Kasaliwal Rajeev, Goroshi Manjunath, Khadilkar Kranti, Bakshi Ganesh, Rangarajan Venkatesh, Malhotra Gaurav, Lila Anurag, Bandgar Tushar, Shah Nalini S
Endocr Pract. 2015 Jul;21(7):719-24. doi: 10.4158/EP14471.OR. Epub 2015 Feb 25.
To describe the clinical presentation, biochemistry, imaging features, and treatment outcome of patients with primary adrenal lymphoma (PAL) presenting to a single tertiary care center.
We performed a retrospective analysis of case records of 7 patients diagnosed with PAL between January 2011 and May 2014 at our institution in Mumbai, India.
Median age of presentation in our series was 48 years (range, 41 to 60 years), with a male to female ratio of 6:1. Bilateral adrenal involvement was seen in 4 of 7 patients (58%). Adrenal insufficiency (AI) was seen in 3 of the 4 patients with bilateral involvement (75%). Computed tomography showed slight to moderate contrast enhancement of adrenal masses in 4 of 5 patients (80%). Diffuse, large, B-cell lymphoma (DLBCL) was the most common immunophenotype (85%). One patient died due to rapid disease progression even before starting chemotherapy. Six patients were treated with chemotherapy and/or external beam radiotherapy. After 1 year, 2 more patients had died, whereas 4 patients were in remission.
PAL should always be considered in differential diagnosis of bilateral adrenal mass with AI. DLBCL is the most common histologic subtype of PAL. Despite treatment, long-term prognosis of PAL remains poor.
描述在一家三级医疗中心就诊的原发性肾上腺淋巴瘤（PAL）患者的临床表现、生化指标、影像学特征及治疗结果。
我们对2011年1月至2014年5月间在印度孟买我们机构确诊为PAL的7例患者的病例记录进行了回顾性分析。
我们系列病例的中位发病年龄为48岁（范围41至60岁），男女比例为6:1。7例患者中有4例（58%）出现双侧肾上腺受累。4例双侧受累患者中有3例（75%）出现肾上腺功能不全（AI）。计算机断层扫描显示5例患者中有4例（80%）肾上腺肿块有轻度至中度强化。弥漫性大B细胞淋巴瘤（DLBCL）是最常见的免疫表型（85%）。1例患者甚至在开始化疗前就因疾病快速进展死亡。6例患者接受了化疗和/或外照射放疗。1年后，又有2例患者死亡，4例患者缓解。
在鉴别诊断伴有AI的双侧肾上腺肿块时应始终考虑PAL。DLBCL是PAL最常见的组织学亚型。尽管进行了治疗，PAL的长期预后仍然很差。
