睾丸原发性淋巴瘤17例临床病理分析

Primary testicular lymphoma:a clinicopathologic analysis of 17 cases

目的 探讨睾丸原发性淋巴瘤（primary testicular lymphoma,PTL）的临床病理学特征。方法 回顾性分析17例PTL的临床病理学特征。结果 患者年龄37~81岁,中位年龄69岁;以睾丸无痛性肿大为主要临床表现;病变位于左侧6例,右侧10例,双侧1例;肿块最大径2~6cm。组织学分型：17例PTL中16例（94%）为弥漫大B细胞淋巴瘤（diffuse large B cell lym-phoma,DLBCL）,1例（6%）为结外NK/T细胞淋巴瘤（鼻型）。免疫表型提示16例DLBCL中14例为非生发中心B细胞样（non-germinal center B-cell-like,non-GCB）型,2例为生发中心B细胞样（germinal center B-cell-like,GCB）型;c-myc与BCL-2或BCL-6抗体共同阳性表达者（c-myc阳性率30%以上且BCL-2阳性率70%以上者）10例。对10例共表达病例行FISH检测,发现仅1例有MYC基因重排,未发现双重打击淋巴瘤（double-hit lymphoma,DHL）。EB病毒原位杂交检测除1例鼻型NK/T细胞淋巴瘤为阳性外均为阴性。AnnArbor临床分期：Ⅰ＋Ⅱ期15例（88%）,Ⅲ＋Ⅳ期2例（12%）。17例患者均行患侧睾丸根治性切除术,8例DLBCL患者行CHOP或R-CHOP方案化疗。结论 PTL发病率低,主要发生于老年人,最常见的组织学类型为DLBCL,以non-GCB型伴c-myc与BCL-2/BCL-6共表达为主,患者预后差。PTL中很少发现伴随MYC和BCL-2同时基因重排的DHL。PTL术前诊断困难,确诊需依靠病理组织学、免疫表型及分子病理学检查等。

Purpose To explore the clinicopathologic features of primary testicular lymphoma （PTL）. Methods Seventeen cases of PTL were selected from the files of Sichuan Provincial People＇ s Hospital. Related clinicopathologic data were analyzed retrospectively. Results The median age of the 17 patients was 69 years （range, 37-81 years）. Patients most primarily presented with a painless testicular mass that had gradually increased in size. 16 cases （94%） presented primarily as a unilateral testicular mass. And 1 case （6%） had bilateral involvement. 16 cases were diagnosed as diffuse large B cell lymphoma （ DLBCL）, of which non-GCB type were predominant. 1 case was extranodal NK/T cell lymphoma, nasal type （ENKTCL-NT）. Immunohistochemical analysis demonstrated that c-myc and BCL-2/BCL-6 were co-expressed in 10 cases. Only 1 case had MYC gene rearrangement and no double-hit lymphoma （DHL） was found by FISH. EBER was just positive in the case of ENKTCL-NT. 15 cases （88%） were in stage Ⅰ＋Ⅱ, and 2 cases （ 12% ） were in stageⅢ＋Ⅳ. Orchiectomy was carried out in all patients. And 8 cases of DLBCL patients received chemotherapy combined with CHOP or R-CHOP following the operation. Conclusion PTL has low incidence and mainly occurs in elderly patients. Non-GCB type of the DLBCL with c-myc and BCL-2/BCL-6 co-expression is the predominant entity of PTL, and the prognosis of the patients is poor. Double hit lymphoma is rare in PTL. The final diagnosis of PTL depends on histomorphology, immunohistochemistry and molecular pathologic studies.