Izquierdo Cristina, Velasco Roser, Vidal Noemí, Sánchez Juan José, Argyriou Andreas A, Besora Sarah, Graus Francesc, Bruna Jordi
Unit of Neuro-Oncology, Hospital Universitari de Bellvitge-ICO Duran i Reynals, Barcelona, Spain (C.I., R.V., N.V., S.B., J.B.); Department Cell Biology, Institute of Neurosciences, Physiology and Immunology, Universitat Autònoma de Barcelona, and Centro de Investigación Biomédica en Red Sobre Enfermedades Neurodegenerativas (CIBERNED), Bellaterra, Spain (R.V., J.B.); Department of Radiology, Institut de Diagnòstic per la Imatge, Hospital Universitari de Bellvitge-ICO Duran i Reynals, Barcelona, Spain (J.J.S.); Department of Neurology, St. Andrew's State General Hospital of Patras, Patras, Greece (A.A.A.); Service of Neurology, Hospital Clínic de Barcelona, Barcelona, Spain (F.G.).
Neuro Oncol. 2016 May;18(5):707-15. doi: 10.1093/neuonc/nov197. Epub 2015 Sep 27.
Primary central nervous system lymphomas may present as diffuse, nonenhancing infiltrative lesions. This rare variant is termed lymphomatosis cerebri (LC). We did a systematic review and analysis of the literature, adding our own cases, to better characterize LC in order to improve early diagnosis and treatment.
PubMed, ISI Web of Knowledge, and hospital databases were reviewed. Information was extracted regarding demographic, clinical, histological, cerebrospinal fluid (CSF), neuroimaging, and treatment variables. The impact of single parameters on overall survival (OS) was determined by applying univariate and multivariate analyses.
Forty-two patients were included (median age: 58 y; range: 28-80 y). At consultation, 52% of patients had a poor KPS. The most common presenting symptom was cognitive decline (59.5%). Imaging studies showed supratentorial and infratentorial infiltration in 55% of patients and bilateral hemispheric involvement in 95%. CSF pleocytosis was present in 51.5% of the patients. Median time to diagnosis was 4.5 (range: 1-30) months, and the diagnosis was not established until autopsy for 33% of patients. The median OS was 2.95 (range: 0.33-56) months; however, those patients who received methotrexate had a median OS of 13.8 (range: 0.7-56) months. Analysis identified KPS ≥ 70 (HR: 0.32; 95% CI: 0.114-0.894; P = .03) and treatment with methotrexate (HR: 0.19; 95% CI: 0.041-0.886; P = .034) as independent favorable prognostic factors, whereas T-cell lymphoma was independently related with a worse outcome (HR: 6.62; 95% CI: 1.317-33.316; P = .022).
LC is a misdiagnosed entity associated with considerable diagnostic delay. MRI evidence of bilateral hemispheric involvement and CSF pleocytosis should be alerts for this diagnosis. Treatment with methotrexate-based chemotherapy must be considered, especially for patients with good KPS.
原发性中枢神经系统淋巴瘤可能表现为弥漫性、无强化的浸润性病变。这种罕见的变异型被称为脑淋巴瘤病（LC）。我们对文献进行了系统回顾和分析，并纳入了我们自己的病例，以更好地描述LC的特征，从而改善早期诊断和治疗。
对PubMed、ISI Web of Knowledge和医院数据库进行了检索。提取了有关人口统计学、临床、组织学、脑脊液（CSF）、神经影像学和治疗变量的信息。通过单因素和多因素分析确定单个参数对总生存期（OS）的影响。
共纳入42例患者（中位年龄：58岁；范围：28 - 80岁）。就诊时，52%的患者KPS评分较差。最常见的首发症状是认知功能下降（59.5%）。影像学研究显示，55%的患者幕上和幕下有浸润，95%的患者双侧半球受累。51.5%的患者脑脊液有细胞增多。诊断的中位时间为4.5（范围：1 - 30）个月，33%的患者直到尸检才确诊。中位总生存期为2.95（范围：0.33 - 56）个月；然而，接受甲氨蝶呤治疗的患者中位总生存期为13.8（范围：0.7 - 56）个月。分析确定KPS≥70（HR：0.32；95%CI：0.114 - 0.894；P = 0.03）和甲氨蝶呤治疗（HR：0.19；95%CI：0.041 - 0.886；P = 0.034）为独立的有利预后因素，而T细胞淋巴瘤与较差的预后独立相关（HR：6.62；95%CI：1.317 - 33.316；P = 0.022）。
LC是一种常被误诊且诊断延迟时间较长的疾病。双侧半球受累的MRI证据和脑脊液细胞增多应警惕该诊断。必须考虑采用以甲氨蝶呤为基础的化疗，尤其是对于KPS评分较好的患者。
