原发性中枢神经系统淋巴瘤35例临床及预后分析

目的：探讨原发中枢神经系统淋巴瘤（PCNSL）临床特点、诊治方案及临床疗效。方法：总结2001年1月-2008年1月收治的35例PCNSL患者，均经病理证实为B细胞来源非霍奇金淋巴瘤并接受放疗，其中25例放疗后接受化疗。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：本病以中老年人多见，发病急，病程短，病情进展快。临床表现复杂，颅内高压为主要表现之一。CT、MRI增强扫描病灶多呈均匀明显强化，可单发或多发。35例患者中位生存时间23月，1年生存率74．3％，3年生存率25．7％，5年生存率5．71％。肿瘤全切及局部切除者，生存率未见明显统计学差异（P=0．053），加化疗疗效优于不加化疗（P=0．012）。结论：PCNSL临床表现多样，影像学缺乏特异性，极易误诊，确诊需要依靠病理学检查，最佳治疗方案是手术加放疗、化疗的联合治疗。PCNSL侵袭性强，生存期短，其预后主要与发病年龄、多灶性、一般状态有关。

Clinical and prognostic factors of 35 patients with primary central nervous system lymphoma

Objective：To analyze the clinical features, diagnosis and treatment of primary central nervous system lymphoma （PCNSL） . Methods： A total of 35 patients were confirmed as non - Hodgkin, s lymphoma showed B lymphocyte origin with pathology between Jannuary 2001 and Jannuany 2008. All of the patients received radiotherapy and 25 patients received chemotherapy after radiotherapy. In combination with literatures,the clinical characteristics, features of images, pathology and therapeutic efficacy were retrospectively analyzed. Results： These patients were of middle or old age, with acute onset and a rapid process. Increased intracranial pressure was the main clinical manifestation. CT and MRI enhanced scanning showed that the lesion mass contrasted evenly and obviously. The patients had single - locus lesion or multi - locus lesion. 35 patients were alive with a median follow - up of 23.0 months. The 1 - ,3 - ,5 - year survival rates were 74.3%, 25.7% and 5.7% respectively. There were no statistical difference between the Group received total resection and the＇ Group received subtotal resection. （ P = 0. 053 ）. Statistical difference existed between the Group received both surgery and radiotherapy and the Group surgery combined with radiotherapy and chemotherapy, （P = 0. 012）. Conclusion： PCNSL has diversify clinical manifestations, but no specific imaging performance, So it is easy misdiagnosed, pathological examination is the only reliable method to confirm PC- NSL. The best therapy includes surgery, radiotherapy and chemotherapy. PCNSL has strong invasive ability and poor prognosis. The prognosis was related with the age at diagnosis, mult - locus lesion and karnofski performance scale （KPS）.