原发性肾上腺淋巴瘤的诊断与临床处理

Diagnosis and Clinical Treatment of Primary Adrenal Lymphoma

原发性肾上腺淋巴瘤(primary adrenal lymphoma,PAL)临床表现不典型,多数患者存在局部疼痛或全身发热、消瘦症状,在双侧受累患者中肾上腺皮质功能不全很常见,但仅少数患者进行全面系统的检查。正电子发射断层扫描/计算机体层成像是定性定位诊断这类肾上腺肿瘤的有效手段,磁共振成像用来区别肾上腺淋巴瘤与皮质癌尚有待更多研究证实。对于PAL的治疗尚需进一步研究,以对此类患者制定最佳的诊疗方案。目前,尚不清楚利妥昔单克隆抗体联合CHOP方案(环磷酰胺+阿霉素+长春新碱+强的松)治疗高危侵袭性PAL患者是否可提高患者完全反应率。病变早期,尤其是在肾上腺皮质功能不全出现之前诊断PAL,有助于减少患者的发病率和死亡率。

The clinical presentation of primary adrenal lymphoma(PAL)is comprised of major general symptoms.Although adrenal insufficiency is very common in patients with bilateral involvement,it has not been systematically tested.Positron emission tomography is an efficient examination to visualize extra-adrenal locations.The preliminary results of magnetic resonance imaging to distinguish between PAL and adrenocortical carcinoma should be confirmed.Further studies are needed to establish an optimal strategy for the management of patients with PAL.At present,it is unclear whether the adjunction of rituximab(R)and CHOP(cyclophosphamide,doxorubicin,vincristine,prednisone)can cure patients with high-risk aggressive lymphoma.It is also unknown whether the prolonged complete response was due to R-CHOP alone.It is necessary to emphasize that early diagnosis of PAL before the appearance of adrenal insufficiency contributes to decreasing the patients’morbidity and mortality.