대한소아청소년과학회

(구연):

Release Date : 2009. 10. 24(토)

Young Joo Kwon¹, Hyeon Jin Park¹, Byung-Kiu Park¹, Jun Ah Lee², Kyung Duk Park², Jun Eun Park³, Hyun Joo Jung³, Nak Gyun Chung⁴, Dae Chul Jeong⁴, Bin Cho⁴, Hack Ki Kim⁴, Dong Kyun Han⁵, Hee Jo Baek⁵, Hoon Kook⁵, Tai Ju Hwang⁵, Eun Sil Park⁶, Jae Young Lim⁶, Soon Ki Kim⁷, Joon Sup Song⁸, Yeon-Jung Lim⁹, Young-Ho Lee⁹, Kwang Chul Lee¹⁰, Jae Sun Park¹¹, Kun Soo Lee¹², Hyung Jin Kang¹³, Hee Young Shin¹³, Hyo Seop Ahn¹³, Hyong Soo Choi¹⁴, Soo Hyun Lee¹⁵, Keon Hee Yoo¹⁵, Ki Woong Sung¹⁵, Hong Hoe Koo¹⁵, Ho Joon Im¹⁶, Jong Jin Seo¹⁶, Sang Kyu Park¹⁷, Sung Chul Won¹⁸, Chuhl Joo Lyu¹⁸ on behalf of the KSPHO

Pediatric Oncology Center, National Cancer Center, Goyang¹, Department of Pediatrics, Korea Cancer Center Hospital, Seoul², Department of Pediatrics, Ajou University School of Medicine, Suwon³, Department of Pediatrics, Seoul St. Mary’s Hospital, The Catholic University of Korea, Seoul⁴, Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju⁵, Department of Pediatrics, Gyeongsang National University School of Medicine, Jinju⁶, Department of Pediatrics, Inha University Hospital, Incheon⁷, Department of Pediatrics, Kangdong Sacred Heart Hospital, University of Hallym Medical Center,Seoul⁸, Department of Pediatrics, Hanyang University Medical Center, Hanyang University College of Medicine, Seoul⁹, Department of Pediatrics, Korea University Anam Hospital, Korea University College of Medicine, Seoul¹⁰, Department of Pediatrics, Kosin University Gospel Hospital, Pusan¹¹, Department of Pediatrics, Kyungpook National University School of Medicine, Daegu¹², Department of Pediatrics, Seoul National University Hospital, Seoul National University College of Medicine, Seoul¹³, Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam¹⁴, Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul¹⁵, Department of Pediatrics, Asan Medical Center, University of Ulsan, Seoul¹⁶, Department of Pediatrics, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan ¹⁷, Department of Pediatrics, Yonsei University, College of Medicine, Seoul¹⁸

Young Joo Kwon¹, Hyeon Jin Park¹, Byung-Kiu Park¹, Jun Ah Lee², Kyung Duk Park², Jun Eun Park³, Hyun Joo Jung³, Nak Gyun Chung⁴, Dae Chul Jeong⁴, Bin Cho⁴, Hack Ki Kim⁴, Dong Kyun Han⁵, Hee Jo Baek⁵, Hoon Kook⁵, Tai Ju Hwang⁵, Eun Sil Park⁶, Jae Young Lim⁶, Soon Ki Kim⁷, Joon Sup Song⁸, Yeon-Jung Lim⁹, Young-Ho Lee⁹, Kwang Chul Lee¹⁰, Jae Sun Park¹¹, Kun Soo Lee¹², Hyung Jin Kang¹³, Hee Young Shin¹³, Hyo Seop Ahn¹³, Hyong Soo Choi¹⁴, Soo Hyun Lee¹⁵, Keon Hee Yoo¹⁵, Ki Woong Sung¹⁵, Hong Hoe Koo¹⁵, Ho Joon Im¹⁶, Jong Jin Seo¹⁶, Sang Kyu Park¹⁷, Sung Chul Won¹⁸, Chuhl Joo Lyu¹⁸ on behalf of the KSPHO

Pediatric Oncology Center, National Cancer Center, Goyang¹, Department of Pediatrics, Korea Cancer Center Hospital, Seoul², Department of Pediatrics, Ajou University School of Medicine, Suwon³, Department of Pediatrics, Seoul St. Mary’s Hospital, The Catholic University of Korea, Seoul⁴, Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju⁵, Department of Pediatrics, Gyeongsang National University School of Medicine, Jinju⁶, Department of Pediatrics, Inha University Hospital, Incheon⁷, Department of Pediatrics, Kangdong Sacred Heart Hospital, University of Hallym Medical Center,Seoul⁸, Department of Pediatrics, Hanyang University Medical Center, Hanyang University College of Medicine, Seoul⁹, Department of Pediatrics, Korea University Anam Hospital, Korea University College of Medicine, Seoul¹⁰, Department of Pediatrics, Kosin University Gospel Hospital, Pusan¹¹, Department of Pediatrics, Kyungpook National University School of Medicine, Daegu¹², Department of Pediatrics, Seoul National University Hospital, Seoul National University College of Medicine, Seoul¹³, Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam¹⁴, Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul¹⁵, Department of Pediatrics, Asan Medical Center, University of Ulsan, Seoul¹⁶, Department of Pediatrics, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan ¹⁷, Department of Pediatrics, Yonsei University, College of Medicine, Seoul¹⁸

Abstract

BACKGROUND Nationwide studies on pediatric Ewing sarcoma family of tumor (ESFT), including Ewing’s sarcoma (ES) and primitive neuroectodermal tumor ( PNET), have not been performed in Korea as yet. We thus made investigation into clinical characteristics, practice patterns of individual institution, and outcome of ESFT throughout the nation. PATIENTS & METHODS A retrospective analysis of medical records regarding demography, pathologic types, clinical stages, treatment modalities, chemotherapeutic regimens utilized, and outcome was made. On the whole, 133 pathologically confirmed ESFT patients were recruited from 18 institutions from 1989 to 2008. RESULTS Mean age of 133 patients was 9.3 (range 0.1-31.6) years. Male to female ratio was 1.3:1. Among 133 patients ES patients comprised 67.7% and PNET 32.3%. Tumors of bones, soft tissues, and bones and soft tissues together occupied 22.1%, 48.9%, and 29.0% of total, respectively. Of 109 patients whose IRS clinical groups were documented, group III patients (52.3%) were most common followed by IV (22.9%), I (19.3%), and II (5.5%). One-hundred twenty-five patients had primary diseases and 7 recurrent ones. Sixty-two patients (46.6%) had remote metastasis at diagnosis. Of 83 metastatic sites, bone (53.0%) was most frequently involved followed by lung (22.9%), bone marrow (10.8%), pleura (7.2%), and others (3.6%). Of 131 patients who were given treatment, 100 (76.3%) underwent surgery, 61 (46.6%) chemotherapy, and 61 (46.6%) radiotherapy, and 27 (20.8%) high dose chemotherapy with stem cell transplantation. Chemotherapeutic regimens utilized in 124 patients were CCG7942 (23.4%), CCG7881B (22.6%), CCG321P2 (16.1%), Euro-Ewing99 (12.9%), ICE (3.2%), and others (21.8%). Of 93 patients evaluable for response, 60 patients (64.5%) had been in CR and 5 (5.4%) in SD, 28 (30.1%) at last follow-up. Five-year OS and EFS were 65.2% and 42.3%, respectively with a median follow-up of 22.7 (range 0.1-191.4) months. Of 67 events, 38 relapses, 28 PD, 1 second malignancy were reported. CONCLUSIONS Proportion of remote metastasis at diagnosis was rather high in our study population. Otherwise, clinical characteristics in our patients were not quite different from those described in the literatures. Our data could be used as a basis in developing nationwide protocols and novel researches in ESFT biology.

Keywords: Ewing sarcoma Family of Tumors, Nationwide Survey,

Ewing Sarcoma Family of Tumors in Children and Young Adults: Nationwide Survey of 133 Patients under the Auspices of the Korean Society of Pediatric Hematology-Oncology (KSPHO)

Ewing Sarcoma Family of Tumors in Children and Young Adults: Nationwide Survey of 133 Patients under the Auspices of the Korean Society of Pediatric Hematology-Oncology (KSPHO)

Abstract