鞍区血管外皮细胞瘤的诊断及显微手术治疗

Clinical Diagnosis and Microsurgery Treatment of Sellar Hemangiopericytoma

目的探讨鞍区血管外皮细胞瘤（hemangiopericytomas,HPC）的临床诊断特点及显微手术的治疗效果。方法对2001年3月-2014年12月6例术后病理证实为鞍区HPC的临床表现、神经影像学、治疗和预后等进行回顾性分析。结果 4例采用额颞入路,1例额眶颧入路,1例额外侧入路。肿瘤全切除3例,近全切除1例,大部分切除2例。术中出血量600-2100 ml,平均1200 ml。6例术后病理证实为HPC,均接受普通放疗。术后并发无菌性脑膜炎2例,抗生素及腰穿置管后治愈。动眼神经麻痹2例,对侧肢体偏瘫1例,神经康复理疗后改善。6例随访6-29个月,平均14.8月,无神经系统转移及死亡。结论鞍区HPC临床少见,误诊率高。肿瘤易复发,最有效的治疗方法是全切除肿瘤。

Objective To discuss the diagnostic features and microsurgery treatment of sellar hemangiopericytoma（ HPC）.Methods From March 2001 to December 2014,six cases of pathologically confirmed sellar aera HPC were retrospectively analyzed with respect to its clinical manifestations,neuroimaging,treatment,and prognosis. Results The operation was performed via frontotemporal approach in 4 cases,orbitozygomatic approach in 1 case,and frontallateral approach in 1 case. The tumor was completely removed in 3 cases,nearly subtotally resected in 1 case,and mostly subtotally resected in 2. The blood loss was 600-2100 ml,with an average of 1200 ml. Postoperatively,all the 6 cases were pathologically confirmed as having hemangiopericytoma,and underwent general radiotherapy. There were 2 cases of aseptic meningitis,who were cured with antibiotics and lumbar puncture.Oculomotor nerve palsy occurred in 2 cases and contralateral limb paralysis in 1 case,all of which underwent neurological rehabilitation therapy until improvement. Follow-up examinations were conducted for 6-29 months,with an average of 14. 8 months. No nervous system metastasis or death was seen. Conclusion Sellar hemangiopericytoma rarely occurs in clinical practice and subjects to misdiagnosis. With high rate of tumor recurrence,the most effective treatment is total resection.