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移植后原发性中枢神经系统淋巴瘤

Posttransplant primary CNS lymphoma.

作者信息

Phan T G, O'Neill B P, Kurtin P J

机构信息

Department of Neurology, Mayo Clinic and Mayo Foundation, Rochester, MN 55905, USA.

出版信息

Neuro Oncol. 2000 Oct;2(4):229-38. doi: 10.1093/neuonc/2.4.229.

DOI:10.1093/neuonc/2.4.229
PMID:11265232
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1920594/
Abstract

The records and neuro-imaging studies of 8 cases of posttransplant primary CNS lymphoma (PT-PCNSL) diagnosed at Mayo Clinic Rochester between 1970 and 1998 were reviewed retrospectively. All patients received organ transplantation. Patients who had hematologic transplantation were not included in the analysis. The median and mean age of the 4 men and 4 women was 45 years (range, 34 to 50 years). The median duration of symptoms before diagnosis was 36 days (range, 5 to 98 days). At diagnosis, the neurologic examination was focally abnormal in 6 of 8 patients. Compared with the initial computed tomographic study, MRI showed 25 additional brain lesions. Only 43.7% of lesions enhanced with contrast agent; of those that did, all but one were heterogeneous. Ependymal contact occurred in 5 patients. MRI lesion burden increased proportionally to the interval between scans. Diagnostic tissue was obtained by stereotactic biopsy from 6 patients and by open biopsy from 2. Hemorrhage occurred in the biopsy area in 4 patients who had stereotactic biopsy and 2 died (all had normal coagulation studies). Slides available for review (7 patients) showed that the tumors were of CD20-positive lineage and were positive for Epstein-Barr virus, using in situ hybridization. Six patients died. Median survival for the cohort was 13 weeks. PT-PCNSL has clinical and imaging features distinct from typical PCNSL. In our series, (1) PT-PCNSL presented nonspecifically and progressed rapidly, (2) stereotactic brain biopsy had significant morbidity, and (3) despite multimodal therapy, survival was poor.

摘要

回顾性分析了1970年至1998年间在梅奥诊所罗切斯特确诊的8例移植后原发性中枢神经系统淋巴瘤(PT-PCNSL)的记录和神经影像学研究。所有患者均接受了器官移植。血液学移植患者未纳入分析。4名男性和4名女性的年龄中位数和平均数为45岁(范围34至50岁)。诊断前症状的中位持续时间为36天(范围5至98天)。诊断时,8例患者中有6例神经系统检查有局灶性异常。与最初的计算机断层扫描研究相比,MRI显示额外有25个脑病变。只有43.7%的病变有造影剂增强;其中除1个外均为不均匀增强。5例患者出现室管膜受累。MRI病变负荷与扫描间隔成正比增加。6例患者通过立体定向活检获取诊断组织,2例通过开放活检获取。4例接受立体定向活检的患者活检区域发生出血,2例死亡(所有患者凝血研究均正常)。可供复查的切片(7例患者)显示,使用原位杂交法,肿瘤为CD20阳性谱系且爱泼斯坦-巴尔病毒呈阳性。6例患者死亡。该队列的中位生存期为13周。PT-PCNSL具有与典型PCNSL不同的临床和影像学特征。在我们的系列研究中,(1)PT-PCNSL表现不特异且进展迅速,(2)立体定向脑活检有显著的发病率,(3)尽管采用多模式治疗,生存率仍很低。

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