Grigg Andrew P, Connors Joseph M
Clinical Haematology and Medical Oncology, Royal Melbourne Hospital, Parkville, Victoria, Australia.
Clin Lymphoma. 2003 Dec;4(3):154-60. doi: 10.3816/clm.2003.n.024.
Primary adrenal lymphoma is a rare extranodal lymphoma with characteristic clinical features including a high incidence of bilateral involvement, predominantly diffuse large B-cell histology, and a low incidence of extra-adrenal disease at diagnosis. Patients are most commonly older men presenting with fever, lumbar pain, and/or symptoms of adrenal insufficiency. Prolonged disease-free survival appears uncommon, which may reflect a publication bias and/or the presence of additional adverse prognostic factors at diagnosis in most patients. Given the rarity of this disease, no prospective chemotherapy studies have been reported. Unresolved therapeutic issues include the optimal chemotherapy regimen (with vs. without monoclonal antibody), the role of bilateral adrenalectomy and/or adjuvant radiation therapy, and the need for central nervous system prophylaxis, given recent reports raising the possibility of a high risk of parenchymal or meningeal relapse. Multicenter collaborative retrospective reviews and prospective trials are needed to address these issues.
原发性肾上腺淋巴瘤是一种罕见的结外淋巴瘤，具有特征性临床特点，包括双侧受累发生率高、组织学上主要为弥漫性大B细胞淋巴瘤，以及诊断时肾上腺外疾病发生率低。患者最常见的是老年男性，表现为发热、腰痛和/或肾上腺功能不全症状。长期无病生存似乎并不常见，这可能反映了发表偏倚和/或大多数患者诊断时存在其他不良预后因素。鉴于这种疾病的罕见性，尚无前瞻性化疗研究报告。尚未解决的治疗问题包括最佳化疗方案（联合或不联合单克隆抗体）、双侧肾上腺切除术和/或辅助放疗的作用，以及鉴于最近有报告提出实质性或脑膜复发高风险的可能性，是否需要进行中枢神经系统预防。需要多中心协作回顾性研究和前瞻性试验来解决这些问题。
