Challenges in the management of childhood intracranial germ cell tumors in middle-income countries: A 20-year retrospective review from a single tertiary center in Malaysia

Background: A higher incidence of pediatric intracranial germ cell tumors (iGCTs) in Asian countries compared with Western countries has been reported. In Malaysia, the literature regarding pediatric iGCTs have been nonexistent. The aim of this study was to review the management, survival, and long-...

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Main Authors: Rajagopal, Revathi, Leong, Sheng Hoay, Jawin, Vida, Foo, Jen Chun, Ahmad Bahuri, Nor Faizal, Mun, Kein Seong, Azman, Raja Rizal, Loh, Jasmin, Yap, Tsiao Yi, Ariffin, Hany, Moreira, Daniel C., Gottardo, Nicholas G., Bouffet, Eric, Ganesan, Dharmendra
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Published: 2021
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Online Access:http://eprints.um.edu.my/26317/
https://doi.org/10.1097/MPH.0000000000002116
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spelling my.um.eprints.263172022-02-18T08:36:02Z http://eprints.um.edu.my/26317/ Challenges in the management of childhood intracranial germ cell tumors in middle-income countries: A 20-year retrospective review from a single tertiary center in Malaysia Rajagopal, Revathi Leong, Sheng Hoay Jawin, Vida Foo, Jen Chun Ahmad Bahuri, Nor Faizal Mun, Kein Seong Azman, Raja Rizal Loh, Jasmin Yap, Tsiao Yi Ariffin, Hany Moreira, Daniel C. Gottardo, Nicholas G. Bouffet, Eric Ganesan, Dharmendra R Medicine RC0254 Neoplasms. Tumors. Oncology (including Cancer) RJ Pediatrics Background: A higher incidence of pediatric intracranial germ cell tumors (iGCTs) in Asian countries compared with Western countries has been reported. In Malaysia, the literature regarding pediatric iGCTs have been nonexistent. The aim of this study was to review the management, survival, and long-term outcomes of pediatric iGCTs at a single tertiary center in Malaysia. Patients and Methods: We retrospectively reviewed data from patients below 18 years of age with iGCTs treated at the University Malaya Medical Center (UMMC) from 1998 to 2017. Results: Thirty-four patients were identified, with a median follow-up of 3.54 years. Sixteen (47%) patients had pure germinoma tumors (PGs), and the remaining patients had nongerminomatous germ cell tumors (NGGCTs). The median age was 12 years, with a male:female ratio of 4.7:1. Abnormal vision, headache with vomiting, and diabetes insipidus were the commonest presenting symptoms. Twenty-eight patients received initial surgical interventions, 24 were treated with chemotherapy, and 28 received radiotherapy. Eight patients experienced relapses. The 5- and 10-year event-free survival rates were similar at 61.1%+/- 12.6% and 42.9%+/- 12.1% for PG and NGGCT, respectively. The 5- and 10-year overall survival rates were the same at 75.5%+/- 10.8% and 53.3%+/- 12.3% for PG and NGGCT, respectively. Four patients died of treatment-related toxicity. Most of the survivors experienced good quality of life with satisfactory neurologic status. Conclusions: The survival rate of childhood iGCTs in UMMC was inferior to that reported in developed countries. Late diagnosis, poor adherence to treatment, and treatment-related complications were the contributing factors. Although these results highlight a single institution experience, they most likely reflect similar treatment patterns, outcomes, and challenges in other centers in Malaysia. 2021-10 Article PeerReviewed Rajagopal, Revathi and Leong, Sheng Hoay and Jawin, Vida and Foo, Jen Chun and Ahmad Bahuri, Nor Faizal and Mun, Kein Seong and Azman, Raja Rizal and Loh, Jasmin and Yap, Tsiao Yi and Ariffin, Hany and Moreira, Daniel C. and Gottardo, Nicholas G. and Bouffet, Eric and Ganesan, Dharmendra (2021) Challenges in the management of childhood intracranial germ cell tumors in middle-income countries: A 20-year retrospective review from a single tertiary center in Malaysia. Journal of Pediatric Hematology/Oncology, Publis. ISSN 1077-4114, DOI https://doi.org/10.1097/MPH.0000000000002116 <https://doi.org/10.1097/MPH.0000000000002116>. https://doi.org/10.1097/MPH.0000000000002116 doi:10.1097/MPH.0000000000002116
institution Universiti Malaya
building UM Library
collection Institutional Repository
continent Asia
country Malaysia
content_provider Universiti Malaya
content_source UM Research Repository
url_provider http://eprints.um.edu.my/
topic R Medicine
RC0254 Neoplasms. Tumors. Oncology (including Cancer)
RJ Pediatrics
spellingShingle R Medicine
RC0254 Neoplasms. Tumors. Oncology (including Cancer)
RJ Pediatrics
Rajagopal, Revathi
Leong, Sheng Hoay
Jawin, Vida
Foo, Jen Chun
Ahmad Bahuri, Nor Faizal
Mun, Kein Seong
Azman, Raja Rizal
Loh, Jasmin
Yap, Tsiao Yi
Ariffin, Hany
Moreira, Daniel C.
Gottardo, Nicholas G.
Bouffet, Eric
Ganesan, Dharmendra
Challenges in the management of childhood intracranial germ cell tumors in middle-income countries: A 20-year retrospective review from a single tertiary center in Malaysia
description Background: A higher incidence of pediatric intracranial germ cell tumors (iGCTs) in Asian countries compared with Western countries has been reported. In Malaysia, the literature regarding pediatric iGCTs have been nonexistent. The aim of this study was to review the management, survival, and long-term outcomes of pediatric iGCTs at a single tertiary center in Malaysia. Patients and Methods: We retrospectively reviewed data from patients below 18 years of age with iGCTs treated at the University Malaya Medical Center (UMMC) from 1998 to 2017. Results: Thirty-four patients were identified, with a median follow-up of 3.54 years. Sixteen (47%) patients had pure germinoma tumors (PGs), and the remaining patients had nongerminomatous germ cell tumors (NGGCTs). The median age was 12 years, with a male:female ratio of 4.7:1. Abnormal vision, headache with vomiting, and diabetes insipidus were the commonest presenting symptoms. Twenty-eight patients received initial surgical interventions, 24 were treated with chemotherapy, and 28 received radiotherapy. Eight patients experienced relapses. The 5- and 10-year event-free survival rates were similar at 61.1%+/- 12.6% and 42.9%+/- 12.1% for PG and NGGCT, respectively. The 5- and 10-year overall survival rates were the same at 75.5%+/- 10.8% and 53.3%+/- 12.3% for PG and NGGCT, respectively. Four patients died of treatment-related toxicity. Most of the survivors experienced good quality of life with satisfactory neurologic status. Conclusions: The survival rate of childhood iGCTs in UMMC was inferior to that reported in developed countries. Late diagnosis, poor adherence to treatment, and treatment-related complications were the contributing factors. Although these results highlight a single institution experience, they most likely reflect similar treatment patterns, outcomes, and challenges in other centers in Malaysia.
format Article
author Rajagopal, Revathi
Leong, Sheng Hoay
Jawin, Vida
Foo, Jen Chun
Ahmad Bahuri, Nor Faizal
Mun, Kein Seong
Azman, Raja Rizal
Loh, Jasmin
Yap, Tsiao Yi
Ariffin, Hany
Moreira, Daniel C.
Gottardo, Nicholas G.
Bouffet, Eric
Ganesan, Dharmendra
author_facet Rajagopal, Revathi
Leong, Sheng Hoay
Jawin, Vida
Foo, Jen Chun
Ahmad Bahuri, Nor Faizal
Mun, Kein Seong
Azman, Raja Rizal
Loh, Jasmin
Yap, Tsiao Yi
Ariffin, Hany
Moreira, Daniel C.
Gottardo, Nicholas G.
Bouffet, Eric
Ganesan, Dharmendra
author_sort Rajagopal, Revathi
title Challenges in the management of childhood intracranial germ cell tumors in middle-income countries: A 20-year retrospective review from a single tertiary center in Malaysia
title_short Challenges in the management of childhood intracranial germ cell tumors in middle-income countries: A 20-year retrospective review from a single tertiary center in Malaysia
title_full Challenges in the management of childhood intracranial germ cell tumors in middle-income countries: A 20-year retrospective review from a single tertiary center in Malaysia
title_fullStr Challenges in the management of childhood intracranial germ cell tumors in middle-income countries: A 20-year retrospective review from a single tertiary center in Malaysia
title_full_unstemmed Challenges in the management of childhood intracranial germ cell tumors in middle-income countries: A 20-year retrospective review from a single tertiary center in Malaysia
title_sort challenges in the management of childhood intracranial germ cell tumors in middle-income countries: a 20-year retrospective review from a single tertiary center in malaysia
publishDate 2021
url http://eprints.um.edu.my/26317/
https://doi.org/10.1097/MPH.0000000000002116
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