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Abstract：
Primary adrenal lymphoma (PAL) is a rare form of adrenal mass. We summarize our experience in its clinical presentation, biochemical indexes, radiological features, pathological information, therapy regimens, and outcomes.
This was an institutional review board-approved retrospective review of medical records and surgical pathology specimens of patients with a diagnosis of PAL at the Chinese People\'s Liberation Army General Hospital and the First Affiliate Hospital of Xiamen University between July 2007 and July 2017.
Twenty-six patients were identified. The mean age at presentation was 60.84 ± 13.14 years with a male-to-female ratio of 2.25:1 (18:8). The most common presenting symptoms were loss of appetite (65%, 17/26), weight loss (62%, 16/26), abdominal pain (58%, 15/26), and fatigue (58%, 15/26). The levels of lactate dehydrogenase (75%, 15/20), β2-microglobulin (100%, 10/10), C-reactive protein (82%, 14/17), and ferritin (88%, 7/8) and the erythrocyte sedimentation rate (83%, 10/12) were elevated. Bilateral involvement was seen in 21 of 26 patients (81%); 12 of 19 evaluated patients with bilateral lesions (63%) were confirmed to have adrenal insufficiency. On computed tomography (CT), the mean tumor diameter was 7.31 ± 3.35 cm and the median Hounsfield density was 37.0 HU (range: 31.0-45.0 HU); 67% (10/15) and 27% (4/15) of lesions presented with mild and moderate enhancement after injection of contrast medium. 18F-fluorodeoxyglucose positron emission tomography (FDG PET)-CT revealed not only an adrenal tumor but also extra-adrenal lesions. Diffuse large B-cell lymphoma (DLBCL) was the most common phenotype (92%, 24/26). Ninety-two percent (24/26) of patients received chemotherapy while 8% (2/26) received unilateral adrenalectomy plus chemotherapy. The prognosis of PAL was poor, with a general survival time of 7.20 ± 5.18 months.
PAL is a rare disease. The clinical characteristics of PAL include loss of appetite and weight loss. Endocrine evaluation should be performed to determine whether patients have adrenal insufficiency, especially patients with bilateral lesions. FDG-PET appears to be more accurate than other imaging modalities in revealing extra-adrenal sites. Better therapy is required to improve the poor prognosis of PAL.
This was an institutional review board-approved retrospective review of medical records and surgical pathology specimens of patients with a diagnosis of PAL at the Chinese People\'s Liberation Army General Hospital and the First Affiliate Hospital of Xiamen University between July 2007 and July 2017.
Twenty-six patients were identified. The mean age at presentation was 60.84 ± 13.14 years with a male-to-female ratio of 2.25:1 (18:8). The most common presenting symptoms were loss of appetite (65%, 17/26), weight loss (62%, 16/26), abdominal pain (58%, 15/26), and fatigue (58%, 15/26). The levels of lactate dehydrogenase (75%, 15/20), β2-microglobulin (100%, 10/10), C-reactive protein (82%, 14/17), and ferritin (88%, 7/8) and the erythrocyte sedimentation rate (83%, 10/12) were elevated. Bilateral involvement was seen in 21 of 26 patients (81%); 12 of 19 evaluated patients with bilateral lesions (63%) were confirmed to have adrenal insufficiency. On computed tomography (CT), the mean tumor diameter was 7.31 ± 3.35 cm and the median Hounsfield density was 37.0 HU (range: 31.0-45.0 HU); 67% (10/15) and 27% (4/15) of lesions presented with mild and moderate enhancement after injection of contrast medium. 18F-fluorodeoxyglucose positron emission tomography (FDG PET)-CT revealed not only an adrenal tumor but also extra-adrenal lesions. Diffuse large B-cell lymphoma (DLBCL) was the most common phenotype (92%, 24/26). Ninety-two percent (24/26) of patients received chemotherapy while 8% (2/26) received unilateral adrenalectomy plus chemotherapy. The prognosis of PAL was poor, with a general survival time of 7.20 ± 5.18 months.
PAL is a rare disease. The clinical characteristics of PAL include loss of appetite and weight loss. Endocrine evaluation should be performed to determine whether patients have adrenal insufficiency, especially patients with bilateral lesions. FDG-PET appears to be more accurate than other imaging modalities in revealing extra-adrenal sites. Better therapy is required to improve the poor prognosis of PAL.
摘要：
原发性肾上腺淋巴瘤（PAL）是一种罕见的肾上腺肿块。我们总结了我们在其临床表现中的经验，生化指标,放射学特征，病理信息，治疗方案,和结果。
这是2007年7月至2017年7月期间在中国人民解放军总医院和厦门大学第一附属医院对诊断为PAL的患者的病历和手术病理标本进行的机构审查委员会批准的回顾性审查。
确定了26名患者。演示时的平均年龄为60.84±13.14岁，男女比例为2.25：1（18：8）。最常见的症状是食欲不振（65%，17/26),体重减轻（62%，16/26），腹痛（58%，15/26），和疲劳(58%，15/26)。乳酸脱氢酶水平(75%，15/20），β2-微球蛋白（100%，10/10),C反应蛋白（82%，14/17),和铁蛋白(88%，7/8)和红细胞沉降率(83%，10/12）升高。26例患者中有21例（81％）出现双侧受累；19例评估的双侧病变患者中有12例（63％）被证实患有肾上腺功能不全。计算机断层扫描(CT),平均肿瘤直径为7.31±3.35cm,中位Hounsfield密度为37.0HU(范围:31.0-45.0HU);67%(10/15)和27%(4/15)的病灶在注射造影剂后出现轻度和中度增强.18F-氟代脱氧葡萄糖正电子发射断层扫描（FDGPET）-CT不仅显示肾上腺肿瘤，而且显示肾上腺外病变。弥漫大B细胞淋巴瘤(DLBCL)是最常见的表型(92%,24/26).92％（24/26）的患者接受了化疗，而8％（2/26）的患者接受了单侧肾上腺切除术加化疗。PAL的预后较差,一般生存时间为7.20±5.18个月。
PAL是一种罕见的疾病。PAL的临床特征包括食欲不振和体重减轻。应进行内分泌评估以确定患者是否有肾上腺功能不全，尤其是双侧病变患者。FDG-PET在显示肾上腺外部位方面似乎比其他成像方式更准确。需要更好的治疗来改善PAL的不良预后。
这是2007年7月至2017年7月期间在中国人民解放军总医院和厦门大学第一附属医院对诊断为PAL的患者的病历和手术病理标本进行的机构审查委员会批准的回顾性审查。
确定了26名患者。演示时的平均年龄为60.84±13.14岁，男女比例为2.25：1（18：8）。最常见的症状是食欲不振（65%，17/26),体重减轻（62%，16/26），腹痛（58%，15/26），和疲劳(58%，15/26)。乳酸脱氢酶水平(75%，15/20），β2-微球蛋白（100%，10/10),C反应蛋白（82%，14/17),和铁蛋白(88%，7/8)和红细胞沉降率(83%，10/12）升高。26例患者中有21例（81％）出现双侧受累；19例评估的双侧病变患者中有12例（63％）被证实患有肾上腺功能不全。计算机断层扫描(CT),平均肿瘤直径为7.31±3.35cm,中位Hounsfield密度为37.0HU(范围:31.0-45.0HU);67%(10/15)和27%(4/15)的病灶在注射造影剂后出现轻度和中度增强.18F-氟代脱氧葡萄糖正电子发射断层扫描（FDGPET）-CT不仅显示肾上腺肿瘤，而且显示肾上腺外病变。弥漫大B细胞淋巴瘤(DLBCL)是最常见的表型(92%,24/26).92％（24/26）的患者接受了化疗，而8％（2/26）的患者接受了单侧肾上腺切除术加化疗。PAL的预后较差,一般生存时间为7.20±5.18个月。
PAL是一种罕见的疾病。PAL的临床特征包括食欲不振和体重减轻。应进行内分泌评估以确定患者是否有肾上腺功能不全，尤其是双侧病变患者。FDG-PET在显示肾上腺外部位方面似乎比其他成像方式更准确。需要更好的治疗来改善PAL的不良预后。
