原发中枢神经系统淋巴瘤17例临床分析

Primary Central Nervous System Lymphoma: A Report of 17 Cases

目的:原发性中枢神经系统淋巴瘤(PCNSL)是一种较少见的中枢神经系统恶性肿瘤。近年发病率有逐年增高的趋势,本文探讨其临床特点、诊治方案及临床疗效。方法:总结1999年9月～2007年12月收治的17例患者(男性8例,女性9例,年龄19～80岁),均经病理证实为B细胞来源非霍奇金淋巴瘤。全部患者接受放疗,其中15例放疗后接受化疗等综合治疗。结合文献对原发性中枢神经系统淋巴瘤患者的临床特点、病理学检查、影像学表现、治疗及预后进行回顾性分析。结果:本病疲中老年人多见,发病急,病程短,病情进展快。临床表现复杂,颅内高压为主要临床表现之一。头颅CT以及MRI检查显示中枢侵及额叶多见,CT扫描多表现为较高密度肿块,MRI显示T1加权像多呈低信号,T2加权像多呈高信号。CT和MRI增强扫描病灶多呈均匀明显强化,可单发或多发,极少发生出血、钙化或囊变。PCNSL主要起源于B细胞,包括手术、放疗、化疗和免疫治疗的综合治疗效果较好。17例患者随访7～65个月,中位时间17个月,平均生存23.9个月。1、3年生存率分别为76.5%和23.5%,5年生存率仅有5.9%。结论:PCNSL是一组异质性肿瘤,侵袭性强,预后差,伴脊髓侵犯者则预后更差。其临床、影像学表现复杂多变,无特异性,术前诊断困难。确诊主要依靠病理检查,三维立体定向穿刺活检(定向活检)可显著提高诊断效率。其病程短,疗效不理想,最佳治疗方案是手术加放疗、化疗的联合治疗。虽然对放射及化学药物治疗敏感,但缺乏共识方案,较全身及其他部位淋巴瘤预后差。中等剂量放疗加足疗程化疗是有效的治疗方法,增加放射剂量未能改善肿瘤控制。综合治疗是提高本病疗效的关键。

Objective： Primary central nervous system lymphoma （PCNSL） is a rare and highly malignant tumor in the central nervous system. The incidence of PCNSL is increasing. The purpose of this study was to analyze the clinical features, diagnosis and treatment of PCNSL. Methods： A total of 17 patients （8 males and 9 females, aged 19-80 years） with MRI and pathologically confirmed PCNSL were admitted between September 1999 and December 2007. The Tumor showed B lymphocyte origin. All of the patients received radiotherapy and 15 patients received chemotherapy after radiotherapy. In combination with literatures, the clinical characteristics, features of images, pathology and therapeutic efficacy of primary CNS lymphoma were retrospectively analyzed. Results： These patients were of middle or old age, with acute disease onset and a rapid disease process. Increased intracranial pressure was the main clinical manifestation. CT and MRI showed solitary or multiple intracranial masses in the frontal lobe. CT scanning showed that the density of the tumor was higher than that of the brain. MRI indicated that the tumor was of hypo-signal in T1WI and hyper-signal in T2WI. Enhanced scanning showed that the lesion wass contrasted evenly and obviously with rare hemorrhage, calcification and cystic degeneration. PCNSL was mostly of B-cell origin. The outcome of comprehensive treatment was favorable. Seventeen patients were alive with a median follow-up of 17.0 months （ranged from 7 to 65 months）. The 1-, 3-, and 5-year survival rates were 76.5%, 23.5%, and 5.9%, respectively. Conclusion： PCNSL is a group of tumor with heterogenicity, strong invasive ability and poor prognosis. Patients accompanied with spinal invasion have poorer prognosis. The imaging features are variable, with no specificity. The diagnosis is determined by pathological examination. Three-dimensional stereotatic biopsy can improve diagnostic efficiency. The patient survival is short. The best therapy includes surgery, radiotherapy and chemo-therapy. Although the introduction of systemic chemotherapy and radiotherapy has consistently improved patient survival, the prognosis of PCNSL is poor. Radiotherapy of moderate dose plus enough chemotherapy is effective. Radiotherapy dose escalation fails to provide better outcome. Combined treatment is valuable for promoting the curative efficacy.