TY - JOUR
T1 - Meta-Analysis of Treatment Modalities in Metastatic Atypical Teratoid/Rhabdoid Tumors in Children
AU - Underiner, Reena M.
AU - Eltobgy, Mostafa
AU - Stanek, Joseph R.
AU - Finlay, Jonathan L.
AU - AbdelBaki, Mohamed S.
N1 - Funding Information:
We thank the Society for Neuro-Oncology as well as the International Symposium on Pediatric Neuro-Oncology for allowing us to present this material at the 2017 and 2018 meetings, respectively. We also thank the Ohio State University College of Medicine Roessler Research Scholarship for its funding support to R.M.U.
Funding Information:
We thank the Society for Neuro-Oncology as well as the International Symposium on Pediatric Neuro-Oncology for allowing us to present this material at the 2017 and 2018 meetings, respectively. We also thank the Ohio State University College of Medicine Roessler Research Scholarship for its funding support to R.M.U.
Publisher Copyright:
© 2020 Elsevier Inc.
PY - 2020/7
Y1 - 2020/7
N2 - Background: Metastatic atypical teratoid/rhabdoid tumors (AT/RTs) 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 patients. Methods: We performed a meta-analysis of 1578 articles published through September 2018, including 44 studies with a total of 123 subjects. In addition, seven patients were included 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 effect on survival (P = 0.0355); 3-year OS for infants less than 18 months was 21%, 18 to 36 months was 26%, and greater than 36 months was 36%. Location of the primary tumor, metastatic stage, and extent of surgical resection did not have a 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% vs 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. Conclusions: Although more research is warranted to make generalizable conclusions, these results suggest that treatment regimens for patients with metastatic AT/RTs should include AuHCR, XRT, and IT.
AB - Background: Metastatic atypical teratoid/rhabdoid tumors (AT/RTs) 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 patients. Methods: We performed a meta-analysis of 1578 articles published through September 2018, including 44 studies with a total of 123 subjects. In addition, seven patients were included 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 effect on survival (P = 0.0355); 3-year OS for infants less than 18 months was 21%, 18 to 36 months was 26%, and greater than 36 months was 36%. Location of the primary tumor, metastatic stage, and extent of surgical resection did not have a 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% vs 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. Conclusions: Although more research is warranted to make generalizable conclusions, these results suggest that treatment regimens for patients with metastatic AT/RTs should include AuHCR, XRT, and IT.
KW - ATRT
KW - Atypical teratoid rhabdoid tumor
KW - Brain neoplasm
KW - Metastasis
KW - Rhabdoid tumors
UR - http://www.scopus.com/inward/record.url?scp=85084387156&partnerID=8YFLogxK
U2 - 10.1016/j.pediatrneurol.2020.03.003
DO - 10.1016/j.pediatrneurol.2020.03.003
M3 - Article
C2 - 32402552
AN - SCOPUS:85084387156
SN - 0887-8994
VL - 108
SP - 106
EP - 112
JO - Pediatric Neurology
JF - Pediatric Neurology
ER -