颅内原发滑膜肉瘤的诊断与治疗预后的影响

Diagnosis and treatment of primary synovial sarcoma of the skull

目的探讨颅内原发滑膜肉瘤的诊断、治疗及预后。方法回顾性分析新疆医科大学第一附属医院自2008年7月-2015年4月收治的4例颅内原发滑膜肉瘤的临床资料。结果 4例颅内原发滑膜肉瘤术前均误诊,其中2例误诊为胶质瘤,2例误诊为脑膜瘤;4例均行显微外科手术全切肿瘤,术后行放疗(剂量范围:60Gy-70Gy,疗程:25～30次);随访3月-2年,2例复发(1例术后13月复发,放弃治疗死亡;1例术后10月复发,再次手术治疗,术后随访7月,未见肿瘤生长。)余2例分别随访9月、3月,未见肿瘤复发。结论颅内原发滑膜肉瘤是一种颅内少见恶性肿瘤,术前易误诊,影像学特征可为诊断提供帮助,病理及基因检测可明确诊断,显微外科手术大范围切除肿瘤辅助术后放疗是主要的治疗手段,肿瘤易复发,预后差。

Objective To investigate the diagnosis, treatment and prognosis of primary intracranial synovial sarcoma.Methods The clinical data of 4 cases of primary intracranial synovial sarcoma were confirmed by pathology in the First Affiliated Hospital of Xinjiang Medical University from April 2008 to February 2015 were analyzed. Results 4 cases were misdiagnosed preoperatively, of which 2 were misdiagnosed as glioma, 2 cases were misdiagnosed as meningioma;4 cases underwent microsurgical total resection and postoperative radiotherapy(dose range: 60 Gy-70 Gy treatment: 25-30);allthe cases were followed up from 3 months to 2 years,2 cases got local tumor recurrence(1 got local tumor recurrence 13 months after operation and dead of abandoning treatment;1 got local tumor recurrence 10 months after operation and with no tumor recurrence 7 months after secondary operation). The other 2 cases were followed up for 9 and 3 months respectivelyand with the result of no tumor recurrence. Conclusion Intracranial primary synovial sarcoma is a rare intracranial malignant tumor. Misdiagnosis commonly occurs preoperatively. CT and MRI imaging have typical features. Diagnosis depending on pathology. Microsurgical wide excision of tumor assisted with postoperative radiotherapy is the main treatment. Intracranial primary synovial sarcoma has a high recurrence rate and poor prognosis.