腺泡状软组织肉瘤22例临床治疗分析

Diagnosis and treatment of alveolar soft tissue sarcomas:report of 22 cases

目的探讨腺泡状软组织肉瘤的临床治疗和预后。方法对１９７３～１９９６年间收治的２２例腺泡状软组织肉瘤患者的临床资料进行回顾性分析。结果２２例均接受手术治疗，其中１７例行肿瘤局部切除，５例行扩大切除。７例病理误诊为其他软组织肿瘤（占３２％）。９例术后进行了预防性辅助放疗和（或）化疗。对２１例患者随访４个月至９年。６例患者术后复发（占２８％），１３例出现肺转移（占６２％），５例在随访８个月至４年５个月时死亡（占２４％），６例患者生存５年以上。全组经ＫａｐｌａｎＭｅｉｒｅ生存率计算其３年、４年、５年生存率分别为８３％、７５％及６６％。结论腺泡状软组织肉瘤多需病理学诊断，肿瘤局部切除是复发和转移最重要的因素，术后预防性辅助治疗对局部复发和转移没有明显的控制作用，对出现肺转移的患者采取多程标准化疗方案进行治疗。

Objective To investigate the treatment and the prognosis of patients with alveolar soft tissue sarcomas. Methods The clinical data of 22 cases of alveolar soft tissue sarcomas admitted from 1973 to 1996, were analyzed retrospectively. Results Local resection of the tumor was performed on 17 patients and 5 patients underwent extensive resection.Seven cases were misdiagnosed pathologically as other sarcomas. Nine patients received adjuvant radiotherapy and (or) chemotherapy postoperatively.21 patients were followed-up from 4 months to 9 years with local recurrence in 6 (28%),and lung metastasis in 13 (62%). Five (24%) of them died during a follow-up period of 8 months to 4 years and 5 months.The 3, 4 and 5 year's survival rate was 83%, 75% and 66% respectively. Conclusions Pathology is the mainstay for the diagnosis of alveolar soft sarcoma. Local palliative resection was the major important factor inducing postoperative recurrence and metastasis. Postoperative adjuvant prophylatic radiotherapy and chemotherapy might reduce local recurrence and distant metastasis to some extent. Standard chemotherapy was effective and is recommended for patients with lung metastasis.