Department of Pathology and Lab Medicine, North Shore University Hospital-Long Island Jewish Medical Center, 300 Community Drive, Manhasset, NY 11030, USA.
J Neurooncol. 2010 Feb;96(3):433-6. doi: 10.1007/s11060-009-9974-5. Epub 2009 Jul 19.
Primary central nervous system lymphoma (PCNSL) is a rare intracranial tumor, with an annual incidence of six per million population. Anaplastic variant of primary CNS diffuse large B-cell lymphoma is less common; to our knowledge, there is only one other case report in the world literature. We describe a 71 year old immunocompetent female without significant past medical history who presented with confusion and a homogeneously enhancing midline mass. The patient underwent craniotomy for tumor biopsy, followed by high-dose methotrexate-based chemotherapy despite a remarkably low performance status. Histologically, this tumor was composed of undifferentiated polymorphic tumor cells, multi-nucleated giant cells, extensive necrosis, and conspicuous mitotic activity, mimicking undifferentiated metastatic tumors. Immunohistochemical stains demonstrated immunopositivity of tumor cells for CD20, MUM-1, and BCL-6, and negative staining for CD3, CD10, and CD30. The clinical course, diagnostic workup, pathologic correlates, and treatment outcomes are described.
原发性中枢神经系统淋巴瘤（PCNSL）是一种罕见的颅内肿瘤，年发病率为每百万人口 6 例。原发性中枢神经系统弥漫性大 B 细胞淋巴瘤的间变型变体较少见；据我们所知，世界文献中只有另一个病例报告。我们描述了一位 71 岁免疫功能正常的女性，无明显既往病史，表现为意识混乱和中线均质强化肿块。尽管患者的一般情况较差，仍对其进行了开颅肿瘤活检，随后进行了大剂量甲氨蝶呤为基础的化疗。组织学上，该肿瘤由未分化的多形性肿瘤细胞、多核巨细胞、广泛坏死和明显有丝分裂活性组成，类似于未分化的转移性肿瘤。免疫组化染色显示肿瘤细胞对 CD20、MUM-1 和 BCL-6 呈免疫阳性，对 CD3、CD10 和 CD30 呈阴性。描述了该病例的临床经过、诊断检查、病理相关性和治疗结果。
