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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.

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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
  • 5,892 View
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  • 2 Web of Science
  • 3 Crossref
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Role of Radiotherapy in the Multimodal Treatment of Ewing Sarcoma Family Tumors
Yunseon Choi, Do Hoon Lim, Soo Hyun Lee, Chuhl Joo Lyu, Jung Ho Im, Yun-Han Lee, Chang-Ok Suh
Cancer Res Treat. 2015;47(4):904-912.   Published online February 16, 2015
DOI: https://doi.org/10.4143/crt.2014.158
AbstractAbstract PDFPubReaderePub
Purpose
The aim of this study was to evaluate the role of radiotherapy (RT) in the management of Ewing sarcoma family tumors (ESFT). Materials and Methods Retrospective analysiswas performed on 91 patientswith localized ESFT treated from 1988 to 2012. Primary tumor size was ≥ 8 cm in 33 patients. Surgery, RT, and combined surgery with RT were applied in 37, 15, and 33 patients, respectively.
Results
Median follow-up was 43.8 months. Forty-three patients (47.3%) showed recurrence or progressive disease. Twelve patients (13.2%) showed local failure after initial treatment. Thirty-nine patients (42.9%) experienced distant metastases. The 5-year overall survival (OS), progression-free survival, and local control (LC) were 60.5%, 58.2%, and 85.1%, respectively. According to treatment, 5-year LCwas 64.8% with RT and 90.2% with combined surgery and RT (p=0.052). Prognostic factors for OS were tumor size (≥ 8 cm, p < 0.001) and surgical resection (p < 0.001). In large tumors (≥ 8 cm), combined surgery and RT produced better LC compared to RT (p=0.033). However, in smaller tumors (< 8 cm), RT without surgery resulted in a similar LC rate as RT with surgery (p=0.374). Conclusion RT used for patients with unfavorable risk factors resulted in worse outcome than for patientswho received surgery. Smallertumors could be controlled locallywith chemotherapy and RT. For large tumors, combined surgery and RT is needed. Proper selection of local treatment modality, RT, surgery, or both is crucial in the management of ESFT.

Citations

Citations to this article as recorded by  
  • Primary Ewing’s Sarcoma of Body of Mandible, Multimodal Treatment with Excellent Spontaneous Bone Regeneration: a Case Report
    Rangila Ram, Priyanka Bhardwaj, Yogesh Bhardwaj, Narotam Ghezta, Ravi Bhatt, Pravesh Dhiman
    Journal of Maxillofacial and Oral Surgery.2023; 22(3): 554.     CrossRef
  • Ewing’s sarcoma masquerading as an odontogenic infection
    Rizwan Hamid, Ambika Gaur, Sunita Gupta, Ritu Garg
    Journal of Cancer Research and Therapeutics.2023; 19(Suppl 2): S950.     CrossRef
  • 18F‐FDG PET and PET/CT as a diagnostic method for Ewing sarcoma: A systematic review and meta‐analysis
    Nimish Seth, Ishith Seth, Gabriella Bulloch, Adrian Hang Yue Siu, Allen Guo, Rukmini Chatterjee, Michael MacManus, Leo Donnan
    Pediatric Blood & Cancer.2022;[Epub]     CrossRef
  • Management of Unresectable Localized Pelvic Bone Sarcomas: Current Practice and Future Perspectives
    Joaquim Soares do Brito, Miguel Esperança-Martins, André Abrunhosa-Branquinho, Cecilia Melo-Alvim, Raquel Lopes-Brás, João Janeiro, Dolores Lopez-Presa, Isabel Fernandes, José Portela, Luis Costa
    Cancers.2022; 14(10): 2546.     CrossRef
  • Radiation Therapy Dose Escalation in Unresectable Ewing Sarcoma: Final Results of a Phase 3 Randomized Controlled Trial
    Siddhartha Laskar, Shwetabh Sinha, Abhishek Chatterjee, Nehal Khanna, Jifmi Jose Manjali, Ajay Puri, Ashish Gulia, Prakash Nayak, Tushar Vora, Girish Chinnaswamy, Maya Prasad, Jyoti Bajpai, Shashikant Juvekar, Subhash Desai, Amit Janu, Venkatesh Rangaraja
    International Journal of Radiation Oncology*Biology*Physics.2022; 113(5): 996.     CrossRef
  • Adjuvant Radiotherapy for the Multimodal Treatment of Pediatric Ewing Sarcoma
    Gonca ALTINIŞIK İNAN, İpek Pınar ARAL, Tarık KARGIOĞLU, Arzu YAZAL ERDEM, Selma ÇAKMAKCI, Hüseyin Furkan ÖZTÜRK, Suheyla AYTAÇ ARSLAN, Yılmaz TEZCAN
    Medical Records.2022; 4(3): 304.     CrossRef
  • Sacral Ewing sarcoma with rib, lung, and multifocal skull metastases: A rare case report and review of treatments
    Chen Ye, Wei Wei, Xuebin Tang, Feng Li, Baoquan Xin, Qianqian Chen, Haifeng Wei, Shaohui He, Jianru Xiao
    Frontiers in Oncology.2022;[Epub]     CrossRef
  • Pelvic Ewing sarcoma: Should all patients receive pre-operative radiotherapy, or should it be delivered selectively?
    Johnathan R. Lex, Vineet Kurisunkal, Yoichi Kaneuchi, Tomohiro Fujiwara, Jenny Sherriff, Catrin Wigley, Jonathan D. Stevenson, Michael C. Parry, Lee M. Jeys
    European Journal of Surgical Oncology.2021; 47(10): 2618.     CrossRef
  • The patterns of distant metastasis and prognostic factors in patients with primary metastatic Ewing sarcoma of the bone
    Lei Zhang, Lu Xiong, Li-Mei Wu, Wen-Hui Shen, Ping Zhou, Chen-Lu Lian, Wen-Tong Zhang, San-Gang Wu
    Journal of Bone Oncology.2021; 30: 100385.     CrossRef
  • The Outcome of Children With Malignant Bone Tumors: A Single-Center Experience
    Mohammadreza Bordbar, Ali Sarfaraz, Sezaneh Haghpanah, Omidreza Zekavat, Soheila Zareifar, Tahereh Zarei
    Global Pediatric Health.2021;[Epub]     CrossRef
  • Ewing's sarcoma of the hip: A case report with no evidence of tumor recurrence and literature review
    Payam Mohammadhoseini, Samira Razzaghi, Mahdi Barazesh, Sajad Jalili
    Bone Reports.2021; 15: 101131.     CrossRef
  • Reconstruction after Subtotal Sacrectomy for Sacral Ewing’s Sarcoma Using Tibial Allograft Strut Grafting: A Case Report
    Ryosuke Hirota, Makoto Emori, Yoshinori Terashima, Kousuke Iba, Noriyuki Iesato, Ryunosuke Fukushi, Mitsunori Yoshimoto, Toshihiko Yamashita
    Case Reports in Oncology.2021; 14(1): 296.     CrossRef
  • Characteristics and prognosis of pelvic Ewing sarcoma: a SEER population-based study
    Li Chen, Cheng Long, Jiaxin Liu, Fei Xing, Xin Duan
    PeerJ.2019; 7: e7710.     CrossRef
  • Anti-tumor Drug Targets Analysis: Current Insight and Future Prospect
    Sheng Wang, Dexi Zhou, Zhenyu Xu, Jing Song, Xueyi Qian, Xiongwen Lv, Jiajie Luan
    Current Drug Targets.2019; 20(11): 1180.     CrossRef
  • Risk analysis factors for local recurrence in Ewing’s sarcoma
    J. I. Albergo, C. L. L. Gaston, M. C. Parry, M. K. Laitinen, L. M. Jeys, R. M. Tillman, A. T. Abudu, R. J. Grimer
    The Bone & Joint Journal.2018; 100-B(2): 247.     CrossRef
  • In Vitro and In Vivo Characterization of a Preclinical Irradiation-Adapted Model for Ewing Sarcoma
    Mary Carroll Shapiro, Tien Tang, Atreyi Dasgupta, Lyazat Kurenbekova, Ryan Shuck, M. Waleed Gaber, Jason T. Yustein
    International Journal of Radiation Oncology*Biology*Physics.2018; 101(1): 118.     CrossRef
  • Comparison of the effects of local treatment strategies in non-metastatic Ewing sarcoma of bone
    Wen-Tong Zhang, Wen-Wen Zhang, Zhen-Yu He, Jia-Yuan Sun, Lei Zhang, Qing Xia, San-Gang Wu
    Expert Review of Anticancer Therapy.2018; 18(5): 501.     CrossRef
  • Ewing sarcoma of the head and neck: The Mayo Clinic experience
    Michael D. Olson, Kathryn M. Van Abel, Rebecca N. Wehrs, Joaquin J. Garcia, Eric J. Moore
    Head & Neck.2018; 40(9): 1999.     CrossRef
  • Hemipelvectomías tras sarcomas de localización pélvica de alto grado: pronóstico en condrosarcomas frente a otros tipos histológicos
    J. Arnal-Burró, J.A. Calvo-Haro, C. Igualada-Blazquez, P. Gil-Martínez, M. Cuervo-Dehesa, J. Vaquero-Martín
    Revista Española de Cirugía Ortopédica y Traumatología.2016; 60(1): 67.     CrossRef
  • Hemipelvectomy for the treatment of high-grade sarcomas: Pronostic of chondrosarcomas compared to other histological types
    J. Arnal-Burró, J.A. Calvo-Haro, C. Igualada-Blazquez, P. Gil-Martínez, M. Cuervo-Dehesa, J. Vaquero-Martín
    Revista Española de Cirugía Ortopédica y Traumatología (English Edition).2016; 60(1): 67.     CrossRef
  • Local therapy in non-metastatic primary Ewing sarcoma of the mandible and maxilla in children
    S.S. Qureshi, M. Bhagat, S. Laskar, S. Kembhavi, T. Vora, M. Ramadwar, G. Chinnaswamy, M. Prasad, N. Khanna, S. Shah, S. Talole
    International Journal of Oral and Maxillofacial Surgery.2016; 45(8): 938.     CrossRef
  • The Role of Radiotherapy in Local Control of Nonextremity Ewing Sarcomas
    Ozlem O. Akagunduz, Serra A. Kamer, Burcin Kececi, Bengu Demirag, Haldun Oniz, Mehmet Kantar, Nazan Cetingul, Dundar Sabah, Yavuz Anacak
    Tumori Journal.2016; 102(2): 162.     CrossRef
  • Ewing's sarcoma of bone tumor cells produces MCSF that stimulates monocyte proliferation in a novel mouse model of Ewing's sarcoma of bone
    B.S. Margulies, S.D. DeBoyace, T.A. Damron, M.J. Allen
    Bone.2015; 79: 121.     CrossRef
  • 14,306 View
  • 199 Download
  • 22 Web of Science
  • 23 Crossref
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Incidence and Survival of Pediatric Soft Tissue Sarcomas: Comparison between Adults and Children
Sun Min Lim, Cheol Joo Yoo, Jung Woo Han, Yong Jin Cho, Soo Hee Kim, Joong Bae Ahn, Sun Young Rha, Sang Joon Shin, Hyun Cheol Chung, Woo Ick Yang, Kyoo-Ho Shin, Jae Kyung Rho, Hyo Song Kim
Cancer Res Treat. 2015;47(1):9-17.   Published online August 21, 2014
DOI: https://doi.org/10.4143/crt.2013.157
AbstractAbstract PDFPubReaderePub
Purpose
Pediatric-type sarcomas such as rhabdomyosarcoma (RMS), Ewing sarcoma (EWS), primitive neuroectodermal tumor (PNET), and desmoplastic small round-cell tumor (DSRCT) are rare in adults, with limited studies on their prognosis and optimal treatment strategies. We aimed to examine the outcome of children and adult patients with RMS, EWS, PNET, and DSRCT and relevant prognostic factors. Materials and Methods We retrospectively reviewed 220 pediatric-type sarcoma patients at a single institution between 1985 and 2011. Comparisons were made in order to examine differences in demographics, disease characteristics, and survival. Survival analyses were performed using the Kaplan-Meier method with log-rank tests and Cox proportional hazards models. Results A total of 220 consecutive patients were identified at our institute. Median age was 15.6 years (range, 0 to 81 years) and there were 108 children (49%) and 112 adult patients (51%). According to histological classification, 106 patients (48.2%) had RMS, 60 (27.3%) had EWS, 50 (22.7%) had PNET, and 4 (1.8%) had DSRCT. With a median follow-up period of 6.6 years, the estimated median overall survival (OS) of all patients was 75 months (95% confidence interval [CI], 27.2 to 122.8 months) and median event-free survival (EFS) for all patients was 11 months (95% CI, 8.8 to 13.2 months). No significant difference in OS and EFS was observed between adults and children. In multivariate analysis, distant metastasis (hazard ratio [HR], 1.617; 95% CI, 1.022 to 2.557; p=0.040) and no debulking surgery (HR, 1.443; 95% CI, 1.104 to 1.812; p=0.012) showed independent association with worse OS. Conclusion Metastatic disease and no surgical treatment are poor prognostic factors for OS among pediatric-type sarcomas for both adults and children.

Citations

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  • CAR-T Therapies in Solid Tumors: Opportunities and Challenges
    Grace Guzman, Megan R. Reed, Kevin Bielamowicz, Brian Koss, Analiz Rodriguez
    Current Oncology Reports.2023; 25(5): 479.     CrossRef
  • Clinicopathological and treatment response characteristics of updated rhabdomyosarcoma histomolecular subtypes: An Asian population‐based study
    Guo Yuan How, Chik Hong Kuick, Min Hwee Yong, Shui Yen Soh, Esther XY Hee, Meng Kang Wong, Richard Quek, Mohd Farid Harunal, Sathiyamoorthy Selvarajan, Kesavan Sittampalam, Chetan Anil Dhamne, Victor Lee, Kenneth TE Chang, Amos HP Loh
    Asia-Pacific Journal of Clinical Oncology.2023;[Epub]     CrossRef
  • Pediatric sarcoma survivorship: A call for a developmental cascades approach
    Peter M. Fantozzi, Gina Sprint, Anna Marie Medina
    Development and Psychopathology.2022; 34(4): 1221.     CrossRef
  • Clinical Perspectives for 18F-FDG PET Imaging in Pediatric Oncology: Μetabolic Tumor Volume and Radiomics
    Vassiliki Lyra, Sofia Chatziioannou, Maria Kallergi
    Metabolites.2022; 12(3): 217.     CrossRef
  • Desmoplastic Small Round Cell Tumors With EWS-WT1 Transcript Expression: Should We Consider Children and Adult Patients Differently?
    Laura Olivier-Gougenheim, Daniel Orbach, Vincent Atallah, Perrine Marec-Berard, Amandine Bertrand
    Journal of Pediatric Hematology/Oncology.2022; 44(3): e637.     CrossRef
  • Next-Generation Sequencing Identifies Potential Actionable Targets in Paediatric Sarcomas
    Antonio Juan Ribelles, Pablo Gargallo, Pablo Berlanga, Vanessa Segura, Yania Yáñez, Bárbara Juan, Marta Salom, Margarita Llavador, Jaime Font de Mora, Victoria Castel, Adela Cañete
    Journal of Personalized Medicine.2021; 11(4): 268.     CrossRef
  • Ewing Sarcoma
    Hee Young Ju
    Clinical Pediatric Hematology-Oncology.2019; 26(1): 27.     CrossRef
  • Emerging trends in immunotherapy for pediatric sarcomas
    Kyle A. Dyson, Brian D. Stover, Adam Grippin, Hector R. Mendez-Gomez, Joanne Lagmay, Duane A. Mitchell, Elias J. Sayour
    Journal of Hematology & Oncology.2019;[Epub]     CrossRef
  • 24,179 View
  • 140 Download
  • 10 Web of Science
  • 8 Crossref
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