Jamal Siraj El, Li Shiyong, Bajaj Renu, Wang Zixuan, Kenyon Lawrence, Glass Jon, Pang Changlee S, Bhagavathi Sharathkumar, Peiper Stephen C, Gong Jerald Z
Department of Pathology, Anatomy and Cell Biology, Thomas Jefferson University, Philadelphia, USA.
Brain Tumor Pathol. 2014 Oct;31(4):265-73. doi: 10.1007/s10014-013-0173-x. Epub 2014 Jan 8.
We report five cases of primary central nervous system (CNS) Epstein-Barr virus (EBV)-positive lymphoma of the elderly. This represented an incidence of 4 % of primary CNS diffuse large B-cell lymphoma (DLBCL) after EBV screening in 134 cases. All five patients were 65 years or older with no previous history of congenital or iatrogenic immune deficiencies. The histologic morphology of all the cases was DLBCL, with variable amounts of necrosis. The cell of origin (COO) as determined by the Hans algorithm disclosed germinal center type in 2 cases and non-germinal center type in 3 cases. MYC translocation was not detected, and MYC overexpression was detected in only one case. Three patients died shortly after diagnosis, and the remaining 2 patients were in complete remission for 2 and 10 years, respectively. We conclude that EBV+ DLBCL among the elderly is uncommon in primary CNS lymphoma in the Eastern United States. The patients usually present with a single mass lesion with headache and sensorimotor symptoms. The histologic morphology is DLBCL, but clonal T-cell gene rearrangement may be detected. The outcome varies from case to case and appears to be unrelated to the COO or MYC status.
我们报告了5例老年原发性中枢神经系统（CNS）爱泼斯坦-巴尔病毒（EBV）阳性淋巴瘤。这占134例经EBV筛查的原发性CNS弥漫性大B细胞淋巴瘤（DLBCL）的4%。所有5例患者均为65岁及以上，既往无先天性或医源性免疫缺陷病史。所有病例的组织形态学均为DLBCL，伴有不同程度的坏死。根据汉斯算法确定的细胞起源（COO）显示，2例为生发中心型，3例为非生发中心型。未检测到MYC易位，仅1例检测到MYC过表达。3例患者在诊断后不久死亡，其余2例患者分别完全缓解2年和10年。我们得出结论，在美国东部，老年EBV+ DLBCL在原发性CNS淋巴瘤中并不常见。患者通常表现为单个肿块病变，伴有头痛和感觉运动症状。组织形态学为DLBCL，但可能检测到克隆性T细胞基因重排。预后因病例而异，似乎与COO或MYC状态无关。
