中枢神经系统孤立性纤维瘤／血管周细胞瘤的诊断和治疗

Diagnosis and treatment of solitary fibrous tumor/hemangiopericytoma of central nervous system

目的探讨中枢神经系统孤立性纤维瘤／血管周细胞瘤（SFT／HPC）的诊断、治疗以及预后。方法回顾性分析2010年12月至2016年6月吉林大学中日联谊医院神经外科收治的15例中枢神经系统孤立性纤维瘤患者和血管周细胞瘤患者的临床资料，按照2016年WHO中枢神经系统肿瘤分类重新评估，总结其临床表现、影像学特征、治疗方法以及预后。中枢神经系统SFT／HPC的临床表现多样，以头痛、颅内压增高、癫痫、局灶性神经功能缺失为主要表现。MRI上病灶多表现为不均匀的混杂信号，12例呈分叶状外观，11例出现坏死、囊变以及不均匀的强化。15例均行手术切除肿瘤。8例接受术后辅助放疗。随访时间为2—67个月，平均21个月。结果15例患者中，全切除9例，近全切除4例，部分切除2例。获随访10例，其中7例全切除患者病情无进展；3例未获全切除的患者中，2例死于肿瘤复发，1例未见临床进展。结论中枢神经系统SFT／HPC的临床表现无特异性，MRI上病灶呈分叶状外观，囊变、坏死以及不均匀强化为其相对特征性的影像学表现。是否全切除肿瘤为影响SFT／HPC患者预后的重要因素。术后是否行辅助放疗应根据肿瘤病理学分级和手术切除程度谨慎选择。

Objective To investigate the diagnosis, treatment and prognosis of solitary fibrous tnmor/hemangiopericytoma of central nervous system （ CNS ）. Methods We retrospectively summarized data of 15 patients with solitary fibrous tumor/hemangiopericytoma of CNS who were recruited to the Department of Neurosurgery, China-Japan Union Hospital of Jilin University from December 2010 to June 2016. The patients＇pathological diagnoses were reevaluated according to the 2016 WHO classification of CNS tumors. Analysis was conducted regarding the patients＂ clinical symptoms, imaging characteristics, treatment and outcomes. The clinical manifestations of solitary fibrous tumor/hemangiopericytoma of CNS were diverse mainly with headaches, increased intraeranial pressure, seizures, and focal neurological deficits. The solitary fibrous tumor/hemangiopericytoma of CNS usually demonstrates heterogeneous signals and enhanced remarkably on MRI：lobulated shape observed in 12 cases and necrotic or cystic changes in ll cases. The tumors of 15 cases were resected surgically. Eight patients received postoperative adjuvant radiotherapy. The follow-up time ranged from 2 to 67 months （mean, 21 months）. Results In the 15 patients, 9 achieved total resection, 4 achieved subtotal resection, and 2 achieved partial resection. Ten patients were followed up, the illness of 7 patients with total resection did not have any progression; in 3 patients without achieving total resection, 2 died of tumor recurrence, 1 has not yet had any clinical progress. Conclusions The clinical manifestations of solitary fibrous tumor/hemangiopericytoma of CNS vary greatly. Lobulated shape, necrotic or cystic changes and heterogeneously remarkable enhancement on MRI are its relatively characteristic imaging features. The extent of resection remains the crucial factor influencing the prognosis. The postoperative radiotheraphy should be chosen cautiously based on the extent of resection and pathologic grades.