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Clinical characteristics and prognosis of childhood rhabdomyosarcoma: a ten-year retrospective multicenter study

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机构: [1]Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics, Ministry of Education, Hematology Oncology Center, Beijing Children’s Hospital, Capital Medical University, Beijing 100045, China [2]Department of Pediatrics, Beijing Tong Ren Hospital, Beijing 100730, China [3]Peking University First Hospital, Beijing 100034, China [4]Beijing Shijitan Hospital, Beijing 100038, China [5]Wuhan Children’s Hospital, Wuhan 430000, China [6]Beijing Tong Ren Hospital, China [7]Beijing Children’s Hospital, Capital Medical University, China [8]Shanghai Childrens’ Medical Center, China
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关键词: Childhood RMS multicenter China

摘要:
Purpose: Rhabdomyosarcoma (RMS) is the most frequent soft tissue sarcoma in children. We have retrospectively explored the treatment results of childhood RMS and identified prognostic factors in multicenter in China, in order to lay the foundation for further multicenter study. Methods: This retrospective study was carried out analyzing the medical records of 161 patients with the pathological diagnosis of RMS from January, 2001 to February, 2014 at 5 large cancer centers in China. The data was reviewed clinico-epidemiological factors. Age, gender, histology type, primary site, tumor size, intergroup rhabdomyosarcoma study (IRS) group and results of treatments were evaluated. Patients were followed up to Dec 31, 2014. Results: The median age of our patients was 51 months. 10.5% of our patients were infants. The genitourinary system was the most common primary site of tumor (43.5%). The proportion of primary site of head and neck except parameningeal, at 28.2% (42 cases), while the proportion of parameningeal region was 4.6% (7 cases). The histological findings were as follows: 130 cases (80.7%) with embryonal, 19 cases (11.9%) with alveolar and 5 cases (3.1%) with botryoid type. According to the classification system of the IRS group, 1 case (0.6%) was group I, 54 cases (33.5%) were group II, 46 cases (28.6%) were group III and 60 cases (37.3 %) were group IV. 149 patients were treated and followed-up regularly, Patients in Beijing children's hospital (n=95) were enrolled in IRS-II/COG-D9803, D9802 protocols. while the other patients (n=54) started on treatment according to Chinese Anti-cancer Association protocol. There were median time of 51 months for following up, 60 occurred event. The ten-year event free survival rate was 53.4+/-5.1%, overall survival was 65.3+/-6.3%. The relations between outcome and age (0.046), primary site (0.022), pathologic subtype (0.013), tumor size (0.008) and IRS group (P=0.000) were associated significantly with event free survival. Among the variables, age (P=0.028) and IRS group (P=0.000) were associated significantly with overall survival. Multivariate analysis showed that overall survival for RMS was dependent on IRS group (P=0.026). Conclusions: The epidemiological characteristics of our patients are quite similarly to the worldwide data. Except for the higher prevalence of group IV in our patients and the higher percentage of patients with primary tumor site in the genitourinary system, this study showed that overall survival for RMS is depended on disease group.

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出版当年[2014]版:
大类 | 4 区 医学
小类 | 4 区 医学:研究与实验
最新[2025]版:
大类 | 4 区 医学
小类 | 4 区 医学:研究与实验
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出版当年[2013]版:
Q3 MEDICINE, RESEARCH & EXPERIMENTAL
最新[2023]版:
Q4 MEDICINE, RESEARCH & EXPERIMENTAL

影响因子: 最新[2023版] 最新五年平均 出版当年[2013版] 出版当年五年平均 出版前一年[2012版] 出版后一年[2014版]

第一作者:
第一作者机构: [1]Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics, Ministry of Education, Hematology Oncology Center, Beijing Children’s Hospital, Capital Medical University, Beijing 100045, China [*1]Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics, Ministry of Education, Hematology Oncology Center, Beijing Children’s Hospital, Capital Medical University, Beijing 100045, China.
通讯作者:
通讯机构: [1]Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics, Ministry of Education, Hematology Oncology Center, Beijing Children’s Hospital, Capital Medical University, Beijing 100045, China [2]Department of Pediatrics, Beijing Tong Ren Hospital, Beijing 100730, China [3]Peking University First Hospital, Beijing 100034, China [4]Beijing Shijitan Hospital, Beijing 100038, China [5]Wuhan Children’s Hospital, Wuhan 430000, China [*1]Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics, Ministry of Education, Hematology Oncology Center, Beijing Children’s Hospital, Capital Medical University, Beijing 100045, China. [*2]Department of Pediatrics, Beijing Tong Ren Hospital, Beijing 100730, China. [*3]Peking University First Hospital, Beijing 100034, China. [*4]Beijing Shijitan Hospital, Beijing 100038, China. [*5]Wuhan Children’s Hospital, Wuhan 430000, China.
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