Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-cho, Sendai 980-8574, Japan.
Exp Hematol Oncol. 2013 Aug 2;2(1):19. doi: 10.1186/2162-3619-2-19.
Primary adrenal lymphoma (PAL) is an extremely rare subtype of extranodal non-Hodgkin's lymphoma. Some researchers have reported some of the characteristics of PAL and its association with poor prognosis; however, the clinicopathological features of PAL remain to be elucidated.
From 2008 to 2011 we experienced seven cases of PAL in our institutions. We retrospectively analyzed the clinical and pathological features of these patients.
The patients ranged in age from 50 to 85 years, with a median of 71 years. The overall male:female ratio was 6:1. All seven patients were diagnosed with diffuse large B-cell lymphoma (DLBCL) pathologically. Bilateral adrenal involvement was confirmed in five patients. The median largest tumor diameter at diagnosis was 58 mm. The Ki-67 index was generally high (>70%). All patients were treated with rituximab-containing chemotherapy, and central nervous system (CNS) prophylaxis was conducted for three patients. One patient with CNS involvement at the time of the diagnosis also received whole-brain radiation. The overall survival rate at two years was 57% (median follow-up; 24.8 months). It is noteworthy that the three patients who received a full course of the rituximab-containing regimen and CNS prophylaxis are currently alive without disease relapse, and that none of the seven patients died due to progression of lymphoma.
Primary adrenal DLBCL can be a clinically aggressive disease entity. Rituximab-containing chemotherapy combined with CNS prophylaxis could be a reasonable option for the treatment of PAL; however, analyses of more PAL cases are needed for the establishment of this strategy.
原发性肾上腺淋巴瘤（PAL）是一种极其罕见的结外非霍奇金淋巴瘤亚型。一些研究人员已经报道了 PAL 的一些特征及其与预后不良的关系；然而，PAL 的临床病理特征仍有待阐明。
2008 年至 2011 年，我们在机构中遇到了 7 例 PAL 病例。我们回顾性分析了这些患者的临床和病理特征。
患者年龄 50-85 岁，中位数为 71 岁。男女比例为 6:1。所有 7 例患者均经病理诊断为弥漫性大 B 细胞淋巴瘤（DLBCL）。5 例患者双侧肾上腺受累。诊断时最大肿瘤直径的中位数为 58mm。Ki-67 指数普遍较高（>70%）。所有患者均接受利妥昔单抗联合化疗治疗，3 例患者行中枢神经系统（CNS）预防。1 例诊断时合并 CNS 受累的患者还接受了全脑放疗。2 年总生存率为 57%（中位随访时间；24.8 个月）。值得注意的是，接受完整利妥昔单抗方案和 CNS 预防的 3 例患者目前无疾病复发且存活，7 例患者均未因淋巴瘤进展而死亡。
原发性肾上腺 DLBCL 可能是一种具有侵袭性的临床疾病实体。利妥昔单抗联合化疗联合 CNS 预防可能是治疗 PAL 的合理选择；然而，需要更多的 PAL 病例分析来建立这种策略。
