腺泡状软组织肉瘤的临床特点和治疗疗效分析

Analysis of Clinical Characteristics and Curative Effects on Alveolar Soft-Part Sarcoma

目的:分析腺泡状软组织肉瘤的临床特点和治疗疗效。方法:总结1962年5月至2008年8月接受治疗的腺泡状软组织肉瘤42例,男性19例,女性23例,中位年龄27岁,原发肿瘤部位以四肢为主(83.3%),原发肿瘤中位直径为4.2 cm,5例病例(12%)就诊时存在远处转移,38例接受了原发肿瘤的扩大切除。结果:中位随诊57个月,全组5、10、15年总生存率分别为81.7%、63.6%、31.8%。随诊期间27例(64.3%)复发或转移,其中单纯局部复发1例,局部复发合并远处转移3例,远处转移23例。远处转移病例中21例出现肺转移,7例脑转移,5例骨转移。远处转移到死亡的中位时间为36个月。20例转移病例接受了化疗,6例还接受了肺内转移灶的切除,有化疗疗效评价的14例中8例进展,6例稳定结论:腺泡状软组织肉瘤发病年龄轻,四肢多见,病程发展缓慢,手术为主要治疗手段腺泡状软组织肉瘤远期预后差,容易出现肺、骨。

Objective： This work aims to analyze the clinical features and curative effects of 42 cases with alveolar soft-part sarcoma （ ASPS ）. Methods： Data of 42 patients with ASPS, who received treatment at this hospital, during the period May 1962 to Au- gust 2008, were reviewed. The age of the patients ranged from 9 years to 56 years （ median 27 ）. There were 23 females and 19 males in the cases. The major site of the primary cancer was the limbs （ 83.3 %, n = 35 ）. The median size of the primary tumor was 4.2 cm. Five patients （ 12 % ） presented metastatis. Wide local excision of the primary cancer was performed in 38 of the 42 patients. Results： After a median follow-up of 57 months （range： 18 months to 187 months）, the overall survival rate at 5, 10, and 15 years were 81.7 %, 63.6 %, and 31.8 %, respectively. Recurrence or metastatis occurred in 27 patients （ 64.3 % ） within the follow-up period. Local recur- rence occurred in only 1 of the cases, local recurrence combined with metastasis in 3, and metastatic disease alone in 23. Pulmonary me- tastasis was found in 21 patients, brain metastasis in 7, and osseous metastasis in 5. The median survival time after diagnosis of distant metastasis was 36 months （ ranging from 17 months to 82 months ）. Chemotherapy using a variety of regimens was performed in 20 of the patients who suffered distant metastasis. Six patients also underwent metastasectomy for pulmonary metastasis. Response to chemo- therapy was recorded in 14 patients, among which stable disease was seen in 6 and progressive disease in 8. Conclusion： ASPS dis- plays a relatively indolent clinical course and features a younger age range. Surgery is the mainstay of therapy. The ultimate prognosis of ASPS is dissatisfactory and is often characterized by late metastasis to the lung, bone, or brain.