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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.

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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; 13(6): 803.     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
  • 3,265 View
  • 163 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
  • 6,957 View
  • 188 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

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  • 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
  • 6,008 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
  • 6,384 View
  • 368 Download
  • 5 Web of Science
  • 4 Crossref
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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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    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
    International Journal of Radiation Oncology*Biology*Physics.2024; 119(4): 1147.     CrossRef
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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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  • Current Challenges of Asian National Children's Cancer Study Groups on Behalf of Asian Pediatric Hematology and Oncology Group
    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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  • Survival and Malignant Transformation of Pineal Parenchymal Tumors: A 30-Year Retrospective Analysis in a Single-Institution
    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
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    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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    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
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    Chang Zhang, Hao Li
    Pediatric Investigation.2022; 6(2): 111.     CrossRef
  • 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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  • 8,993 View
  • 300 Download
  • 13 Web of Science
  • 16 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.

Citations

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    Yu-Han Tsai, Yu-Tung Teng, Tai-Chuan Kuan, Jason J. Liu
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    Chenyu Luo, Na Li, Bin Lu, Jie Cai, Ming Lu, Yuhan Zhang, Hongda Chen, Min Dai
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  • Fertility‐sparing surgery for early‐stage cervical cancer: A case series study on the efficacy and feasibility of cervical conization followed by pelvic lymphadenectomy
    Mayuko Yamamoto, Takeshi Motohara, Yutaka Iwagoi, Shingo Tayama, Hironori Tashiro, Eiji Kondoh, Hidetaka Katabuchi
    Journal of Obstetrics and Gynaecology Research.2022; 48(6): 1444.     CrossRef
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    Hsin Wang, Yu-Han Tsai, Yaa-Hui Dong, Jason J. Liu
    Cancer Epidemiology.2022; 78: 102144.     CrossRef
  • Osteosarcoma in Adolescents and Young Adults
    Jun Ah Lee, Jiwon Lim, Hye Young Jin, Meerim Park, Hyeon Jin Park, Jong Woong Park, June Hyuk Kim, Hyun Guy Kang, Young-Joo Won
    Cells.2021; 10(10): 2684.     CrossRef
  • 8,144 View
  • 212 Download
  • 12 Web of Science
  • 10 Crossref
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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.

Citations

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Original Article
Risk Factor Analysis for Secondary Malignancy in Dexrazoxane-Treated Pediatric Cancer Patients
Hyery Kim, Hyoung Jin Kang, Kyung Duk Park, Kyung-Nam Koh, Ho Joon Im, Jong Jin Seo, Jae Wook Lee, Nack-Gyun Chung, Bin Cho, Hack Ki Kim, Jae Min Lee, Jeong Ok Hah, Jun Ah Lee, Young Ho Lee, Sang Kyu Park, Hee Jo Baek, Hoon Kook, Ji Yoon Kim, Heung Sik Kim, Hwang Min Kim, Hee Won Chueh, Meerim Park, Hoi Soo Yoon, Mee Jeong Lee, Hyoung Soo Choi, Hyo Seop Ahn, Yoshifumi Kawano, Ji Won Park, Seokyung Hahn, Hee Young Shin
Cancer Res Treat. 2019;51(1):357-367.   Published online May 14, 2018
DOI: https://doi.org/10.4143/crt.2017.457
AbstractAbstract PDFSupplementary MaterialPubReaderePub
Purpose
Dexrazoxane has been used as an effective cardioprotector against anthracycline cardiotoxicity. This study intended to analyze cardioprotective efficacy and secondary malignancy development, and elucidate risk factors for secondary malignancies in dexrazoxane-treated pediatric patients.
Materials and Methods
Data was collected from 15 hospitals in Korea. Patients who received any anthracyclines, and completed treatment without stem cell transplantation were included. For efficacy evaluation, the incidence of cardiac events and cardiac event-free survival rates were compared. Data about risk factors of secondary malignancies were collected.
Results
Data of total 1,453 cases were analyzed; dexrazoxane with every anthracyclines group (D group, 1,035 patients) and no dexrazoxane group (non-D group, 418 patients). Incidence of the reported cardiac events was not statistically different between two groups; however, the cardiac event-free survival rate of patients with more than 400 mg/m2 of anthracyclines was significantly higher in D group (91.2% vs. 80.1%, p=0.04). The 6-year cumulative incidence of secondary malignancy was not different between both groups after considering follow-up duration difference (non-D, 0.52%±0.37%; D, 0.60%±0.28%; p=0.55). The most influential risk factor for secondary malignancy was the duration of anthracycline administration according to multivariate analysis.
Conclusion
Dexrazoxane had an efficacy in lowering cardiac event-free survival rates in patients with higher cumulative anthracyclines. As a result of multivariate analysis for assessing risk factors of secondary malignancy, the occurrence of secondary malignancy was not related to dexrazoxane administration.

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Case Report
Severe Hepatic Sinusoidal Obstruction Syndrome in a Child Receiving Vincristine, Actinomycin-D, and Cyclophosphamide for Rhabdomyosarcoma: Successful Treatment with Defibrotide
Aery Choi, Young Kyung Kang, Sewon Lim, Dong Ho Kim, Jung Sub Lim, Jun Ah Lee
Cancer Res Treat. 2016;48(4):1443-1447.   Published online March 30, 2016
DOI: https://doi.org/10.4143/crt.2016.096
AbstractAbstract PDFPubReaderePub
Hepatic sinusoidal obstruction syndrome (SOS) is a life-threatening syndrome that generally occurs as a complication after hematopoietic stem cell transplantation or, less commonly, after conventional chemotherapy. Regarding SOS in rhabdomyosarcoma patients who received conventional chemotherapy, the doses of chemotherapeutic agents are associated with the development of SOS. Several cases of SOS in rhabdomyosarcoma patients after receiving chemotherapy with escalated doses of cyclophosphamide have been reported. Here, we report on a 9-year-old female with rhabdomyosarcoma who developed severe SOS after receiving chemotherapy consisting of vincristine, actinomycin-D, and a moderate dose of cyclophosphamide. She was treated successfully with defibrotide without sequelae to the liver.

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Original Articles
Epidermal Growth Factor Receptor: Is It a Feasible Target for the Treatment of Osteosarcoma?
Jun Ah Lee, Yunmi Ko, Dong Ho Kim, Jung Sub Lim, Chang-Bae Kong, Wan Hyeong Cho, Dae-Geun Jeon, Soo-Yong Lee, Jae-Soo Koh
Cancer Res Treat. 2012;44(3):202-209.   Published online September 30, 2012
DOI: https://doi.org/10.4143/crt.2012.44.3.202
AbstractAbstract PDFPubReaderePub
PURPOSE
Features of epidermal growth factor receptor (EGFR) expression in osteosarcoma and in vitro efficacies of EGFR inhibitors against osteosarcoma cells were evaluated.
MATERIALS AND METHODS
Thirty biopsy samples of osteosarcoma patients were retrospectively analyzed for EGFR protein expression by immunohistochemistry. Relationships between EGFR expression and clinicopathologic characteristics and treatment outcomes were evaluated. Four osteosarcoma cell lines were analyzed for EGFR and p-EGFR expression by western blotting. Efficacies of gefitinib and BIBW2992 on osteosarcoma cells were evaluated using a 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide assay. Tyrosine kinase domains in exons 18 to 21 were sequenced and gene expression analyses of EGFR and PTEN were performed in four osteosarcoma cell lines.
RESULTS
EGFR protein was expressed in 27 (90%) samples (6 low, 12 intermediate, 9 high) and in three cell lines. Intermediate or high staining for EGFR was related to a tumor volume<150 mL (p<0.001) and histologic subtype other than osteoblastic type (p=0.03). However, EGFR expression was not associated with histologic response to preoperative chemotherapy or survival. Gefitinib and BIBW 2992 did not have any significant inhibitory effect on cell viabilities. DNA sequencing analysis revealed three osteosarcoma cell lines have single base changes at codon 2361 of exon 20 (G to A), without affecting translation results. Furthermore, no mutation was found to be associated with constitutive EGFR activation.
CONCLUSION
In the present study, gefitinib and BIBW2992 were not effective against osteosarcoma cells. However, as osteosarcoma cells express EGFR, further studies are necessary to explore the potential of other therapeutic agents targeting EGFR.

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Assessment of Chemotherapy Response Using FDG-PET in Pediatric Bone Tumors: A Single Institution Experience
Dong Hwan Kim, Seung Yeon Kim, Hyeon Jeong Lee, Bong Sup Song, Dong Ho Kim, Joong Bum Cho, Jung Sub Lim, Jun Ah Lee
Cancer Res Treat. 2011;43(3):170-175.   Published online September 30, 2011
DOI: https://doi.org/10.4143/crt.2011.43.3.170
AbstractAbstract PDFPubReaderePub
PURPOSE
Response to neo-adjuvant chemotherapy is an important prognostic factor for osteosarcoma (OS) and the Ewing sarcoma family of tumors (ESFT). [F-18]-fluorodeoxy-D-glucose (FDG)-positron emission tomography (PET) is a non-invasive imaging modality that predicts histologic response to chemotherapy of various malignancies; however, limited data exist about the usefulness of FDG-PET in predicting the histologic response of pediatric bone tumors to chemotherapy. We analyzed the FDG-PET imaging characteristics of pediatric bone tumors and determined the association with response to chemotherapy.
MATERIALS AND METHODS
Pediatric patients with OS (n=19) or ESFT (n=17) were evaluated for FDG-PET standard uptake values before (SUV1) and after (SUV2) chemotherapy. The relationship to the chemotherapy response was assessed by histopathology in surgically-excised tumors. A complete data set (SUV1, SUV2, and histologic response) was available in 23 patients.
RESULTS
While the mean SUV1s were not different between patients with OSs and ESFTs (9.44 vs. 6.07, p=0.24), the SUV2s were greater in the patients with OSs than ESFTs (4.55 vs. 1.66, p=0.01). The ratios of SUV2-to-SUV1 (SUV2 : SUV1) were 0.65 and 0.35 for OS and ESFT, respectively (p=0.08). All of the patients with ESFTs and 47% of the patients with OS had a favorable histologic response to chemotherapy. The SUV2 : 1 [(SUV1-SUV2)/SUV1]> or =0.5 and SUV2< or =2.5 were related to favorable histologic responses to chemotherapy; the sensitivity and specificity of SUV2 : 1 at 0.5 and SUV2 at 2.5 were 93% and 88%, and 88% and 78%, respectively.
CONCLUSION
FDG-PET can be used as a non-invasive surrogate to predict response to chemotherapy in children with bone tumors.

Citations

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  • 18F-FDG PET/CT in the Management of Osteosarcoma
    Chiwoo Oh, Michael W. Bishop, Steve Y. Cho, Hyung-Jun Im, Barry L. Shulkin
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Case Report
Two Pediatric Osteosarcoma Cases with Delayed Methotrexate Excretion: Its Clinical Course and Management
Kang Min Lee, Hee Woo Lee, Seung Yeon Kim, Hyeon Jeong Lee, Dong Hwan Kim, Joongbum Cho, Dong Ho Kim, Jung Sub Lim, Jin Kyung Lee, Jun Ah Lee
Cancer Res Treat. 2011;43(1):67-70.   Published online March 31, 2011
DOI: https://doi.org/10.4143/crt.2011.43.1.67
AbstractAbstract PDFPubReaderePub
High-dose methotrexate (MTX) chemotherapy extends the duration of hospitalization and introduces the risks of serious complications related to delayed MTX excretion. The treatment of delayed MTX excretion is largely dependent on invasive measures such as hemodialysis because the clinical data regarding the efficacy or safety of carboxypetidase G2 is limited. We report here on the cases of two pediatric osteosarcoma patients with delayed MTX excretion and who were successfully managed using supportive measures. Potential life-threatening complications were prevented by administering high doses of leucovorin.

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  • The Role of Single Nucleotide Polymorphisms in Transporter Proteins and the Folate Metabolism Pathway in Delayed Methotrexate Excretion: A Case Report and Literature Review
    Jun Wang, Yue-Tao Zhao, Meng-Jiao Sun, Feng Chen, Hong-Li Guo
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Original Articles
The Survival of Osteosarcoma Patients 10 Years Old or Younger Is Not Worse than the Survival of Older Patients: A Retrospective Analysis
Jun Ah Lee, Dong Ho Kim, Jung Sub Lim, Kyung Duk Park, Won Seok Song, Soo-Yong Lee, Dae-Geun Jeon
Cancer Res Treat. 2007;39(4):160-164.   Published online December 31, 2007
DOI: https://doi.org/10.4143/crt.2007.39.4.160
AbstractAbstract PDFPubReaderePub
Purpose

This study aimed to assess whether a young age at the time of diagnosis with osteosarcoma has value to predict the prognosis.

Materials and Methods

Sixty-seven children with stage II osteosarcoma were stratified according to the age of 10. There were 32 preadolescents (≤10 years) and 35 adolescents (10<age≤15 years). The patients were analyzed for their clinical characteristics, the histologic response to preoperative chemotherapy, event-free survival (EFS) and the patterns of relapse.

Results

After a median follow-up of 54 months (range: 6~153 months), the 5-year EFS of the preadolescent and adolescent groups was 64.5±9.3% and 58.2±9.1%, respectively, and age did not have any statistical significance for survival (p=0.55). Cox regression analysis revealed that both the serum level of alkaline phosphatase and the histologic response to preoperative chemotherapy were significantly related to survival of the 67 patients. Those patients aged less than 7 years responded poorly to preoperative chemotherapy and their rate of amputation was 43%. However, their 5-year EFS was not statistically different from the older patients (57.1±18.7 vs 67.7±6.3%, respectively, p=0.58).

Conclusions

We could not find any statistical difference in the clinical characteristics and survival from osteosarcoma for the preadolescents and adolescents, so the current approach of having the same protocol for both groups of patients seems to be reasonable.

Citations

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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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