同一研究所60例儿童横纹肌肉瘤临床特征和预后表

　　Clinical Characteristics and the Prognosis of Childhood Rhabdomyosarcoma in 60 Patients Treated at a Single Institute

　　同一研究所60例儿童横纹肌肉瘤临床特征和预后表现

　　Rhabdomyosarcoma (RMS) is the most frequent soft tissue sarcoma in children. The aim of study was to retrospectively review the treatment results of childhood rhabdomyosarcoma and identify prognostic factors. 60 children with rhabdomyosarcoma treated between 1996 and 2002 in Shafa Hospital were reviewed.The data were analyzed for clinico-epidemiological factors. Age, gender, race, histology type, primary site, tumor size and intergroup rhabdomyosarcoma study (IRS) group were evaluated. The primary site of involvement was orbit in 6 cases (10%) head and neck nonparameningial in 12 cases (20%), parameningial region in 12 cases (20%). The histological findings were as follows: 12 cases (72.5%) for embryonal, 6 cases (10%) for alveolar and 11 cases (17. 5%) for botryoid type. With respect to the IRS III (15%) were group II, 32 (52.5%) were group III and 24 cases (40%) were group IV. The 5-year survival rate was 47.9%. Primary tumor site (P=0.0003), and histology (P=0.05) were associated significantly with survival after recurrence. Among the variables, age, gender, regional lymph node involvement, and IRS group did not affect 5-year survival but the type and time of recurrence (P=0.0002), and its relation with therapy (P=0.0001) were associated with survival. This study showed that overall survival for rhabdomyosarcoma is dependent on histological subtype, primary site, disease group, duration of disease before treatment. The outcome for infant with RMS is less satisfactory than older children and the patients aged 1-9 years had the best 5 year survival.

　　横纹肌肉瘤是儿童常见的软组织肿瘤。研究目的是回顾性分析儿童横纹肌肉瘤的疗效及辨别预后影响因素。回顾60位患者在1996年到2002年在shafa 医院治疗过程。数据分析的临床流行性病学因素包括:年龄，性别，种族，组织学分类，原发部位，肿瘤部位及IRS。 6例(10%)原发部位在头部，12例(20%)在颈部。组织学研究发现如下：12 例(72.5%)属 胚胎型横纹肌肉瘤，6例(10%)肺泡型横纹肌肉瘤，11例(17. 5%)葡萄状型横纹肌肉瘤。II组 IRS III期 (15%)，III 组32例 (52.5%)，IV组24例(40%)。5年的生存率是 47.9%。原发肿瘤部位(P=0.0003)和组织学结构(P=0.05)对复发后生存率影响最大。这些因素中，年龄，性别，局部淋巴结，和IRS 组没有影响反而类型和复发时间及局部化疗影响生存率。研究显示影响总生存率的是组织学类型，原发部位，疾病组，疾病持续时间。婴儿横纹肌肉瘤比更大的儿童及1-9岁的患者5年生存率低。