Spyroglou A, Schneider H J, Mussack T, Reincke M, von Werder K, Beuschlein F
Department of Medicine, Endocrine Research, University Hospital Innenstadt, Ludwig Maximilians University, Munich, Germany.
Exp Clin Endocrinol Diabetes. 2011 Apr;119(4):208-13. doi: 10.1055/s-0031-1271629. Epub 2011 Mar 3.
Primary adrenal lymphoma (PAL) is an extremely rare entity, with approximately 70 cases reported in the English literature and 120 cases worldwide. Here we report the cases of a 53-year-old and a 62-year-old male patient and a 60-year-old female patient affected by large B-cell non-Hodgkin lymphoma of the adrenal gland. We summarize the diagnostic approaches that confirmed the diagnosis of PAL and describe individual treatment outcomes after therapy. Based on these case reports and a review of the literature patients are usually in the 6th or 7th decade of life and present with B-symptoms or rapidly progressive adrenal insufficiency in case of bilateral involvement. The identification of bilateral adrenal masses often causes a severe diagnostic problem. An etiological approach with assessment of the hormonal profile and detailed diagnostic imaging should be aimed at. Furthermore, if PAL is suspected biopsy of the adrenal mass should be performed after biochemical exclusion of a pheochromocytoma. Once the diagnosis is established further treatment decisions should be made in a multi-disciplinary setting in specialized centers.
原发性肾上腺淋巴瘤（PAL）是一种极其罕见的疾病，英文文献报道约70例，全球范围内有120例。在此，我们报告了1例53岁男性、1例62岁男性和1例60岁女性患者罹患肾上腺大B细胞非霍奇金淋巴瘤的病例。我们总结了确诊PAL的诊断方法，并描述了治疗后的个体治疗结果。基于这些病例报告及文献回顾，患者通常处于60或70岁年龄段，若双侧受累则表现为B症状或快速进展的肾上腺功能不全。双侧肾上腺肿块的识别常常会引发严重的诊断难题。应采用评估激素水平及详细诊断性影像学检查的病因学方法。此外，如果怀疑为PAL，应在生化排除嗜铬细胞瘤后对肾上腺肿块进行活检。一旦确诊，应在专业中心的多学科环境中做出进一步的治疗决策。
