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RARE-07. BONE MARROW ABLATIVE CHEMOTHERAPY ASSOCIATED WITH IMPROVED OUTCOMES AMONG PATIENTS WITH RELAPSED CENTRAL NERVOUS SYSTEM NON-GERMINOMATOUS GERM CELL TUMORS

PURPOSE: Patients with relapsed/refractory central nervous system (CNS) non-germinomatous germ cell tumors (NGGCTs) have poor outcomes. There is no universal agreement on managing these patients secondary to the rarity of the disease and lack of prospective trials. We aim to explore prognostic facto...

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Autores principales: Abu-Arja, Mohammad H, McGlynn, Mary Claire, Zaghloul, Mohammed Saad, Elhemaly, Ahmed, Bertrand, Kelsey, Gajjar, Amar, Ronsley, Rebecca, Cappellano, Andrea M, Dassi, Natalia, Czogala, Malgorzata, Graham, Richard, Baglivo, Sofi Jares, Dhall, Girish, Nagabushan, Sumanth, Abdelbaki, Mohamed S
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10259958/
http://dx.doi.org/10.1093/neuonc/noad073.136
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author Abu-Arja, Mohammad H
McGlynn, Mary Claire
Zaghloul, Mohammed Saad
Elhemaly, Ahmed
Bertrand, Kelsey
Gajjar, Amar
Ronsley, Rebecca
Cappellano, Andrea M
Dassi, Natalia
Czogala, Malgorzata
Graham, Richard
Baglivo, Sofi Jares
Dhall, Girish
Nagabushan, Sumanth
Abdelbaki, Mohamed S
author_facet Abu-Arja, Mohammad H
McGlynn, Mary Claire
Zaghloul, Mohammed Saad
Elhemaly, Ahmed
Bertrand, Kelsey
Gajjar, Amar
Ronsley, Rebecca
Cappellano, Andrea M
Dassi, Natalia
Czogala, Malgorzata
Graham, Richard
Baglivo, Sofi Jares
Dhall, Girish
Nagabushan, Sumanth
Abdelbaki, Mohamed S
author_sort Abu-Arja, Mohammad H
collection PubMed
description PURPOSE: Patients with relapsed/refractory central nervous system (CNS) non-germinomatous germ cell tumors (NGGCTs) have poor outcomes. There is no universal agreement on managing these patients secondary to the rarity of the disease and lack of prospective trials. We aim to explore prognostic factors that may help guide the management of patients with relapsed/refractory CNS NGGCTs. METHODS: We performed a multinational retrospective study of relapsed/refractory CNS NGGCTs. We summarized all data using standard descriptive statistics. Overall survival (OS) was evaluated using Kaplan-Meier and Fisher’s exact test to estimate odds ratios (OR) and 95% confidence intervals (CI) using SPSS software. RESULTS: We identified 28 patients from 7 centers. Twenty-three patients (82%) were males. The median age at initial diagnosis was 11 years (range: 8-15). Twelve patients (43%) suffered from relapse, and 16 patients (57%) had an initial refractory disease. The median time to first relapse/progression was 29 months (range: 5-26). Eight patients (29%) received bone marrow ablative chemotherapy (HDCx) at relapse/progression. Sixteen patients (57%) received re-irradiation at relapse/progression. Six patients (21%) achieved complete remission (CR) at relapse. Only eight patients (29%) were alive at the last follow-up. The median follow-up time since relapse/progression was 16 months (range: 1 -153 months). HDCx (OR=25, 95%CI: 2.9-2223) – not re-irradiation (OR=7.8 95%CI: 0.795-76.1) – was associated with being alive at the last follow-up. All patients who achieved CR at the end of relapse treatment survived. Only one patient with the refractory disease was alive at the last follow-up. CONCLUSIONS: Patients with refractory CNS NGGCTs have dismal outcomes. HDCx and achieving CR at the end of relapse therapy are associated with improved survival. A multinational registry is needed to collate standardized data to inform future prospective clinical trials and improve the outcomes of patients with relapsed/refractory CNS NGCCTs.
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spelling pubmed-102599582023-06-13 RARE-07. BONE MARROW ABLATIVE CHEMOTHERAPY ASSOCIATED WITH IMPROVED OUTCOMES AMONG PATIENTS WITH RELAPSED CENTRAL NERVOUS SYSTEM NON-GERMINOMATOUS GERM CELL TUMORS Abu-Arja, Mohammad H McGlynn, Mary Claire Zaghloul, Mohammed Saad Elhemaly, Ahmed Bertrand, Kelsey Gajjar, Amar Ronsley, Rebecca Cappellano, Andrea M Dassi, Natalia Czogala, Malgorzata Graham, Richard Baglivo, Sofi Jares Dhall, Girish Nagabushan, Sumanth Abdelbaki, Mohamed S Neuro Oncol Final Category: Germ Cell Tumors/Rare Tumors - RARE PURPOSE: Patients with relapsed/refractory central nervous system (CNS) non-germinomatous germ cell tumors (NGGCTs) have poor outcomes. There is no universal agreement on managing these patients secondary to the rarity of the disease and lack of prospective trials. We aim to explore prognostic factors that may help guide the management of patients with relapsed/refractory CNS NGGCTs. METHODS: We performed a multinational retrospective study of relapsed/refractory CNS NGGCTs. We summarized all data using standard descriptive statistics. Overall survival (OS) was evaluated using Kaplan-Meier and Fisher’s exact test to estimate odds ratios (OR) and 95% confidence intervals (CI) using SPSS software. RESULTS: We identified 28 patients from 7 centers. Twenty-three patients (82%) were males. The median age at initial diagnosis was 11 years (range: 8-15). Twelve patients (43%) suffered from relapse, and 16 patients (57%) had an initial refractory disease. The median time to first relapse/progression was 29 months (range: 5-26). Eight patients (29%) received bone marrow ablative chemotherapy (HDCx) at relapse/progression. Sixteen patients (57%) received re-irradiation at relapse/progression. Six patients (21%) achieved complete remission (CR) at relapse. Only eight patients (29%) were alive at the last follow-up. The median follow-up time since relapse/progression was 16 months (range: 1 -153 months). HDCx (OR=25, 95%CI: 2.9-2223) – not re-irradiation (OR=7.8 95%CI: 0.795-76.1) – was associated with being alive at the last follow-up. All patients who achieved CR at the end of relapse treatment survived. Only one patient with the refractory disease was alive at the last follow-up. CONCLUSIONS: Patients with refractory CNS NGGCTs have dismal outcomes. HDCx and achieving CR at the end of relapse therapy are associated with improved survival. A multinational registry is needed to collate standardized data to inform future prospective clinical trials and improve the outcomes of patients with relapsed/refractory CNS NGCCTs. Oxford University Press 2023-06-12 /pmc/articles/PMC10259958/ http://dx.doi.org/10.1093/neuonc/noad073.136 Text en © The Author(s) 2023. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Final Category: Germ Cell Tumors/Rare Tumors - RARE
Abu-Arja, Mohammad H
McGlynn, Mary Claire
Zaghloul, Mohammed Saad
Elhemaly, Ahmed
Bertrand, Kelsey
Gajjar, Amar
Ronsley, Rebecca
Cappellano, Andrea M
Dassi, Natalia
Czogala, Malgorzata
Graham, Richard
Baglivo, Sofi Jares
Dhall, Girish
Nagabushan, Sumanth
Abdelbaki, Mohamed S
RARE-07. BONE MARROW ABLATIVE CHEMOTHERAPY ASSOCIATED WITH IMPROVED OUTCOMES AMONG PATIENTS WITH RELAPSED CENTRAL NERVOUS SYSTEM NON-GERMINOMATOUS GERM CELL TUMORS
title RARE-07. BONE MARROW ABLATIVE CHEMOTHERAPY ASSOCIATED WITH IMPROVED OUTCOMES AMONG PATIENTS WITH RELAPSED CENTRAL NERVOUS SYSTEM NON-GERMINOMATOUS GERM CELL TUMORS
title_full RARE-07. BONE MARROW ABLATIVE CHEMOTHERAPY ASSOCIATED WITH IMPROVED OUTCOMES AMONG PATIENTS WITH RELAPSED CENTRAL NERVOUS SYSTEM NON-GERMINOMATOUS GERM CELL TUMORS
title_fullStr RARE-07. BONE MARROW ABLATIVE CHEMOTHERAPY ASSOCIATED WITH IMPROVED OUTCOMES AMONG PATIENTS WITH RELAPSED CENTRAL NERVOUS SYSTEM NON-GERMINOMATOUS GERM CELL TUMORS
title_full_unstemmed RARE-07. BONE MARROW ABLATIVE CHEMOTHERAPY ASSOCIATED WITH IMPROVED OUTCOMES AMONG PATIENTS WITH RELAPSED CENTRAL NERVOUS SYSTEM NON-GERMINOMATOUS GERM CELL TUMORS
title_short RARE-07. BONE MARROW ABLATIVE CHEMOTHERAPY ASSOCIATED WITH IMPROVED OUTCOMES AMONG PATIENTS WITH RELAPSED CENTRAL NERVOUS SYSTEM NON-GERMINOMATOUS GERM CELL TUMORS
title_sort rare-07. bone marrow ablative chemotherapy associated with improved outcomes among patients with relapsed central nervous system non-germinomatous germ cell tumors
topic Final Category: Germ Cell Tumors/Rare Tumors - RARE
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10259958/
http://dx.doi.org/10.1093/neuonc/noad073.136
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