Lauw Marietya I S, Lucas Calixto-Hope G, Ohgami Robert S, Wen Kwun Wah
Department of Pathology, University of California, San Francisco, CA 94143, USA.
Department of Pathology, Helen Diller Family Comprehensive Cancer Center, University of California, San Francisco, CA 94158, USA.
Diagnostics (Basel). 2020 Dec 11;10(12):1076. doi: 10.3390/diagnostics10121076.
Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma that primarily arises in the brain, spinal cord, leptomeninges, and vitreoretinal compartment of the eye. The term is sometimes used interchangeably with primary central nervous system diffuse large B-cell lymphoma (PCNS DLBCL) because DLBCL comprises a great majority (90-95%) of PCNSL. Although rare, other types of lymphomas can be seen in the central nervous system (CNS), and familiarity with these entities will help their recognition and further workup in order to establish the diagnosis. The latter is especially important in the case of PCNSL where procurement of diagnostic specimen is often challenging and yields scant tissue. In this review, we will discuss the most common types of primary lymphomas that can be seen in the CNS with emphasis on the diagnostic histomorphologic, immunophenotypic, and molecular genetic features. The differential diagnostic approach to these cases and potential pitfalls will also be discussed.
原发性中枢神经系统淋巴瘤（PCNSL）是一种罕见的结外非霍奇金淋巴瘤，主要发生于脑、脊髓、软脑膜和眼的玻璃体视网膜腔。该术语有时与原发性中枢神经系统弥漫性大B细胞淋巴瘤（PCNS DLBCL）互换使用，因为DLBCL占PCNSL的绝大多数（90-95%）。虽然罕见，但中枢神经系统（CNS）中也可见其他类型的淋巴瘤，熟悉这些实体将有助于对其进行识别和进一步检查以确立诊断。在PCNSL病例中这一点尤为重要，因为获取诊断标本往往具有挑战性且组织量很少。在本综述中，我们将讨论CNS中可见的最常见原发性淋巴瘤类型，重点是诊断性组织形态学、免疫表型和分子遗传学特征。还将讨论这些病例的鉴别诊断方法和潜在陷阱。
