原发性中枢神经系统淋巴瘤107例临床病理观察

目的：对原发性中枢神经系统淋巴瘤（PCNSLs）进行临床与病理观察，进一步认识其病理特性。方法：分析107例PCNSLs的临床资料，行HE染色与免疫组化（ABC法）LCA、L26、UCHL－1、Lyso、α1－AT、MAC、GFAP、S－100蛋白、EMA等标记，并区分T、B细胞类型。结果：该肿瘤以40岁以上中老年人多见，以颅内压力增高或肢体麻瘫为主要表现，肿瘤可发生于中枢神经系统的任何部位，以侵犯大脑半球（尤以额、颞叶）较多，PCNSLs的组织学特征为淋巴瘤的瘤细胞形态较单一，如体躯性淋巴瘤胞质常少；病灶周边区瘤细胞常围绕血管形成袖套状，病灶中央的瘤细胞呈片状分布伴灶性坏死和出血，肿瘤侵及脑膜可引起胶原纤维增多，免疫组化染色的33例中，有29例（占84.8%）为B细胞型，其中1例为富T的B细胞型；4例（15.2%）为T细胞型。结论：PCNSLs为高度恶性肿瘤，临床上起病急，主要表现为颅内压力增高、肢体乏力、瘫痪和神经精神症状；瘤细胞形态相似于体躯性淋巴瘤肿瘤周边部瘤细胞，有向血管性，以血管为中心，侵犯血管壁开成袖套状，中心部瘤细胞呈温分布，有坏死、出血，免疫组化显示为B细胞型与T细胞型，患者预后差。

Clinical pathology of primary central nervous system lymphomas: a study of 107 cases

Purpose To review 107 cases of primary central nervous system lymphoma (PCNSLs) and to investigate its clinical and pathological features. Methods A retrospective study of 107 cases of PCNSLs were carried out by the clinical and pathological surveys, HE stain and immunohistochemical detection using LCA, L26, UCHL 1, Lyso and Mam antibody and ABC method. Results This tumor predominantly involved the patients above 40 years old (man>woman). The signs of elevated intracranial pressure and hemiparalysis were the main clinical findings in majority of 107 cases. The lesions located in any region of the CNS, but the cerebral hemisphere (especial the frontal or temporal lobes) was more often involved. The histopathology of PCNSLs showed the following characterstics: (1) Tumor cells were similar to the systemic lymphoma cells; (2) The cells surrounded vessel walls to form concentric rings cuffing in the peripheral areas, diffuse in the central parts and the meningaes involvement resulting in the collagen and reticular fibers proliferation. (3) The immunohistochemical results showed that the predominant immunophenotype was B cell type with positive stain for L26(84 8%). Only 4 cases were T cell type (15 2%). Conclusions The PCNSLs are high malignant tumors predominant involving the older patients. Acute onset presents with increasing intracramial pressure, hemiparalysis and neuropsychiatric symptoms clinically; the tumor cells surround and involve vessels forming cuffing in perepheral, and diffuse with necrosis and heamorrage in central parts pathologically. B cell type is more than T cell type in immunostaining. The prognosis is poor.