From the Department of Endocrinology, University Hospital of Dijon, Dijon, France.
Department of Hematology, University Hospital of Dijon, Dijon, France.
QJM. 2017 Feb 1;110(2):103-109. doi: 10.1093/qjmed/hcw174.
This study aimed to identify the clinical, radiological and prognostic features of primary adrenal lymphoma (PAL) in order to diagnose the disease more accurately.
A retrospective multi-centre study was conducted on the clinical, biological and radiological features as well as the treatment and overall survival outcomes in PAL.
Between 1994 and 2014, 28 patients from five regions of eastern France were diagnosed with primary adrenal lymphoma. The revealing symptoms were a worsening general state (77%), weight loss (77%) and abdominal pain (42%). Biological features of PAL were almost omnipresent: increased LDH, β2 microglobulin, CRP or ferritinaemia levels. The PAL was bilateral in 20 cases (71%), adrenal insufficiency was searched for in 11 patients and found in eight (73%). CT scans showed masses of various sizes measuring up to 180 mm. On MRI, the lesions were hypointense in T1 and hyperintense in T2. When done, positron emission tomography with fluorodeoxyglucose (FDG-PET) showed locations not seen on the CT and revealed extra-adrenal locations in 70% of examinations. Adrenalectomy brought no benefit. The overall survival rate was poor (61.9% at 2 years) despite polychemotherapy.
The clinical presentation of PAL comprised major general symptoms. Adrenal insufficiency was very common in patients with bilateral involvement but was not systematically tested. PET was an efficient examination to visualize extra-adrenal locations. The preliminary results of MRI to distinguish between PAL and adrenocortical carcinoma should be confirmed. Further studies are needed to establish an optimal strategy for the management of these primary adrenal lymphomas.
本研究旨在确定原发性肾上腺淋巴瘤（PAL）的临床、影像学和预后特征，以便更准确地诊断该疾病。
对法国东部五个地区的 PAL 患者的临床、生物学和影像学特征以及治疗和总生存结局进行了回顾性多中心研究。
1994 年至 2014 年间，法国东部五个地区的 28 名患者被诊断为原发性肾上腺淋巴瘤。主要表现为进行性全身状态恶化（77%）、体重减轻（77%）和腹痛（42%）。PAL 的生物学特征几乎普遍存在：LDH、β2 微球蛋白、CRP 或铁蛋白水平升高。20 例（71%）为双侧病变，11 例（73%）患者检查发现肾上腺皮质功能不全，8 例（73%）患者确诊。CT 扫描显示大小不一的肿块，最大直径达 180mm。在 MRI 上，病变在 T1 上呈低信号，在 T2 上呈高信号。当进行氟脱氧葡萄糖正电子发射断层扫描（FDG-PET）时，显示了 CT 上未发现的位置，并在 70%的检查中发现了肾上腺外位置。尽管进行了化疗，但肾上腺切除术并不能带来获益。尽管进行了化疗，但总生存率仍较差（2 年时为 61.9%）。
PAL 的临床表现主要为全身重大症状。双侧受累患者常合并肾上腺皮质功能不全，但并未系统检查。PET 是一种有效的检查方法，可显示肾上腺外位置。MRI 区分 PAL 和肾上腺皮质癌的初步结果尚需进一步证实。需要进一步研究以确定这些原发性肾上腺淋巴瘤的最佳治疗策略。
