肾上腺弥漫大B细胞淋巴瘤6例临床病理分析

Adrenal diffuse large B-cell lymphoma: a clinicopathological analysis of 6 cases

目的 探讨肾上腺弥漫大B细胞淋巴瘤（diffuse large B-cell lymphoma,DLBCL）的临床病理学特征、免疫表型、鉴别诊断及治疗。方法 回顾性分析6例肾上腺DLBCL的临床病理学特征、影像学资料、免疫表型、治疗及预后,并复习相关文献。结果6例肾上腺DLBCL中男性3例,女性3例;2例为双侧肾上腺占位,3例为右侧,1例为左侧;其中仅1例确诊为原发,1例可疑原发,其他4例为继发;2例伴脾肿大,1例伴外周血皮质醇升高。免疫表型：6例肾上腺DLBCL表达CD20、CD79a或PAX5,T细胞标记CD3和CD43均阴性,Ki-67增殖指数50%-80%;4例表达MUM1,不表达CD10和BCL-6。随访：3例死亡,分别生存9个月、12个月和24个月。2例存活。1例失访。结论 肾上腺淋巴瘤少见,其中以继发性多见,原发性罕见。病理类型以DLBCL最常见。治疗以联合化疗为主,预后差。

Purpose To investigate the clinicopathological characteristics, diagnosis, differential diagnosis, immunohistochemistry and treatment of adrenal diffuse large B-cell lymphoma （DLBCL）. Methods Six cases of adrenal DLBCL pathological features, clinical data, imaging data, treatment and prognosis were observed and related literatures were reviewed. Results The 6 cases consisted of 3 males and 3 females. 3 cases involved the right adrenal gland, 1 case the left, and the other 2 cases bilateral adrenal glands. Only 1 case was confirmed as primary, another case was suspected as primary, and 4 cases were secondary. There were 2 cases presenting with splenomegaly, and 1 case with increased cortisol level. Immunohistochemically, CD20, CD79a and PAX5 were expressed with diffuse and strong staining for adrenal DLBCL of 6 cases. CD3 and CD43 were negative. Ki-67 proliferation index of adrenal DLBCL of 6 cases was expressed for 50% - 80%. MUM1 was diffusely positive and CD10, BCL-6 were negative for four cases. The date of last follow-up was September 22, 2015. 3 patients died with a follow-up survival time of 9, 12, 24 months, respectively. 2 patients were alive, and 1 patient dropped out. Conclusion A majority of adrenal lymphoma is secondary, and primary adrenal lymphoma is rare. The most common type is DLBCL. The principal treatment is adjuvant combination chemotherapy, and prognosis is poor.