15例腺泡状软组织肉瘤的临床特点及预后分析  

Clinical features and prognostic factors of 15 patients with alveolar soft part sarcoma

作　　者：孙馨[1] 郭卫[1] 杨荣利[1] 唐顺[1] 董森[1] 

机构地区：[1]北京大学人民医院骨与软组织肿瘤治疗中心,北京100044

出　　处：《中国癌症杂志》2012年第9期658-662,共5页China Oncology

摘　　要：背景与目的:腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS)是一种罕见的恶性软组织肿瘤,生长缓慢,无典型临床症状,常伴有肺转移、脑转移或骨转移等远处转移。本研究旨在初步探讨ASPS的临床特点,评估影响其预后的因素。方法:收集2003年1月—2008年12月就诊于北京大学人民医院骨与软组织肿瘤治疗中心的15例ASPS患者的临床资料,对患者的性别(女性9例,男性6例)、年龄(≤30岁8例,>30岁7例)、肿瘤大小(直径>5 cm 11例,≤5 cm 4例)、肿瘤原发部位(软组织12例,骨骼3例)、有无远处转移(6例未见转移,9例有肺、骨或脑转移)、术后局部复发情况(2例)、术后是否接受放疗(9例接受放疗,6例未接受放疗)及术后是否接受化疗(11例接受化疗,4例未接受化疗,方案MAID:多柔比星,氮唏米胺,异环磷酰胺)等资料作回顾性分析,并进行随访。结果:患者性别、年龄对预后的影响差异无统计学意义(P>0.05),肿瘤大小、部位及有无远处转移是影响预后的重要因素(P<0.05)。15例患者随访时间为7～76个月,接受广泛或边缘切除后只有2例局部复发,但远处转移率较高。患者预后较差,其中无瘤生存6例,带瘤生存4例,死亡5例,中位生存时间为42.0个月,5年生存率为41.7%。在本研究中,患者并没有从术后放化疗中受益。结论:ASPS是高度恶性软组织肿瘤。患者预后较差,伴有远处转移的患者5年生存率远低于整体生存率。但由于本研究病例较少,还需扩大样本量进一步深入研究。Background and purpose： Alveolar soft part sarcoma （ASPS） is a rare malignant neoplasm characterized by slow growth and indolent behavior, and most of patients exhibited metastases either presentation or later. This study aimed to define clinical features and assess prognostic factors of ASPS, and to review our clinical experience. Methods： Between Jan. 2003 and Dec. 2008, there were 15 patients with ASPS who received treatments and prospectively followed up in the Department of Orthopedic Oncology of Peking University People＇s Hospital. We retrospectively analyzed the clinical factors below to investigate their value in prognosis： gender （9 females, 6 males）, age （8 patients younger than 30, another 7 older than 30）, tumor size （4 with ≤ 5 cm, 11 with 〉5 cm）, tumor primary location （12 in soft tissues, 3 in bones）, metastasis （6 without metastasis, 6 with lung involvements, 3 with bone involvements and 2 with brain involvements）, local recurrence （2 patients with local recurrence）, postoperative radiotherapy （9 patients received）, postoperative chemotherapy （11 patients received MAID： THP, DTIC, IFO）. Results： In this study, gender and age were not prognostic indicators （P〉0.05）. The independent favorable factors associated with patient survival included tumor size and primary tumor site for metastasis patients （P〈0.05）. At a follow-up from 7 months to 76 months, only 2 patients had local recurrences after excision, but the high incidence of pulmonary or bone metastases indicated poor survival. Only 6 patients achieved disease free survival, 5 patients died of metastases or other complications. The five-year overall survival rate was 41.7%, and the median survival time was 42.0 months. No benefit was demonstrated from chemotherapy or radiotherapy. Conclusion： ASPS is kind of high grade malignancy of soft tissue neoplasm. The follow-up data presented a poor prognosis, especially for the patients with metastasis.

关 键 词：腺泡状软组织肉瘤 临床特点 预后因素 

分 类 号：R738.6[医药卫生—肿瘤]