Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, 1 Shuai Fu Yuan Hu Tong, Beijing, 100730, People's Republic of China.
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, People's Republic of China.
Virchows Arch. 2018 Jul;473(1):95-103. doi: 10.1007/s00428-018-2378-1. Epub 2018 May 25.
Primary adrenal lymphoma is a rare entity that, in most cases, is derived from B cells. The most commonly seen primary adrenal lymphoma is diffuse large B cell lymphoma (DLBCL). To better understand the clinicopathological and molecular features of these tumors, we studied 14 Chinese patients with DLBCL who initially presented with an adrenal tumor. The clinicopathological features of the 14 primary adrenal DLBCL cases were retrospectively reviewed using immunohistochemistry, immunoglobulin gene rearrangement analysis, evaluation of Epstein-Barr virus status, and fluorescence in situ hybridization. Patient age ranged from 43 to 69 years, with a mean age of 58 years. The patients most commonly presented with abdominal pain and adrenal mass. Ten patients exhibited bilateral adrenal masses, and four had unilateral adrenal masses (three left, one right). Thirteen of 14 DLBCLs were DLBCL not otherwise specified, and one was an intravascular large B cell lymphoma. According to the algorithm of Hans et al. (Blood 103:275-282, 2004), 13 and 1 cases were classified as the non-germinal center B-cell-like subtype and the germinal center B-cell-like subtype, respectively. The Ki-67 index ranged from 35 to 80%. Epstein-Barr virus-encoded RNA was detected by in situ hybridization in 6 of the 12 available cases. Two patients showed BCL-6 rearrangements. The follow-up period ranged from 1 to 87 months. During the follow-up period, four patients died of the disease, five were alive with the disease, four were alive without disease, and one was lost during the follow-up period. In summary, most primary adrenal lymphomas are non-germinal-center B-cell-like subtype DLBCLs, which have high proliferative activity and a poor prognosis.
原发性肾上腺淋巴瘤是一种罕见的疾病，大多数情况下来源于 B 细胞。最常见的原发性肾上腺淋巴瘤是弥漫性大 B 细胞淋巴瘤（DLBCL）。为了更好地了解这些肿瘤的临床病理和分子特征，我们研究了 14 例最初表现为肾上腺肿瘤的中国 DLBCL 患者。通过免疫组织化学、免疫球蛋白基因重排分析、评估 EBV 状态和荧光原位杂交，回顾性分析了 14 例原发性肾上腺 DLBCL 病例的临床病理特征。患者年龄 43-69 岁，平均年龄 58 岁。患者最常见的表现为腹痛和肾上腺肿块。10 例患者表现为双侧肾上腺肿块，4 例为单侧肾上腺肿块（3 例左侧，1 例右侧）。14 例 DLBCL 中有 13 例为非特指性弥漫大 B 细胞淋巴瘤，1 例为血管内大 B 细胞淋巴瘤。根据 Hans 等人的算法（Blood 103:275-282, 2004），13 例和 1 例分别归类为非生发中心 B 细胞样亚型和生发中心 B 细胞样亚型。Ki-67 指数为 35-80%。12 例可检测到的病例中，6 例通过原位杂交检测到 EBV 编码 RNA。2 例患者显示 BCL-6 重排。随访时间为 1-87 个月。随访期间，4 例患者死亡，5 例患者带瘤生存，4 例患者无瘤生存，1 例患者在随访期间失访。总之，大多数原发性肾上腺淋巴瘤是非生发中心 B 细胞样亚型的 DLBCL，具有高增殖活性和不良预后。
