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Original Articles
Pediatric cancer
Gonadal Function in Female Adolescent and Young Adult Survivors of Childhood Cancer
Hye Young Jin, Jun Ah Lee, Meerim Park, Hyeon Jin Park
Cancer Res Treat. 2023;55(3):992-1000.   Published online January 31, 2023
DOI: https://doi.org/10.4143/crt.2022.1518
AbstractAbstract PDFPubReaderePub
Purpose
Childhood cancer survivors (CCSs) are at risk for premature ovarian insufficiency (POI). The aim of this study is to evaluate ovarian function and associated health outcomes in female adolescent and young adult survivors of childhood cancer.
Materials and Methods
Sixty-nine female CCSs were enrolled. Medical records of CCSs were retrospectively reviewed. The subjects were categorized into three groups according to follicular stimulating hormone (FSH) levels (cutoff, 12, 40 IU/L). Anti-müllerian hormone (AMH) level less than 1 ng/mL was considered low AMH level.
Results
Of 69 subjects, 14 (20.3%) had POI and 14 (20.3%) had FSH levels between 12 and 40 IU/L. Forty-one of 69 (59.4%) had normal FSH levels. Pelvic irradiation and stem cell transplantation (SCT) were more frequently performed in subjects with POI (p=0.001 and p < 0.001). AMH levels were remarkably low when FSH levels were over 12 IU/L (p < 0.001). In multivariate analysis, cyclophosphamide equivalent dose and SCT were significant treatment factors for developing low AMH levels (p=0.005 and p=0.002, respectively). Total, low-density lipoprotein cholesterol and triglyceride were significantly different in three groups according to FSH levels (p=0.047, p=0.030, and p=0.045). Z-score of femur neck bone mineral density was significantly reduced when FSH levels were increased (p=0.011).
Conclusion
Gonadal dysfunction is common in CCSs. Gonadal function was associated with a few treatment factors known to increase the risk of POI. Regular monitoring of gonadal function is needed for better health outcomes.

Citations

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  • Systematic Review of the Gonadotoxicity and Risk of Infertility of Soft Tissue Sarcoma Chemotherapies in Pre- and Postpubertal Females and Males
    Marcel Steinmann, Anita Rietschin, Flavia Pagano, Tanya Karrer, Attila Kollár, Susanna Weidlinger, Michael von Wolff
    Journal of Adolescent and Young Adult Oncology.2024;[Epub]     CrossRef
  • Bone Mineral Density in Survivors of Childhood Cancer: A Meta-Analysis
    Lilly Velentza, Panagiotis Filis, Mari Wilhelmsson, Per Kogner, Nikolas Herold, Lars Sävendahl
    Pediatrics.2024;[Epub]     CrossRef
  • 3,166 View
  • 160 Download
  • 2 Web of Science
  • 2 Crossref
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Epidemiologic and Clinical Outcomes of Pediatric Renal Tumors in Korea: A Retrospective Analysis of The Korean Pediatric Hematology and Oncology Group (KPHOG) Data
Kyung-Nam Koh, Jung Woo Han, Hyoung Soo Choi, Hyoung Jin Kang, Ji Won Lee, Keon Hee Yoo, Ki Woong Sung, Hong Hoe Koo, Kyung Taek Hong, Jung Yoon Choi, Sung Han Kang, Hyery Kim, Ho Joon Im, Seung Min Hahn, Chuhl Joo Lyu, Hee-Jo Baek, Hoon Kook, Kyung Mi Park, Eu Jeen Yang, Young Tak Lim, Seongkoo Kim, Jae Wook Lee, Nack-Gyun Chung, Bin Cho, Meerim Park, Hyeon Jin Park, Byung-Kiu Park, Jun Ah Lee, Jun Eun Park, Soon Ki Kim, Ji Yoon Kim, Hyo Sun Kim, Youngeun Ma, Kyung Duk Park, Sang Kyu Park, Eun Sil Park, Ye Jee Shim, Eun Sun Yoo, Kyung Ha Ryu, Jae Won Yoo, Yeon Jung Lim, Hoi Soo Yoon, Mee Jeong Lee, Jae Min Lee, In-Sang Jeon, Hye Lim Jung, Hee Won Chueh, Seunghyun Won, the Korean Pediatric Hematology and Oncology Group (KPHOG)
Cancer Res Treat. 2023;55(1):279-290.   Published online August 11, 2022
DOI: https://doi.org/10.4143/crt.2022.073
AbstractAbstract PDFSupplementary MaterialPubReaderePub
Purpose
Renal tumors account for approximately 7% of all childhood cancers. These include Wilms tumor (WT), clear cell sarcoma of the kidney (CCSK), malignant rhabdoid tumor of the kidney (MRTK), renal cell carcinoma (RCC), congenital mesoblastic nephroma (CMN) and other rare tumors. We investigated the epidemiology of pediatric renal tumors in Korea.
Materials and Methods
From January 2001 to December 2015, data of pediatric patients (0–18 years) newly-diagnosed with renal tumors at 26 hospitals were retrospectively analyzed.
Results
Among 439 patients (male, 240), the most common tumor was WT (n=342, 77.9%), followed by RCC (n=36, 8.2%), CCSK (n=24, 5.5%), MRTK (n=16, 3.6%), CMN (n=12, 2.7%), and others (n=9, 2.1%). Median age at diagnosis was 27.1 months (range 0-225.5) and median follow-up duration was 88.5 months (range 0-211.6). Overall, 32 patients died, of whom 17, 11, 1, and 3 died of relapse, progressive disease, second malignant neoplasm, and treatment-related mortality. Five-year overall survival and event free survival were 97.2% and 84.8% in WT, 90.6% and 82.1% in RCC, 81.1% and 63.6% in CCSK, 60.3% and 56.2% in MRTK, and 100% and 91.7% in CMN, respectively (p < 0.001).
Conclusion
The pediatric renal tumor types in Korea are similar to those previously reported in other countries. WT accounted for a large proportion and survival was excellent. Non-Wilms renal tumors included a variety of tumors and showed inferior outcome, especially MRTK. Further efforts are necessary to optimize the treatment and analyze the genetic characteristics of pediatric renal tumors in Korea.

Citations

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  • Congenital Mesoblastic Nephroma Mimic Wilms Tumor on 18F-FDG PET/CT and PET/MR
    Wenzhu Hu, Chunxia Qin, Fuqiang Shao, Mengting Li, Xiaoli Lan
    Clinical Nuclear Medicine.2024; 49(4): 353.     CrossRef
  • Progress towards Therapies for Solid Renal Tumors in Children
    洁 林
    Advances in Clinical Medicine.2024; 14(06): 245.     CrossRef
  • 6,811 View
  • 184 Download
  • 1 Web of Science
  • 2 Crossref
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Sarcoma
Incidence Patterns and Outcomes of Ewing Sarcoma in South Korea (1999-2017): A Retrospective Analysis Using Korea Central Cancer Registry Data
Jun Ah Lee, Jiwon Lim, Dayeon Park, Hye Young Jin, Meerim Park, Hyeon Jin Park, Jong Woong Park, June Hyuk Kim, Hyun Guy Kang, Young-Joo Won
Cancer Res Treat. 2022;54(2):590-596.   Published online July 20, 2021
DOI: https://doi.org/10.4143/crt.2021.311
AbstractAbstract PDFPubReaderePub
Purpose
Due to low incidence, epidemiologic data of Ewing sarcoma in the Asian population are scarce. We aimed to examine the incidence pattern and outcome of patients with Ewing sarcoma in the Republic of Korea.
Materials and Methods
Data of patients with Ewing sarcoma diagnosed between 1999 and 2017 were obtained from the Korea Central Cancer Registry (KCCR). Incidence, clinical characteristics, and survival rates were analyzed and compared between different age groups.
Results
There were 788 cases (459 males, 329 females), with a median age at diagnosis of 20 years. The age-standardized rate of Ewing sarcoma was 1.01. The number of cases and incidence rates in each age group were as follows: children, 1.6; adolescents and young adults (AYA), 0.93; adults, 0.44; and elderly, 0.53. There were more male cases in children and the AYA group (p < 0.001). Extraskeletal tumors (p < 0.001), primary sites other than extremity (p=0.007), and presence of metastasis at diagnosis (p=0.031) were more frequent in the adults and elderly group. With a median survival time of 78 months, the 5-year overall survival (OS) rate of the entire cohort was 52%. Children fared best (5-year OS, 75%), and the 5-year OS of AYA patients (51%) approximated the OS of the entire cohort. A two-fold difference of 5-year OS was observed between adults and elderly patients (42% vs. 19%). On univariate and multivariate analyses, age ≥ 15 years and presence of metastasis were adverse prognostic factors.
Conclusion
This was the first epidemiologic study of Ewing sarcoma using the KCCR data. With a similar incidence to other Asian countries, the survival rate was slightly lower than that of Euro-American cases. Collaborative clinical studies are necessary to improve the outcome of Ewing sarcoma in low-incidence populations.

Citations

Citations to this article as recorded by  
  • Twenty‐year outcome of prevalence, incidence, mortality and survival rate in patients with malignant bone tumors
    Yao Xu, Fanqi Shi, Yanting Zhang, Mengfan Yin, Xiuxin Han, Jinyan Feng, Guowen Wang
    International Journal of Cancer.2024; 154(2): 226.     CrossRef
  • A Machine Learning-Based Predictive Model for Predicting Lymph Node Metastasis in Patients With Ewing’s Sarcoma
    Wenle Li, Qian Zhou, Wencai Liu, Chan Xu, Zhi-Ri Tang, Shengtao Dong, Haosheng Wang, Wanying Li, Kai Zhang, Rong Li, Wenshi Zhang, Zhaohui Hu, Su Shibin, Qiang Liu, Sirui Kuang, Chengliang Yin
    Frontiers in Medicine.2022;[Epub]     CrossRef
  • TrkC, a novel prognostic marker, induces and maintains cell survival and metastatic dissemination of Ewing sarcoma by inhibiting EWSR1-FLI1 degradation
    Min Soo Kim, Won Sung Lee, Hanki Lee, Wook Jin
    Cell Death & Disease.2022;[Epub]     CrossRef
  • 5,884 View
  • 120 Download
  • 2 Web of Science
  • 3 Crossref
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Pediatric cancer
Vincristine, Irinotecan, and Temozolomide as a Salvage Regimen for Relapsed or Refractory Sarcoma in Children and Young Adults
Hee Young Ju, Meerim Park, Jun Ah Lee, Hyeon Jin Park, Seog Yun Park, June Hyuk Kim, Hyun Guy Kang, Hee Chul Yang, Byung-Kiu Park
Cancer Res Treat. 2022;54(2):563-571.   Published online June 14, 2021
DOI: https://doi.org/10.4143/crt.2021.178
AbstractAbstract PDFPubReaderePub
Purpose
No standard salvage regimen is available for relapsed or refractory sarcoma. We investigated the efficacy and toxicity of the vincristine, irinotecan, and temozolomide combination (VIT) for relapsed or refractory sarcomas of variable histology in children and young adults.
Materials and Methods
We retrospectively reviewed data from the relapsed or refractory sarcoma patients who were treated with VIT. The VIT protocol was given every 3 weeks as follows: vincristine, 1.5 mg/m2 intravenously on day 1, irinotecan, 50 mg/m2/day intravenously on days 1-5, and temozolomide, 100 mg/m2/day orally on days 1-5.
Results
A total of 26 patients (12 males) with various sarcoma histology were included in the study. Most common diagnosis was rhabdomyosarcoma (n=8) followed by osteosarcoma (n=7). Median age at the start of VIT was 18.5 years (range, 2.0 to 39.9). VIT was delivered as 2nd to 7th line of treatment, with 4th line most common (9/26, 34.6%). Median number of VIT courses given was 3 (range, 1 to 18). Of the 25 evaluable patients, there was two partial response (PR) and 11 stable disease (SD) with an overall control rate (complete remission+PR+SD) of 52%. PR was seen in one (50%) of the two evaluable patients with Ewing sarcoma and one (14.3%) of the seven patients with osteosarcoma. Overall survival and progression-free survival rates were 79.3% and 33.9% at 1 year, and 45.5% and 25.4% at 2 years, respectively. There was no treatment-related mortality.
Conclusion
The VIT regimen was effective and relatively safe in our cohort of sarcoma patients.

Citations

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  • MGMT protein expression is a reliable predictive biomarker for temozolomide‐containing chemotherapy in osteosarcoma
    Yoshinori Uchihara, Katsutsugu Umeda, Yosuke Yamada, Hiroaki Ito, Keiji Tasaka, Kiyotaka Isobe, Ryo Akazawa, Naoko Kawabata, Satoshi Saida, Itaru Kato, Hidefumi Hiramatsu, Takashi Noguchi, Akio Sakamoto, Yoshiki Arakawa, Ayumu Arakawa, Nobuyuki Yamamoto,
    Cancer Science.2024; 115(10): 3394.     CrossRef
  • Anlotinib combined with radiotherapy and chemotherapy for recurrent pelvic osteosarcoma treatment: a case report and literature review
    Qian Chen, Kai Zheng, Ming Xu, Ning Yan, Gong Hai, Xiuchun Yu
    Frontiers in Oncology.2023;[Epub]     CrossRef
  • Irinotecan/temozolomide/vincristine

    Reactions Weekly.2022; 1910(1): 267.     CrossRef
  • Acetylenic Synthetic Betulin Derivatives Inhibit Akt and Erk Kinases Activity, Trigger Apoptosis and Suppress Proliferation of Neuroblastoma and Rhabdomyosarcoma Cell Lines
    Sylwia K. Król, Ewa Bębenek, Magdalena Dmoszyńska-Graniczka, Adrianna Sławińska-Brych, Stanisław Boryczka, Andrzej Stepulak
    International Journal of Molecular Sciences.2021; 22(22): 12299.     CrossRef
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  • 362 Download
  • 5 Web of Science
  • 4 Crossref
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Special Article
Guidelines for Cancer Care during the COVID-19 Pandemic in South Korea
Jii Bum Lee, Minkyu Jung, June Hyuk Kim, Bo Hyun Kim, Yeol Kim, Young Seok Kim, Byung Chang Kim, Jin Kim, Sung Ho Moon, Keon-Uk Park, Meerim Park, Hyeon Jin Park, Sung Hoon Sim, Hong Man Yoon, Soo Jung Lee, Eunyoung Lee, June Young Chun, Youn Kyung Chung, So-Youn Jung, Jinsoo Chung, Eun Sook Lee, Hyun Cheol Chung, Tak Yun, Sun Young Rha
Cancer Res Treat. 2021;53(2):323-329.   Published online March 15, 2021
DOI: https://doi.org/10.4143/crt.2020.1256
AbstractAbstract PDFPubReaderePub
At the end of 2019, the cause of pneumonia outbreaks in Wuhan, China, was identified as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. In February 2020, the World Health Organization named the disease cause by SARS-CoV-2 as coronavirus disease 2019 (COVID-19). In response to the pandemic, the Korean Cancer Association formed the COVID-19 task force to develop practice guidelines. This special article introduces the clinical practice guidelines for cancer patients which will help oncologists best manage cancer patients during the COVID-19 pandemic.

Citations

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    Jae-Woo Ju, Soo-Hyuk Yoon, Tak Kyu Oh, Ho-Jin Lee
    Annals of Surgical Oncology.2024; 31(10): 6394.     CrossRef
  • Impact of the COVID-19 Pandemic on Esophagogastroduodenoscopy and Gastric Cancer Claims in South Korea: A Nationwide, Population-Based Study
    Min Ah Suh, Su Bee Park, Min Seob Kwak, Jin Young Yoon, Jae Myung Cha
    Yonsei Medical Journal.2023; 64(9): 549.     CrossRef
  • The elderly population are more vulnerable for the management of colorectal cancer during the COVID-19 pandemic: a nationwide, population-based study
    Hong Sun Kang, Seung Hoon Jeon, Su Bee Park, Jin Young Youn, Min Seob Kwak, Jae Myung Cha
    Intestinal Research.2023; 21(4): 500.     CrossRef
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    Journal of Gastric Cancer.2023; 23(4): 574.     CrossRef
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    Eun-Gyeong Lee, Yireh Han, Dong-Eun Lee, Hyeong-Gon Moon, Hyoung Won Koh, Eun-Kyu Kim, So-Youn Jung
    Cancer Research and Treatment.2023; 55(4): 1222.     CrossRef
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    Medical Oncology.2022;[Epub]     CrossRef
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    Brenda Bogaert, Victoria Buisson, Zizis Kozlakidis, Pierre Saintigny
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Original Articles
Central nervous system
Atypical Teratoid/Rhabdoid Tumor of the Central Nervous System in Children under the Age of 3 Years
Meerim Park, Jung Woo Han, Seung Min Hahn, Jun Ah Lee, Joo-Young Kim, Sang Hoon Shin, Dong-Seok Kim, Hong In Yoon, Kyung Taek Hong, Jung Yoon Choi, Hyoung Jin Kang, Hee Young Shin, Ji Hoon Phi, Seung-Ki Kim, Ji Won Lee, Keon Hee Yoo, Ki Woong Sung, Hong Hoe Koo, Do Hoon Lim, Hyung Jin Shin, Hyery Kim, Kyung-Nam Koh, Ho Joon Im, Seung Do Ahn, Young-Shin Ra, Hee-Jo Baek, Hoon Kook, Tae-Young Jung, Hyoung Soo Choi, Chae-Yong Kim, Hyeon Jin Park, Chuhl Joo Lyu
Cancer Res Treat. 2021;53(2):378-388.   Published online October 28, 2020
DOI: https://doi.org/10.4143/crt.2020.756
AbstractAbstract PDFPubReaderePub
Purpose
Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive malignancy with peak incidence in children aged less than 3 years. Standard treatment for central nervous system ATRT in children under the age of 3 years have not been established yet. The objective of this study was to analyze characteristics and clinical outcomes of ATRT in children aged less than 3 years.
Materials and Methods
A search of medical records from seven centers was performed between January 2005 and December 2016.
Results
Forty-three patients were enrolled. With a median follow-up of 90 months, 27 patients (64.3%) showed at least one episode of disease progression (PD). The first date of PD was at 160 days after diagnosis. The 1- and 3-year progression-free survivals (PFS) were 51.2% and 28.5%, respectively. The 1- and 3-year overall survivals were 61.9% and 38.1%, respectively. The 3-year PFS was improved from 0% in pre-2011 to 47.4% in post-2011. Excluding one patient who did not receive any further therapy after surgery, 27 patients died due to PD (n=21), treatment-related toxicity (n=5), or unknown cause (n=1). In univariate analysis, factors associated with higher 3-year PFS were no metastases, diagnosis after 2011, early adjuvant radiotherapy, and high-dose chemotherapy (HDCT). In multivariate analysis, the use of HDCT and adjuvant radiotherapy remained significant prognostic factors for PFS (both p < 0.01).
Conclusion
Aggressive therapy including early adjuvant radiotherapy and HDCT could be considered to improve outcomes of ATRT in children under the age of 3 years.

Citations

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  • Supratentorial ATRT in a young Infant: Expanding the diagnostic spectrum beyond medulloblastoma
    Ali Msheik, Mohamad Aoun, Youssef Fares
    Interdisciplinary Neurosurgery.2024; 35: 101857.     CrossRef
  • Radiation Therapy Plays an Important Role in the Treatment of Atypical Teratoid/Rhabdoid Tumors: Analysis of the EU-RHAB Cohorts and Their Precursors
    Sabine Frisch, Hanna Libuschewski, Sarah Peters, Joachim Gerß, Katja von Hoff, Rolf-Dieter Kortmann, Karolina Nemes, Stefan Rutkowski, Martin Hasselblatt, Torsten Pietsch, Michael C. Frühwald, Beate Timmermann
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    Antoinette J Charles, Vanessa L Smith, C Rory Goodwin, Margaret O Johnson
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    Sihao Chen, Yi He, Jiao Liu, Ruixin Wu, Menglei Wang, Aishun Jin
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    Beate Timmermann, Claire Alapetite, Karin Dieckmann, Rolf-Dieter Kortmann, Yasmin Lassen-Ramshad, John H. Maduro, Monica Ramos Albiac, Umberto Ricardi, Damien C. Weber
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    L. V. Olkhova, O. G. Zheludkova, L. S. Zubarovskaya, A. S. Levashov, A. Yu. Smirnova, Yu. V. Dinikina, Yu. V. Kushel, A. G. Melikyan, S. K. Gorelyshev, M. V. Ryzhova, Yu. Yu. Trunin, A. G. Gevorgyan, O. B. Polushkina, V. E. Popov, L. P. Privalova, N. B. Y
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    Chi-kong Li, Purna Kurkure, Ramandeep Singh Arora, Bow Wen Chen, Kirill Kirgizov, Yasuhiro Okamoto, Panya Seksarn, Yongmin Tang, Keon Hee Yoo, Bharat Agarwal, Godfrey C.F. Chan, Rashmi Dalvi, Hiroki Hori, Muhammad Saghir Khan, Alice Yu, Akira Nakagawara
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    Tae-Hwan Park, Seung-Ki Kim, Ji Hoon Phi, Chul-Kee Park, Yong Hwy Kim, Sun Ha Paek, Chang-Hyun Lee, Sung-Hye Park, Eun Jung Koh
    Brain Tumor Research and Treatment.2023; 11(4): 254.     CrossRef
  • Atypical Teratoid/Rhabdoid Tumor in Taiwan: A Nationwide, Population-Based Study
    Yen-Lin Liu, Min-Lan Tsai, Chang-I Chen, Noi Yar, Ching-Wen Tsai, Hsin-Lun Lee, Chia-Chun Kuo, Wan-Ling Ho, Kevin Li-Chun Hsieh, Sung-Hui Tseng, James S. Miser, Chia-Yau Chang, Hsi Chang, Wen-Chang Huang, Tai-Tong Wong, Alexander T. H. Wu, Yu-Chun Yen
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    Cinzia Baiano, Rosa Della Monica, Raduan Ahmed Franca, Maria Laura Del Basso De Caro, Luigi Maria Cavallo, Lorenzo Chiariotti, Tamara Ius, Emmanuel Jouanneau, Teresa Somma
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    Vismaya S. Bachu, Pavan Shah, Adrian E. Jimenez, Adham M. Khalafallah, Jignesh Tailor, Debraj Mukherjee, Alan R. Cohen
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    Yukitomo Ishi, Yongzhan Zhang, Ali Zhang, Takahiro Sasaki, Andrea Piunti, Amreena Suri, Jun Watanabe, Kouki Abe, Xingyao He, Hiroaki Katagi, Pankaj Bhalla, Manabu Natsumeda, Lihua Zou, Ali Shilatifard, Rintaro Hashizume
    Molecular Cancer Therapeutics.2022; 21(5): 715.     CrossRef
  • Molecular targeted therapies for pediatric atypical teratoid/rhabdoid tumors
    Chang Zhang, Hao Li
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  • The results of multicenter treatment of atypical teratoid/rhabdoid tumors of the central nervous system in children under 3 years
    L. V. Olkhova, O. G. Zheludkova, L. S. Zubarovskaya, A. Yu. Smirnova, Yu. V. Dinikina, Yu. V. Kushel, A. G. Melikyan, S. K. Gorelyshev, M. V. Ryzhova, Yu. Yu. Trunin, E. I. Shults, A. G. Gevorgyan, S. V. Gorbatykh, A. N. Kislyakov, V. E. Popov, L. P. Priv
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  • 8,899 View
  • 297 Download
  • 13 Web of Science
  • 15 Crossref
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General
Cancer Incidence and Survival among Adolescents and Young Adults in Korea: An Update for 2016
Meerim Park, Jiwon Lim, Jun Ah Lee, Byung Kiu Park, Kyu-Won Jung, Young-Joo Won, Hyeon Jin Park
Cancer Res Treat. 2021;53(1):32-44.   Published online October 5, 2020
DOI: https://doi.org/10.4143/crt.2020.644
AbstractAbstract PDFSupplementary MaterialPubReaderePub
Purpose
This study investigated the incidence and relative survival rates (RSRs) for cancers among adolescents and young adults (AYAs) aged 15-39 years between 1993 and 2016 in Korea
Materials and Methods
Data from the Korea Central Cancer Registry were used to calculate percent distributions, age-specific incidence rates, age-standardized incidence rates (ASRs) per million, annual percent changes (APCs), average APCs, and RSRs for cancers diagnosed in AYAs.
Results
ASR of all cancers among AYAs was 654.5 per million. The largest diagnosed group of cancers was carcinomas (almost 80%). Crude incidence increased with age, from 170.4 per million for those aged 15-19 years to 1,639.8 per million for those aged 35-39 years. ASR increased from 414.8 per million to 820.4 per million, with an APC of 9.0%. The incidence of thyroid carcinoma showed the most rapid increment (APC, 14.0%), followed by non-Hodgkin lymphoma (APC, 13.4%). The 5-year RSR among AYAs significantly improved from 62.1% to 90.8%. Survival improvement in AYAs was higher than that in children but lower than that in older adults (APC, 2.1% vs. 1.9% vs. 3.1%). The most marked survival improvement was found for leukemia and lymphoma. Astrocytoma, rhabdomyosarcoma, and carcinoma of the trachea, bronchus, and lung had a 5-year RSR of < 50%.
Conclusion
There was an improvement in cancer survivals in AYAs, comparable to that achieved in children. However, survivals in several cancer types do not appear to be improving. Further research focusing on the epidemiology and therapeutic strategies for cancers in AYAs is needed.

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Case Report
Hyperammonemic Encephalopathy Mimicking Ornithine Transcarbamylase Deficiency in Fibrolamellar Hepatocellular Carcinoma: Successful Treatment with Continuous Venovenous Hemofiltration and Ammonia Scavengers
Jeong-Seon Lee, Hye Young Jin, Jung Min Ko, Seoung Hoon Kim, Nayoung Han, Byung Kiu Park, Meerim Park, Hyeon Jin Park, Jun Ah Lee
Cancer Res Treat. 2021;53(1):283-288.   Published online September 7, 2020
DOI: https://doi.org/10.4143/crt.2020.575
AbstractAbstract PDFPubReaderePub
Fibrolamellar hepatocellular carcinoma (FLHCC) is a rare liver cancer affecting adolescents and young adults without any pre existing liver disease. Hyperammonemic encephalopathy (HAE) is a serious paraneoplastic syndrome, and several cases of HAE have been reported in patients with FLHCC. This condition is rare; hence, there are currently no management guidelines for cancer-related HAE. Herein, we report a case of an 18-year-old man with advanced FLHCC who developed HAE during the first course of chemotherapy consisting of cisplatin, doxorubicin, 5-fluorouracil, and interferon-α. He was successfully treated with continuous venovenous hemofiltration, sodium benzoate, sodium phenylbutyrate, and amino acid supplementation for HAE. After the second course of chemotherapy, he underwent surgery, and thereafter, his ammonia levels were normal without any ammonia scavenger therapy. Treatments for HAE described here will be helpful for this rare, but serious metabolic complication of FLHCC and could partially applied to HAE related to any malignancies.

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Original Articles
Nationwide Statistical Analysis of Lymphoid Malignancies in Korea
Hyewon Lee, Hyeon Jin Park, Eun-Hye Park, Hee Young Ju, Chang-Mo Oh, Hyun-Joo Kong, Kyu-Won Jung, Byung-Kiu Park, Eunyoung Lee, Hyeon-Seok Eom, Young-Joo Won
Cancer Res Treat. 2018;50(1):222-238.   Published online March 30, 2017
DOI: https://doi.org/10.4143/crt.2017.093
AbstractAbstract PDFSupplementary MaterialPubReaderePub
Purpose
Regional differences in the incidence of lymphoid malignancies have been reported worldwide, but there has been no large-scale epidemiologic analysis in Korea. The aim of this study was to provide a nationwide population-based statistical analysis of Korean patients with lymphoid malignancies.
Materials and Methods
The Korea Central Cancer Registry analyzed the incidence and survival of patients with lymphoid malignancies from the Korean National Cancer Incidence Database. Diseases were grouped by clinically relevant categories based on the 2008 World Health Organization classification.
Results
Overall 65,948 lymphoid diseases were identified between 1999 and 2012. The incidence of most subtypes increased with age, except for precursor cell neoplasms. Male predominance (male:female ratio=1.28:1) was observed. In 2012, annual age-standardized incidence rates per 100,000 persons of Hodgkin’s lymphoma, mature B-cell neoplasm, mature T/natural killer (NK)–cell neoplasm, and precursor cell neoplasm were 0.46, 6.60, 0.95, and 1.50, respectively, and they increased yearly from 1999. Composite Hodgkin’s and non-Hodgkin’s lymphomas were extremely rare. Survival improvement estimated using 5-year relative survival rate was observed in patients with Hodgkin’s lymphoma (71.1%- 83.0%), diffuse large B-cell lymphoma (49.5%-61.5%), plasma cell neoplasms (20.2%- 36.9%), and lymphoblastic lymphoma/leukemia (41.5%-56.3%) between 1993 and 2012. However, survival rates of T/NK-cell lymphoma (excluding cutaneous T-cell lymphoma) ranged from 40.5%-43.5% during the study period. Survival rates decreased with age in most subtypes.
Conclusion
This report presented the subtype-specific statistical analysis of lymphoid malignancies in the Korean population, showing increasing incidences and survival rates in most subtypes.

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Gonadal and Sexual Dysfunction in Childhood Cancer Survivors
Ju Young Yoon, Hyeon Jin Park, Hee Young Ju, Jong Hyung Yoon, Jin Soo Chung, Sang Hyun Hwang, Dong Ock Lee, Hye Young Shim, Byung-Kiu Park
Cancer Res Treat. 2017;49(4):1057-1064.   Published online January 25, 2017
DOI: https://doi.org/10.4143/crt.2016.197
AbstractAbstract PDFSupplementary MaterialPubReaderePub
Purpose
Few studies have addressed gonadal and sexual dysfunctions in childhood cancer survivors. We evaluated the prevalence rates and risk factors for gonadal failure among adolescent/young adult childhood cancer survivors and their sexual function.
Materials and Methods
Subjects were childhood cancer survivors aged 15-29 years who had completed therapy more than 2 years ago. Demographic and medical characteristics were obtained from the patients’ medical records. In addition, hormonal evaluation and semen analysis were performed and sexual function was evaluated via questionnaire.
Results
The study included 105 survivors (57 males, 48 females), of which 61 were adults (age > 19 years) and 44 were adolescents. In both males and females, the proportion of survivors with low sex hormone levels did not differ among age groups or follow-up period. Thirteen female subjects (27.1%) needed sex hormone replacement, while five males subjects (8.8%) were suspected of having hypogonadism, but none were receiving sex hormone replacement. Of 27 semen samples, 14 showed azospermia or oligospermia. The proportion of normospermia was lower in the high cyclophosphamide equivalent dose (CED) group (CED ≥ 8,000 mg/m2) than the low CED group (27.3% vs. 62.5%, p=0.047). Among adults, none were married and only 10 men (35.7%) and eight women (34.3%) were in a romantic relationship. Though a significant proportion (12.0% of males and 5.3% of females) of adolescent survivors had experienced sexual activity, 13.6% had not experienced sex education.
Conclusion
The childhood cancer survivors in this study showed a high prevalence of gonadal/sexual dysfunction; accordingly, proper strategies are needed to manage these complications.

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Nationwide Population-Based Incidence and Survival Rates of Malignant Central Nervous System Germ Cell Tumors in Korea, 2005-2012
Seung Hoon Lee, Kyu-Won Jung, Johyun Ha, Chang-Mo Oh, Hyeseon Kim, Hyeon Jin Park, Heon Yoo, Young-Joo Won
Cancer Res Treat. 2017;49(2):494-501.   Published online August 24, 2016
DOI: https://doi.org/10.4143/crt.2016.129
AbstractAbstract PDFPubReaderePub
Purpose
Malignant central nervous system (CNS) germ cell tumors (GCTs), although rare, are thought to occur more frequently among Asians. However, a recent population-based study revealed no differences in GCT incidence between Asians and Caucasians. Therefore, this study was conducted to determine the incidence and survival rates of CNS GCTs using the national cancer incidence database, and to compare these rates to those in the United States and Japan.
Materials and Methods
We extracted CNS GCT patients diagnosed between 2005 and 2012 from the Korea Central Cancer Registry database. Age-standardized rates (ASRs), annual percentage change, and the male-female incidence rate ratios (IRRs) were calculated. To estimate the survival rate, we used data for patients diagnosed between 2005 and 2010 and followed their cases until December 31, 2013.
Results
The ASR for CNS GCT between 2005 and 2012 was 0.179 per 100,000 (95% confidence interval, 0.166 to 0.193), with an overall male-to-female (M:F) IRR of 2.95:1. However, when stratified by site, the M:F IRR was 13.62:1 for tumors of the pineal region and 1.87:1 for those located in nonpineal regions. The most frequent histologic type was germinoma (76.0%), and the most frequent location was the suprasellar region (48.5%). The 5-year survival rate of germinoma patients was 95.3%.
Conclusion
The incidence rate of CNS GCTs in Korea during 2005-2012 was 0.179 per 100,000, which was similar to that of the Asian/Pacific Islander subpopulation in the United States. Moreover, the CNS GCT survival rate in Korea was similar to rates in Japan and the United States.

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Incidence and Survival of Childhood Cancer in Korea
Hyeon Jin Park, Eun-Kyeong Moon, Ju Young Yoon, Chang-Mo Oh, Kyu-Won Jung, Byung Kiu Park, Hee Young Shin, Young-Joo Won
Cancer Res Treat. 2016;48(3):869-882.   Published online January 21, 2016
DOI: https://doi.org/10.4143/crt.2015.290
AbstractAbstract PDFPubReaderePub
Purpose
An epidemiologic study of childhood cancer would provide useful information on cancer etiology and development of management guidelines.
Materials and Methods
Data from the Korea National Cancer Incidence Database were used to examine the incidence and survival of cancer in patients aged 0-14 years. Patients were grouped according to the International Classification of Childhood Cancer, 3rd edition. Age-specific and age-standardized incidences per million and estimated annual percentage change (APC) were calculated by sex and age. Five-year relative survival was calculated for four periods from 1993 to 2011.
Results
The study comprised 15,113 patients with malignant neoplasms. Age-standardized incidence rates for all cancers were 134.9 per million children in 1999-2011 and 144.0 and 124.9 per million for males and females, respectively (M/F ratio, 1.2; p < 0.05). The highest incidences were observed for ‘leukemias, myeloproliferative diseases, and myelodysplastic diseases’ (group I) (46.4), ‘central nervous system neoplasms’ (group III) (18.3), and ‘lymphomas and reticuloendothelial neoplasms’ (group II) (13.4). Age-standardized incidence increased from 117.9 in 1999 to 155.3 in 2011, with an APC of 2.4% (95% confidence interval, 2.1 to 2.7). There was a significant increase of APC in ‘neuroblastoma and other peripheral nervous cell tumors’ (group IV) (5.6%) and ‘other malignant epithelial neoplasms and malignant melanomas’ (group XI) (5.6%). The 5-year relative survival rate for all childhood cancers improved significantly from 56.2% (1993-1995) to 78.2% (2007-2011) (males, 56.7% to 77.7%; females, 55.5% to 78.8%).
Conclusion
This study provides reliable information on incidence and survival trends for childhood cancer in Korea.

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Clinical Results of Chemotherapy based Treatment in Retinoblastoma Patients: A Single Center Experience
Hyery Kim, Ji Won Lee, Hyoung Jin Kang, Hyeon Jin Park, Yoon Yi Kim, Hee Young Shin, Young Suk Yu, Il Han Kim, Hyo Seop Ahn
Cancer Res Treat. 2008;40(4):164-171.   Published online December 31, 2008
DOI: https://doi.org/10.4143/crt.2008.40.4.164
AbstractAbstract PDFPubReaderePub
Purpose

Retinoblastoma is the most common intraocular malignancy in children. Since the 1990s, chemotherapy was indicated for intraocluar disease to reduce the frequency of enucleation and spare the complications associated with external beam radiation. In this study, we analyzed treatment results of retinoblastoma in our institute.

Materials and Methods

Datas from children diagnosed with retinoblastoma and treated at Seoul National University Children's Hospital between 1986 and 2008 were analyzed retrospectively. We utilized cyclophosphamide, vincristine, adriamycin, and methotrexate (CVAM) for OPD-based adjuvant chemotherapy. From 1990, primary chemotherapy was administered to patients with intraocular disease for eyeball-saving and patients received a combination of etoposide, vincristine, cisplatin (or ifosfamide) as a moderately intensive regimen, or a combination of cisplatin, doxorubicin, etoposide, and cycophosphamide (CDEC) as a highly intensive regimen.

Results

One hundred eighteen children were analyzed. There were 68 unilateral and 50 bilateral diseases. The median age at diagnosis was 1 year and Reese-Ellsworth stage V was the most common stage at the time of diagnosis. All patients were treated by chemotherapy-based multimodality methods, and primary chemotherapy was administered to 80 patients. The 10-year overall and event-free survival rate of all patients were 93.9% and 91.6%, respectively. Two patients who died were in the CDEC regimen group, but there was no significant statistical difference in survival rates by chemotherapy regimens. Fifty-six of 114 eyeballs were saved after primary chemotherapy-based treatment, and the eyeball-saving rate was 49.1%. Six patients relapsed after enucleation and 2 patients were treated successfully after autologous PBSCT. Osteosarcoma occurred in 2 patients as a secondary malignancy, and facial asymmetry after radiotherapy was the most common long-term sequelae.

Conclusions

In this study, the overall and event-free survival rates of retinoblastoma were satisfactory and eye-saving was possible with primary chemotherapy. Development of new chemotherapeutic regimens and a team approach are necessary to improve the eyeball-saving rate.

Citations

Citations to this article as recorded by  
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Treatment Result of Pediatric Osteosarcoma with Intraarterial Cisplatin
Hyoung Soo Choi, Hyoung Jin Kang, Jun Ah Lee, Hyo Heong Han, Hyeon Jin Park, Eun Sun Yoo, Woo Sun Kim, Hee Young Shin, In One Kim, Sang Hoon Lee, Hyo Seop Ahn, Han Koo Lee
J Korean Cancer Assoc. 1998;30(1):169-177.
AbstractAbstract PDF
PURPOSE
This study was performed to determine the outcome after treatment of osteosarcoma with intraarterial cisplatin as a preoperative chematherapy regimen.
MATERIALS AND METHODS
Twenty five patients with extremity osteosarcoma were treated with intraarterial cisplatin at Seoul National University Children's Hospital from January 1987 to April 1996. The dose of cisplatin was 130 mg/m2 and three to six courses were repeated two- to three-week intervals, Systemic doxorubicin was added to six of these patients. This was followed by surgical resection(limb salvage or amputation) and postoperative adjuvant chemotherapy.
RESULTS
Limb-salvage was possible in twenty of these twenty five patients. Pulmonary metastasis was present in five patients at diagnosis and developed later in three patients. In six patients treated with systemic doxorubicin, pulmonary metastasis was absent at diagnosis and during follow-up period. Local recurrence after limb salvage was occurred in one patient and treated with amputation and systemic chemotherapy. Seven patients died from pulmonary metastssis and one from unknown cause. The follow-up duration of these patients was three to eighty eight months(median twenty two months) and the overall five-year survival and event free survival rate were 62.1% and 57.5%, respectively.
CONCLUSION
These data demonstrate that intraarterial cisplatin can be used as an effective regimen preoperatively for pediatric patients with extremity osteosarcoma. The combined use of systemic doxorubicin is expected to improve survival in patients with pulmonary metastasis.
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