Department of Neurology, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra, India.
Neurol India. 2009 Nov-Dec;57(6):756-63. doi: 10.4103/0028-3886.59472.
Primary central nervous system (CNS) lymphoma (PCNSL) is a rare malignant non-Hodgkin's lymphoma and it accounts for 1% of all intracranial tumors. Only a few PCNSL studies have been reported from India, and studies on prognostic factors determining outcome, or evaluation of the response to currently accepted treatment, are lacking.
This study attempts to further delineate the clinical, radiological and pathological profile of PCNSL in India, to evaluate response to treatment and to assess usefulness of the International Extranodal Lymphoma Study Group (IELSG) score.
All patients with pathologically proven PCNSL admitted over three years at a large tertiary care institution were studied.
Clinical features, IELSG prognostic score, imaging and pathological features, and response to treatment were evaluated. Results were analyzed using chi 2 test.
Of 26 patients found, all except two were immunocompetent. Median age at diagnosis was 59 years. Focal deficits (76.9%) and neuropsychiatric symptoms (57.6%) were the commonest presenting complaints. Except for one case, at least some contrast enhancement was seen in brain lesions of all patients. Pathological studies showed high grade diffuse large B-cell (DLBCL) histology in 96.2% of patients. Of 22 patients who received methotrexate (MTX) based chemotherapy with/without radiotherapy; six died, with a response rate of 72.7%. Median survival was 10 months. Median follow-up duration was 14.5 months. Four patients developed treatment-related cognitive decline. All six patients with IELSG score of 4/5 died, while all 16 patients with a score of 0-3 survived.
PCNSL presents most commonly in the sixth decade with focal neurological deficit, behavioral symptoms and cognitive decline. High grade DLBCL is the commonest histological subtype. Steroids should ideally be withheld until biopsy as they may confound the diagnosis. Most immunocompetent patients respond well to high dose MTX-based chemotherapy with/without radiation. High IELSG scores correlate with worse prognosis in patients with PCNSL.
原发性中枢神经系统（CNS）淋巴瘤（PCNSL）是一种罕见的恶性非霍奇金淋巴瘤，占所有颅内肿瘤的 1%。仅有少数来自印度的 PCNSL 研究报告，缺乏关于决定预后的预后因素研究，或缺乏对目前可接受治疗的反应评估。
本研究旨在进一步阐明印度 PCNSL 的临床、放射学和病理学特征，评估对治疗的反应，并评估国际结外淋巴瘤研究组（IELSG）评分的有用性。
在一家大型三级医疗机构中，对三年期间内经病理证实为 PCNSL 的所有患者进行了研究。
评估了临床特征、IELSG 预后评分、影像学和病理学特征以及对治疗的反应。使用卡方检验分析结果。
共发现 26 例患者，除 2 例外均为免疫功能正常。诊断时的中位年龄为 59 岁。局灶性缺损（76.9%）和神经精神症状（57.6%）是最常见的首发症状。除 1 例外，所有患者的脑部病变至少有一些对比增强。病理研究显示 96.2%的患者为高级弥漫性大 B 细胞（DLBCL）组织学。在接受甲氨蝶呤（MTX）为基础的化疗联合/不联合放疗的 22 例患者中，有 6 例死亡，反应率为 72.7%。中位生存期为 10 个月。中位随访时间为 14.5 个月。4 例患者出现与治疗相关的认知能力下降。所有 IELSG 评分为 4/5 的 6 例患者均死亡，而 IELSG 评分为 0-3 的 16 例患者均存活。
PCNSL 最常见于 6 旬，表现为局灶性神经缺损、行为症状和认知功能下降。高级 DLBCL 是最常见的组织学亚型。在进行活检之前，理想情况下应避免使用类固醇，因为它们可能会混淆诊断。大多数免疫功能正常的患者对大剂量 MTX 为基础的化疗联合/不联合放疗反应良好。高 IELSG 评分与 PCNSL 患者的预后较差相关。
