原发性肾上腺淋巴瘤7例临床分析

Clinical analysis of 7 cases of primary adrenal lymphoma

目的探讨原发性肾上腺淋巴瘤(PAL)患者的临床特征、治疗情况及预后。方法回顾性分析7例PAL患者,包括弥漫大B细胞淋巴瘤(DLBCL)6例、NK/T细胞淋巴瘤1例的临床资料。结果7例患者中位年龄为63(39~76)岁,男6例,女1例;双侧发病5例,单侧发病2例;7例患者均接受单侧肾上腺肿块切除术。DLBCL组5例化疗,2例(40%)完全缓解(CR),1例(20%)部分缓解(PR),总反应率(0 RR)为60%;NK/T细胞淋巴瘤患者采用以左旋门冬酰胺酶为主的方案化疗联合放疗,患者达CR。4例DLBCL患者分别生存0.2、2.7、2.8、9.6个月后死亡。结论PAL很罕见,恶性度高,预后差,DLBCL最常见,好发于老年男性,双侧多见,治疗上以联合化疗为主。

Objective To explore the clinical characteristics,treatment and prognosis of patients with primary adrenal lymphoma(PAL).Methods The clinical data of 7 cases of PAL were retrospectively analyzed,including 6 cases of diffuse large B-cell lymphoma(DLBCL)and 1 case of NK/T-cell lymphoma.Results The median age of 7 patients was 63 years(range 39-76 years),6 cases were male and 1 case was female.Two cases presented with unilateral and 5 cases with bilateral masses.All the 7 patients underwent unilateral adrenalectomy.Of the 6 cases with DLBCL,5 cases received chemotherapy,of which complete remission(CR)was achieved in 2 cases(2/5)and partial remission(P R)was achieved in 1 case(1/5),and the overall response rate(ORR)was 60%(3/5).The 1 case with NK/T cell lymphoma was treated with L-asparaginase based chemotherapy combined with radiotherapy,and the patient achieved CR.Four cases of DLBCL died with a follow-up survival time of 0.2,2.7,2.8,and 9.6 months,respectively.Conclusions PAL is rare with high malignancy and poor prognosis.DLBCL is the most common type of PAL,predominantly occurring in elderly males and mostly bilateral presence.Combined chemotherapy is the main treatment for PAL.