原发性中枢神经系统淋巴瘤23例临床分析并文献复习

Primary central nervous system lymphoma- -a clinical analysis of 23 cases and literature review

目的探讨原发性中枢神经系统淋巴瘤（PCNSL）的临床特征、诊断、治疗及预后。方法回顾性分析我院2014年11-2016年11月手术治疗确诊的23例PCNSL患者的临床及随访资料。COX回归用于预后风险因素分析；Kaplan-Meier法进行亚组内生存分析。结果23例患者中，平均年龄57．1岁；颅高压症状15例（65．2％），意识改变7例（30．4％），1例（14．3％）以局灶性损害为主要表现。单发12例（52．2％），多发11例（47．8％）；累及部位以额叶、颞叶、脑室系统较多（58．5％）。23例均行手术治疗，术后5例未特殊治疗，单纯放疗2例，放化疗联合3例，仅化疗13例；化疗者中9例为HD-MTX为主的方案，7例为类CHOP样方案。病理主要是弥漫性大B细胞淋巴瘤（DLBCL）（95．7％）。截至随访终点，全组中位生存期为16．9个月。半年、1年无进展生存率分别为73．9％、60．9％，2年生存率为26．1％。COX回归提示年龄和治疗方式是对预后有影响的临床因素。其中治疗方式的相对危险度（RR）值为0．164（95．0％c，0．056-0．484）；Kaplan-Meier分析发现，化疗较单纯放疗、放化疗联合有优势；基于HD-MTX的化疗方案更优（P=0．02）。结论PCNSL临床表现复杂，确诊困难，预后不佳。年龄和治疗方案是影响PCNSL生存结局的主要临床因素。活检与手术有助于确诊，适当的情况仍可考虑手术治疗。病理确诊后基于HD-MTX的联合化疗方案为最优方案。

Objective To investigate the characteristics, diagnosis, treatment and prognosis of primary central nervous system lymphoma （PCNSL）. Methods A total of 23 patients with PCNSL treated in our hospital from November 2014 to November 2016 were enrolled into this retrospective study. Cox regression model was fit to analyze predictors and multiclinical factors. Kaplan - Meier analysis was conducted in different subgroup. Results Among the cases series, the median age was 57.1 years, 15 （65.2%） suffered intracranial hypertension, 7 （ 30.4% ） had disturbance of consciousness, another case（4.3% ） mainly presented on admission with a focal neurological deficit. 12 （52.2%） had single tumor, 11 （47. 8%）were multiple. Most of lesions were located in the frontal lobe, temporal lobe and ventricle （58.5%）, the rest were parietal lobe, parallel callus, basal ganglia and cerebellum. The entire cohort had craniotomy operation, after that, 5 patients accepted support therapy, 2 patients received simple radiotherapy, and 13 patients un- derwent simple chemotherapy. High - dose methotrexate （ HD - MTX） - based chemotherapy were conducted in 9 patients, and 7 cases received CHOP - like regimens. The great majority of cases were diffuse large B cell lymphoma （ DL- BCL）. During follow-up, the median overall survival time was 16.9 months, the halfyear, one year progression - free survival rate were 73.9% and 60.9% ,two year survival rate were 26.1%. Cox regression showed that age and therapy plan were the potential influential prognostic factors. The RR value of treatment was 0. 164 （95.0% CI：0. 056 - 0. 484）. Survival analysis by Kaplan - Meier revealed that chemotherapy based on HD - MTX was superior to simply ra- diotherapy and combined treatment （P = 0.02）. Conclusions The clinical manifestation of PCNSL is complicated, diffi- cult for definitive diagnosis, facing a poor prognosis. Age and therapy plan are the key prognostic factors. Stereotactic bi-opsy and surgery will help for definitive diagnosis; surgery is still needed in proper cases. Chemotherapy based on HD - MTX was superior after pathological diagnosis.