Koeller K K, Smirniotopoulos J G, Jones R V
Department of Radiologic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
Radiographics. 1997 Nov-Dec;17(6):1497-526. doi: 10.1148/radiographics.17.6.9397461.
Once an extremely rare neoplasm, primary lymphoma of the central nervous system (CNS) now ranks behind only meningiomas and low-grade astrocytomas in prevalence. Understanding of primary CNS lymphoma has increased greatly in recent years as a result of special immunohistochemical stains. Virtually all primary CNS lymphomas are composed of B cells. Although a viral cause has been suggested in some cases, the exact cause of the disease is still under investigation. Primary CNS lymphoma has a distinct affinity for perivascular extension. Although granular nodules may be seen at gross pathologic inspection, diffuse microscopic spread is always present, which accounts for the ability of this tumor to produce distant disease and local recurrences. The dense cellularity of the tumor and its predilection for the periventricular region also explains its typical hyperattenuated appearance on unenhanced computed tomographic scans and hypointensity on T2-weighted magnetic resonance images. Virtually all lesions enhance with contrast material. Although the overall prognosis for patients with primary CNS lymphoma remains poor, some advances have been made with radiation therapy and chemotherapy for this once uniformly fatal disease.
原发性中枢神经系统淋巴瘤曾经是一种极为罕见的肿瘤，如今在发病率上仅次于脑膜瘤和低级别星形细胞瘤。近年来，由于特殊的免疫组化染色，对原发性中枢神经系统淋巴瘤的认识有了极大提高。几乎所有原发性中枢神经系统淋巴瘤都由B细胞组成。尽管在某些病例中提示有病毒病因，但该疾病的确切病因仍在研究中。原发性中枢神经系统淋巴瘤对血管周围扩展有明显的亲和力。尽管在大体病理检查中可能会看到颗粒状结节，但总是存在弥漫性的微观扩散，这解释了这种肿瘤产生远处疾病和局部复发的能力。肿瘤的致密细胞性及其对脑室周围区域的偏好也解释了其在未增强计算机断层扫描上典型的高密度外观以及在T2加权磁共振图像上的低信号强度。几乎所有病变在使用对比剂时都会增强。尽管原发性中枢神经系统淋巴瘤患者的总体预后仍然很差，但对于这种曾经一致认为是致命的疾病，放射治疗和化疗已经取得了一些进展。
