目的:会阴或肛门横纹肌肉瘤(RMS)是一种罕见的儿童期肉瘤,预后不良。本研究回顾了横纹肌肉瘤研究组(IRSG)I至IV的研究,以确定患者预后的决定因素并完善手术治疗指南。
方法:从1972年到1997年,对71例符合条件的患者进行了治疗和研究。患者年龄中位数为6岁。大多数(64%)在初次就诊时处于晚期(临床III和IV组),50%的区域淋巴结(LN)受累。
结果:所有患者的5年无失败生存率(FFS)为45%,总生存率(OS)为49%。与生存率显着提高相关的特征是原发肿瘤大小小于5cm,较低(较先进)的临床组和阶段,阴性区域淋巴结状态,年龄小于10岁。当疾病的程度在多变量分析中得到控制时,只有不到10岁的人预测结果会有所改善。10岁以下患者的5年总生存率为71%,而老年患者为20%(P<0.001)。组织学(肺泡与胚胎)和原发部位(会阴与肛门)没有显着影响结果。
结论:由于这些患者区域性LN受累的发生率很高,建议对所有会阴或肛门RMS患者的髂腹股沟淋巴结进行常规手术评估.
OBJECTIVE: Rhabdomyosarcoma (RMS) of the perineum or anus is a rare sarcoma of childhood with a poor prognosis. This study reviews the Intergroup Rhabdomyosarcoma Study Group (IRSG) studies I through IV to identify determinants of patient outcome and to refine surgical treatment
guidelines.
METHODS: From 1972 through 1997, 71 eligible patients were treated and studied. The median patient age was 6 years. The majority (64%) were at an advanced stage (clinical group III and IV) at initial presentation and 50% had positive regional lymph node (LN) involvement.
RESULTS: The 5-year failure-free survival rate (FFS) for all patients was 45% and the overall survival rate (OS) was 49%. Characteristics that were associated with significantly improved survival rate were primary tumor size less than 5 cm, lower (less advanced) clinical group and stage, negative regional lymph node status, and age less than 10 years. When the extent of disease was controlled for in multivariate analysis, only age less than 10 predicted an improved outcome. The 5-year overall survival rate for patients less than 10 years of age was 71% versus 20% in older patients (P <.001). Histology (alveolar versus embryonal) and primary site (perineal versus anal) did not significantly affect outcome.
CONCLUSIONS: Because of the high incidence of regional LN involvement in these patients, a strategy of routine surgical evaluation of ilioinguinal lymph nodes in all patients with perineal or anal RMS is recommended.