PMID- 31594063 OWN - NLM STAT- MEDLINE DCOM- 20191029 LR - 20191029 IS - 0578-1310 (Print) IS - 0578-1310 (Linking) VI - 57 IP - 10 DP - 2019 Oct 2 TI - [Clinical and prognostic analysis of single-center multidisciplinary treatment for rhabdomyosarcoma in children]. PG - 767-773 LID - 10.3760/cma.j.issn.0578-1310.2019.10.008 [doi] AB - Objective: To summarize the clinical characteristics, treatment response and prognostic factors of rhabdomyosarcoma (RMS) in children. Methods: The clinical characteristics such as age at diagnosis, primary tumor site, tumor size, pathological type, clinical stage, and risk grouping of 213 RMS patients (140 males and 73 females) treated in Hematology Oncology Center of Beijing Children's Hospital, Capital Medical University, from May 2006 to June 2018 were analyzed retrospectively. The clinical characteristics, overall survival (OS), event free survival (EFS) and prognostic factors of children treated with the Beijing Children's Hospital-Rhabdomyosarcoma (BCH-RMS) regimen were analyzed. Survival data were analyzed by Kaplan-Meier survival analysis, and single factor analysis was performed by Log-Rank test. Results: The diagnostic age of 213 cases was 48.0 months (ranged 3.0-187.5 months), of which 136 cases (63.8%) were younger than 10 years old. The head and neck region was the most common primary site of tumor (30%, 64 cases), followed by the genitourinary tract (26.8%, 57 cases). Among pathological subtypes, embryonal RMS accounted for 71.4% (152 cases), while alveolar RMS and anaplastic RMS accounted for only 26.8% (57 cases) and 1.9% (4 cases), respectively. According to the Intergroup Rhabdomyosarcoma Study Group (IRS), IRS-Ⅲ and Ⅳ accounted for 85.0% (181 cases) of all RMS patients. In all patients, 9.4% (20 cases) patients were divided in to low-risk group, 52.1% (111 cases) patients in to intermediate -risk group, 25.8% (55 cases) patients in to high-risk group, and 12.7% (27 cases) patients in to the central nervous system invasion group, respectively. All patients with RMS received chemotherapy. The cycles of chemotherapy were 13.5 (ranged 5.0-18.0) for patients without event occurrence, while 14.2 (ranged 3.0-30.0) for patients with event occurrence. Among the 213 patients, 200 patients had surgical operation, of whom 103 patients underwent surgery before chemotherapy and 97 patients at the end of chemotherapy, 21 patients had secondary surgical resection. Radiotherapy was performed in 114 patients. The follow-up time was 23.0 months (ranged 0.5-151.0 months) . There were 98 patients with relapsed or progressed disease and 67 patients with death. The median time to progression was 10 months, of which 67 (68.4%) relapse occurred within 1 year and no recurrence occurred after follow-up for more than 5 years. The 3-year EFS and 5-year EFS were (52+/-4) % and (48+/-4) %, while the 3-year OS and 5-year OS were (65+/-4) % and (64+/-4) % by survival analysis. The 5-year OS of the low-risk, intermediate-risk, the high-risk were 100%, (74+/-5) %, (48+/-8) %, and the 2-year OS of the central nervous system invasion group was (36+/-11) % (chi(2)=33.52, P<0.01). The 5-year EFS of the low-risk, intermediate-risk, the high-risk were (93+/-6) %, (51+/-5) %, (36+/-7) % and the 2-year EFS of the central nervous system invasion group was (31+/-10) % (chi(2)=24.73, P<0.01) . Survival factor analysis suggested that the OS of children was correlated with age(chi(2)=4.16, P=0.038), tumor TNM stage (chi(2)=22.02, P=0.001), IRS group (chi(2)=4.49, P<0.01) and the risk group (chi(2)=33.52, P<0.01). Conclusions: This study showed that the median age of newly diagnosed RMS patients was 4 years. The head and neck and the genitourinary tract were the most common primary origin of RMS. The OS was low in single-center RMS children. The median time to recurrence was 10 months, and recurrence was rare 3 years later. FAU - Xu, N AU - Xu N AD - Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China. FAU - Duan, C AU - Duan C AD - Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China. FAU - Jin, M AU - Jin M AD - Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China. FAU - Zhang, D W AU - Zhang DW AD - Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China. FAU - Su, Y AU - Su Y AD - Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China. FAU - Yu, T AU - Yu T AD - Department of Radiology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China. FAU - He, L J AU - He LJ AD - Department of Pathology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China. FAU - Fu, L B AU - Fu LB AD - Department of Pathology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China. FAU - Zeng, Q AU - Zeng Q AD - Department of Thoracic Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China. FAU - Wang, H M AU - Wang HM AD - Department of Surgical Oncology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China. FAU - Zhang, W P AU - Zhang WP AD - Department of Urological Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China. FAU - Ni, X AU - Ni X AD - Department of Otolaryngology Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China. FAU - Ma, X L AU - Ma XL AD - Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Beijing 100045, China. LA - chi GR - 2017ZX09304029/National Major Scientific and Technological Special Project/ GR - XTYB201803/Special Fund of the Pediatric Medical Coordinated Development Center of Beijing Hospitals Authority/ PT - Journal Article PL - China TA - Zhonghua Er Ke Za Zhi JT - Zhonghua er ke za zhi = Chinese journal of pediatrics JID - 0417427 SB - IM MH - Adolescent MH - Antineoplastic Combined Chemotherapy Protocols/*therapeutic use MH - Child MH - Child, Preschool MH - Combined Modality Therapy MH - Disease-Free Survival MH - Female MH - Head and Neck Neoplasms/*diagnosis/mortality/pathology/*therapy MH - Humans MH - Male MH - Neoplasm Recurrence, Local/therapy MH - Prognosis MH - Retrospective Studies MH - Rhabdomyosarcoma/*diagnosis/mortality/pathology/*therapy MH - Rhabdomyosarcoma, Alveolar MH - Rhabdomyosarcoma, Embryonal MH - Survival Analysis MH - Urogenital Neoplasms/*diagnosis/mortality/pathology/*therapy OTO - NOTNLM OT - Child OT - Follow-up studies OT - Prognosis OT - Rhabdomyosarcoma EDAT- 2019/10/09 06:00 MHDA- 2019/10/30 06:00 CRDT- 2019/10/10 06:00 PHST- 2019/10/10 06:00 [entrez] PHST- 2019/10/09 06:00 [pubmed] PHST- 2019/10/30 06:00 [medline] AID - 10.3760/cma.j.issn.0578-1310.2019.10.008 [doi] PST - ppublish SO - Zhonghua Er Ke Za Zhi. 2019 Oct 2;57(10):767-773. doi: 10.3760/cma.j.issn.0578-1310.2019.10.008.