原发中枢神经系统组织细胞肉瘤一例并文献复习

Primary central nervous system histiocytic sarcoma： one case report and literature review

目的探讨中枢神经系统组织细胞肉瘤（HS）的临床病理特征、诊断、治疗及预后相关因素。方法报道1例原发于大脑中枢神经系统的HS患者，结合文献进行分析。结果肿瘤位于倾叶，术前影像检查（包括磁共振波谱分析）考虑胶质瘤，手术全切，术后病理组织学HE染色和免疫组织化学染色确诊为HS。患者接受了调强放疗联合替莫唑胺化疗，至随访期结束未复发。结论HS是少见的淋巴造血系统肿瘤，原发于中枢神经系统者罕见，诊断与鉴别诊断还依赖于组织形态与免疫组织化学结果。目前治疗方法为手术、放疗和化疗的综合治疗，预后多很差。术后同步放化疗可能改善患者预后。

Objective To investigate the diagnostic criteria, clinical management and prognostic factors of primary central nervous system （CNS） histiocytic sarcoma （HS）. Methods An adult patients with primary CNS HS was reported, and literature on the rare entity were reviewed. Results The patient had no previous history of disease. Magnetic resonance imaging （including magnetic resonance spectroscopy） suggested a mass in the frontal lobe with obvious vasogenic edema, which was considered glioma. Surgery was the initial treatment and the tumor was totally removed. The histological findings revealed the HS. The patient received concomitant chemoradiotherapy postoperatively and currently lived without recurrence. Conclusions Primary CNS HS is extremely rare hematopoietic malignant tumor. The pathological feature and comprehensive immunophenotype panel are the useful ways to establish the diagnosis till now. Primary CNS HS should be treated intensively with surgery, chemotherapy, and/or radiation therapy. However, prognosis is disappointed in most of patients. Concomitant chemoradiotherapy might be an alternative treatment.