原发性中枢神经系统淋巴瘤七例临床分析

The Clinical Feature of Primary Central Nervous System Lymphoma: Report of Seven Cases

目的探讨原发性中枢神经系统淋巴瘤(PCNSL)的诊断、治疗和转归。方法总结近10年来我院诊治的7例PCNSL患者的临床表现、影像学、病理、治疗方案及随诊情况。结果临床主要表现为精神障碍、颅内压增高、中枢神经功能缺损症状。影像学表现为单发或多发类圆形肿块或结节影,肿瘤周围水肿相对较轻。7例患者均为非霍奇金淋巴瘤(NHL),4例免疫组织化学染色证实为弥漫大B细胞淋巴瘤(DLBCL),采用以大剂量甲氨蝶呤(HD-MTX)和大剂量地塞米松(HD-DEX)为基础的系统化疗联合鞘内(或侧脑室内)局部注射加或不加全颅脑放疗,生存期6～83个月,平均生存32个月。结论PCNSL多发生于中老年人,脑组织活检是重要的确诊手段,PCNSL多为B细胞来源的NHL,预后与年龄及行为状态相关。以HD-MTX和HD-DEX为基础的全身化疗联合鞘内注射治疗PC-NSL效果较好,远期神经毒性轻微。

Objective To study retrospectively the clinical and pathological features of primary central system lymphoma （PCNSL） to decrease mlsdiagnosis or mistreatment and to improve the prognosis of PCNSL. Methods We analyzed the clinical data, pathological and imaging findings, treatment programs and followup of 7 patients with PCNSL in our hospital since 1996. Results The main clinical symptoms were mental disorder, intracranial hypertension and dysfunction of central nervous system （CNS） ; Single or multiple quasi - round tumor or nodule was the imaging presentation, and hydrops around tumor was relatively small. All of the 7 patients were with non - Hodgkin lymphoma （NHL） , 4 of whom were verified by immunohisto- chemistry, stain to be with diffuse large B cell lymphoma （DLBCL）. The international prognostic index was less than 2 scores in presentation. Their life span ranged from 6 to 83 months with an average of 32 months after they were given systematic chemotherapy [based on high - dose methotrexate （ HD - MTX） and high - dose dexamethasone （ HD - DEX） ] combined with intrathcal injection with or without whole brain radiotherapy （WBRT）. Conclusion The incidence of PCNSL is higher among elderly patients. Brain biopsy may be the only reliable method to confirm diagnosis. Most PCNSLs are derived from B cells. Prognosis is related with age and behavior pattern. Intrathecal injection combined with systemic chemotherapy based on HD - MTX and HD - DEX has a good curative effect and a mild long - term neurotoxicity.