免疫适任者原发性中枢神经系统淋巴瘤(附125例病例分析)

Primary Central Nervous System Lymphoma in Patients with Normal Immunologic Function(with Clinical Analysis of 125 Cases)

目的分析免疫适任者原发性中枢神经系统淋巴瘤的临床资料,结合文献复习,探讨其发病率、临床表现、影像学特点、治疗及预后。方法从1995年8月至2006年10月对125例中枢神经系统淋巴瘤患者采取手术治疗,经病理检验证实。对其中89例进行了随访。对于近12年来中枢神经系统淋巴瘤的发病总数及其占中枢神经系统肿瘤的百分率进行统计。结果在125例患者中,男性82例,女性43例,年龄2～75岁,临床表现以颅压高为主。肿瘤单发96例,多发29例。其中全切69例,近全切除37例,大部切除8例,立体定向取活检11例。术后有81例放疗加化疗;21例单纯放疗;15例单纯化疗;6例未行放化疗;2例不详。随访89例,死亡62例。平均生存期13.62个月。存活27例。结论原发性淋巴瘤在免疫适任者为少见的中枢神经系统恶性肿瘤,其临床特点是恶性程度高、病情进展迅速、预后差,影像学有特征性表现。单纯手术治疗无助于提高患者生存期,应结合化疗、放疗。影响预后的因素为年龄、病人态和肿瘤累及的范围等。

Objective To investigate the incidence, clinical and radiological features, treatment and prognosis of immunocompetent patients with primary central nervous system lymphoma（PCNSL） through analyzing the clinical data. Methods One hundred and twenty-five consecutive patients certified histologically through craniotomy and/or stereotaxic biopsy were reviewed restropectively and underwent a systematic work-up including radiographs and/or computerized scan（CT）, ultrasound etc from August, 1995 to October, 2006 at Tiantan Hospital, Beijing. Data were inputted into the database set up by Epidata 3.02. Statistical analysis was performed with SPSS 12.0. Results There were 82 men and 43 women, 96 cases had single lesion and 29 had multiple lesions. They had surgical treatment at the age of 2 ～ 75 years. The main presentation was related to increased intracranial pressure. Total resection were achieved in 69 cases, subtotal in 37 cases, partial in 8 cases, stereotactic biopsy in 11 cases. Postoperatively, combined radiotherapy and chemotherapy were given in 81 cases, radiotherapy alone in 21 cases, chemotherapy alone in 15 cases. Eight-nine cases have been followed up. At the time of last contact, 62 patients had died, all but five from PCNSL disease or from complications due to its treatment. The median survival time was 13.62 months. Twenty-seven patients were still alive at the last contact. Conclusion PCNSL is a rare highly malignant CNS tumor in the general immunocompetent population and is a very aggressive neoplasm with a poor prognosis. CT scan generally provide results that are suggestive but not conclusive for PCNSL in immunocompetent patients, but MRI scan with rim enhancement is specific for it. Because of the location and the infiltrating nature of the lesions, surgical resection alone is of no good to improve the survival of patients with PCNSL. Surgery combined with chemotherapy and radiotherapy should be the main treatment options. Controversy continues regarding prognostic factors in PCNSL in immunocompetent patients. The following, however are the most widely accepted factors： the age of onset performance status, location of the tumors.