Patel Bimal, Chacko Geeta, Nair Sheila, Anandan Jamie, Chacko Ari G, Rajshekhar Vedantam, Turel Mazda
Department of General Pathology, Section of Neuropathology, Christian Medical College, Vellore, Tamil Nadu, India.
Neurol India. 2015 Jan-Feb;63(1):77-82. doi: 10.4103/0028-3886.152658.
Primary central nervous system lymphomas (PCNSL) constitute a rare group of extranodal non-Hodgkin's lymphomas (NHLs).
To study the clinical and immunophenotypic profile of patients with a PCNSL who presented between the years 2000 and 2013 in a tertiary care center in South India.
This was a retrospective study. Demographic and clinical data were obtained from the clinical case records.
Cases of PCNSL involving brain.
Cases of PCNSL involving the spinal cord, meninges and orbit as well as intravascular large B-cell lymphoma, lymphomas with evidence of systemic disease or secondary lymphomas. Archived slides and tissue blocks were retrieved. All cases had hematoxylin and eosin stained sections and immunohistochemistry for CD20, CD3, and MIB-1. Additional immunohistochemistry was performed for CD10, BCL6, and MUM1 on paraffin blocks with sufficient tissue.
There were a total of 73 cases with the mean age of presentation being 45.9 years (range 8-71 years) and with a male predominance (male: female (M:F) = 2.3:1). Headache was the commonest presenting complaint. The mean duration of symptoms was 10.6 weeks. All patients were immunocompetent. Most tumors were supratentorial in location. Out of 73 cases, 70 presented with a diffuse large B-cell lymphoma (DLBCL), two with a Burkitt's lymphoma, and one with a lymphomatoid granulomatosis. Only 51 of the DLBCL cases had sufficient tissue for additional studies. Non-germinal center was the most common phenotype seen in 65.7% (33/51) of cases. Germinal center B-cell (GCB) phenotype was seen in 18/51 cases (34.3%).
DLBCL constituted the majority of PCNSLs and although non-germinal center was the predominant phenotype, more than a third of the cases were of the GCB phenotype. As the germinal center phenotype is known to have a better prognosis, further studies to explore its relevance in the Asian population are indicated.
原发性中枢神经系统淋巴瘤（PCNSL）是一组罕见的结外非霍奇金淋巴瘤（NHL）。
研究2000年至2013年间在印度南部一家三级护理中心就诊的PCNSL患者的临床和免疫表型特征。
这是一项回顾性研究。从临床病例记录中获取人口统计学和临床数据。
累及脑部的PCNSL病例。
累及脊髓、脑膜和眼眶的PCNSL病例以及血管内大B细胞淋巴瘤、有全身疾病证据的淋巴瘤或继发性淋巴瘤。检索存档的玻片和组织块。所有病例均有苏木精和伊红染色切片以及CD20、CD3和MIB-1的免疫组化检测。对有足够组织的石蜡块进行CD10、BCL6和MUM1的额外免疫组化检测。
共有73例，平均发病年龄为45.9岁（范围8 - 71岁），男性占优势（男：女（M:F）= 2.3:1）。头痛是最常见的主诉。症状的平均持续时间为10.6周。所有患者免疫功能正常。大多数肿瘤位于幕上。73例中，70例为弥漫性大B细胞淋巴瘤（DLBCL），2例为伯基特淋巴瘤，1例为淋巴瘤样肉芽肿病。DLBCL病例中只有51例有足够组织进行额外研究。非生发中心是最常见的表型，见于65.7%（33/51）的病例。生发中心B细胞（GCB）表型见于18/51例（34.3%）。
DLBCL构成了PCNSL的大多数，虽然非生发中心是主要表型，但超过三分之一的病例为GCB表型。由于已知生发中心表型预后较好，因此有必要进一步研究其在亚洲人群中的相关性。
