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中枢神经系统原发性非霍奇金淋巴瘤

Primary non-Hodgkin's lymphomas of the CNS.

作者信息

Bogdahn U, Bogdahn S, Mertens H G, Dommasch D, Wodarz R, Wünsch P H, Kühl P, Richter E

出版信息

Acta Neurol Scand. 1986 Jun;73(6):602-14. doi: 10.1111/j.1600-0404.1986.tb04607.x.

Abstract

This paper reports on 10 patients (4 male, 6 female) with primary non-Hodgkin's lymphomas of the brain (CNS-NHL--mean age 46.8 years, mean postdiagnostic survival 10 months). Pathological CSF (cerebrospinal fluid) was found in all 8 patients examined (positive cytology in 7/8 cases). Solitary tumors, diffuse periventricular infiltration or diffuse cerebral infiltration were demonstrated in cerebral computer-assisted tomography (CAT). Angiographical findings were unspecific. The histologic subtypes were lymphoplasmacytoid immunocytoma (4), unclassified low grade (1), centroblastic (1), B-immunoblastic (1), T-immunoblastic (1), lymphoblastic convoluted T-cell type (1), unclassified high grade (1) NHL. Patients who had received radiotherapy (+/- surgery) in this group had a mean survival of 15.66 months (sigma = 7.63). In addition, an overview of 83 well-documented, cases of the literature tries to characterize main histological and topographical distributions, histology-, patient's age-, and therapy-related survival. Patients with primary CNS-NHL have a 5-year survival expectancy of 30% compared with 2.3% in secondary CNS-manifestations of systemic non-Hodgkin's lymphomas. In this report, the beneficial effect of radiotherapy (mean survival 30.3 months) compared to surgery or symptomatic treatment (3.6 or 3.3 months) could be confirmed. It is concluded that primary CNS-NHL frequently present with atypical neuropsychiatric syndromes; diagnosis should be established preferentially with CAT and CSF-examinations or stereotactic biopsies, whereas open surgery should be avoided. An approach to exact classification should be attempted, as survival is clearly related to histological subtypes.

摘要

本文报告了10例原发性脑非霍奇金淋巴瘤患者(4例男性,6例女性)(中枢神经系统非霍奇金淋巴瘤——平均年龄46.8岁,诊断后平均生存期10个月)。在所有8例接受检查的患者中均发现病理性脑脊液(脑脊液)(7/8例细胞学检查呈阳性)。脑部计算机断层扫描(CAT)显示为孤立性肿瘤、弥漫性脑室周围浸润或弥漫性脑浸润。血管造影结果不具有特异性。组织学亚型为淋巴浆细胞样免疫细胞瘤(4例)、未分类低级别(1例)、中心母细胞型(1例)、B免疫母细胞型(1例)、T免疫母细胞型(1例)、淋巴母细胞卷曲T细胞型(1例)、未分类高级别(1例)非霍奇金淋巴瘤。该组接受放疗(±手术)的患者平均生存期为15.66个月(标准差=7.63)。此外,对文献中83例记录完整的病例进行了综述,试图描述主要的组织学和部位分布、组织学、患者年龄及治疗相关的生存期。原发性中枢神经系统非霍奇金淋巴瘤患者的5年生存率为30%,而系统性非霍奇金淋巴瘤继发性中枢神经系统表现患者的5年生存率为2.3%。在本报告中,放疗(平均生存期30.3个月)与手术或对症治疗(3.6或3.3个月)相比的有益效果得到了证实。结论是,原发性中枢神经系统非霍奇金淋巴瘤常表现为非典型神经精神综合征;诊断应优先通过CAT和脑脊液检查或立体定向活检来确立,而应避免进行开放性手术。应尝试进行精确分类,因为生存期与组织学亚型明显相关。

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