal cord and infratentorial location compared to the supratentorial location:67,37,and 25%(p=0.0876).The survival rate was higher among the patients who got treatment according to the ATRT-2006 protocol compared to EU-RHAB, individual regimens, CWS, and HIT-SKK:39,19,17,17,and 0% respectively;p=0.00159.The survival was higher among the patients who got intraventricular/intrathecal Methotrexate,Cytarabine, Prednisolone than among the patients who got only Methotrexate or none at all:40,0,and 5%, respectively; p=0.00015. CONCLUSIONS: Survival was significantly better in patients more than 12month, without metastases, with total removal tumor, chemotheradiotherapy by ATRT-2006 protocol with i/t, i/v Methotrexate/Cytarabine/Prednisolone.

scholarly journals Histomorphology Evolution of Recurrent Atypical Teratoid/Rhabdoid Tumors

2015 ◽  
Vol 144 (suppl 2) ◽  
pp. A023-A023
Author(s):  
Esther Yoon ◽  
Taliya Farooq ◽  
Lawrence Sann ◽  
Eric Vail ◽  
George Kleinman
Keyword(s):  
Atypical Teratoid ◽  
Atypical Teratoid Rhabdoid Tumors ◽  
Rhabdoid Tumors
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