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中枢神经系统孤立性纤维瘤/血管外皮细胞瘤的诊断与治疗。17例患者的回顾性报告及文献复习

Diagnosis and treatment of solitary fibrous tumor/hemangiopericytoma of central nervous system. Retrospective report of 17 patients and literature review.

作者信息

Ma Long, Wang Lu, Fang Xiaoxuan, Zhao Cong-Hai, Sun Libo

机构信息

Department of Neurosurgery, China-Japan Union Hospital, JiLin University, China.

出版信息

Neuro Endocrinol Lett. 2018 May;39(2):88-94.

Abstract

To investigate the diagnosis, treatment and prognosis of solitary fibrous tumor (SFT)/ hemangiopericytoma (HPC) of central nervous system (CNS), we retrospectively reviewed records of 17 patients who were treated for CNS SFT/HPC at the Department of Neurosurgery, China-Japan Union Hospital of Jilin University from December 2010 to June 2016, and reevaluated their pathological diagnoses according to the 2016 WHO classification of CNS tumors. We then analyzed their clinical symptoms, imaging characteristics, treatments and outcomes. Clinical manifestations of CNS SFT/HPC were diverse, but mainly included headache, increased intracranial pressure, seizures, and focal neurological deficits. In MRI, CNS SFT/HPC usually shows heterogeneous signals, and unusual enhancements; we saw lobulated shapes in 13 patients and necrotic or cystic changes in 12 patients. Tumors of all 17 patients were resected surgically; 9 patients also received postoperative adjuvant radiotherapy. Mean follow-up time was 21 months (range: 2-67 months). The 17 surgeries included 11 total resections, 4 subtotal resection, and 2 partial resections. We followed up 12 patients; 9 of the patients who received total resections had no disease progression; among the 6 patients who did not receive total resections, 2 died of tumor recurrence, 1 has not shown any disease progression. Thus, extent of resection has an apparently crucial influence on prognosis. Postoperative radiotherapy should be chosen carefully, based on resection extent and pathologic grade.

摘要

为探讨中枢神经系统(CNS)孤立性纤维瘤(SFT)/血管外皮细胞瘤(HPC)的诊断、治疗及预后,我们回顾性分析了2010年12月至2016年6月在吉林大学中日联谊医院神经外科接受治疗的17例CNS SFT/HPC患者的病历,并根据2016年世界卫生组织(WHO)中枢神经系统肿瘤分类重新评估了他们的病理诊断。然后分析了他们的临床症状、影像学特征、治疗方法及治疗结果。CNS SFT/HPC的临床表现多样,但主要包括头痛、颅内压升高、癫痫发作及局灶性神经功能缺损。在磁共振成像(MRI)中,CNS SFT/HPC通常表现为信号不均匀及异常强化;我们发现13例患者肿瘤呈分叶状,12例患者有坏死或囊变。17例患者的肿瘤均行手术切除;9例患者术后还接受了辅助放疗。平均随访时间为21个月(范围:2 - 67个月)。17例手术包括11例全切除、4例次全切除及2例部分切除。我们对12例患者进行了随访;11例接受全切除的患者中9例无疾病进展;在未接受全切除的6例患者中,2例死于肿瘤复发,1例未出现任何疾病进展。因此,切除范围对预后有明显的关键影响。应根据切除范围和病理分级谨慎选择术后放疗。

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