目的观察睾丸弥漫性大B细胞淋巴瘤(DLBCL)的临床病理及免疫表型特点,探讨其病理诊断及预后。方法对58例睾丸DLBCL进行回顾性临床病理研究,包括形态学复习、免疫组织化学(EnVision法)染色、EB病毒编码小RNA(EBER)1/2原位杂交以及预后相关因素分析。结果58例睾丸DLBCL患者平均年龄62.1岁,中位年龄65岁。多数病程较短,51例(87.9%)就诊时处于临床I~Ⅱ期;48例(82.8%)为单侧睾丸受累。12例(20.7%)伴同侧腹股沟淋巴结肿大,少有其他器官累及。52例(89.7%)病理形态学表现为中心母细胞样细胞在睾丸间质内弥漫性浸润,并浸润曲细精管。睾丸白膜受侵、血管浸润分别见于14例(24.1%)和10例(17.2%)。Hans分型以非生发中心B细胞型为主(48/58,82.8%)。缺乏EB病毒感染。所有病例行病变睾丸切除,35例(60.3%)接受了术后化疗和(或)放疗。随访率为82.8%(48/58),其中28例(58.3%)患者死亡;1、3、5年总体生存率分别为55.7%、31.6%和27.6%。年龄〉60岁、B症状、血清乳酸脱氢酶水平升高、高临床分期及未接受术后联合治疗者预后差。结论睾丸DLBCL确诊时以局部病变为主,低临床分期,有一定的病理形态学特征,多为非生发中心型DLBCL,预后不良;术后联合化疗可延长患者的生存期。
Objective To investigate the clinicopathologic features, immunophenotype, diagnosis and differential diagnosis, and prognostic factors of testicular diffuse large B-cell lympholna ( DLBCL ) . Methods The clinical and pathologic profiles of 58 cases of testieular DLBCL were investigated. Immunohistoehemieal stainings and EBER1/2 in situ hybridization were performed on formalin fixed tissues. Results The average age of the patients was 62. 1 years, and the median age was 65 years. The course of disease was short in most of the cases. Clinical stages at diagnosis were mainly stage I or ]] (87.9%, 51/58 ) . Forty eight patients (82.8%) had unilateral testis involvement. Inguinal lymphadenopathy was observed in 12 (20. 7% ) patients and the other organs were seldom involved. Morphologically, centroblast-like neoplastic cells infiltrated interstitial tissue of testis diffusely and invaded into seminiferous tubules. Tunica albuginea and vessels were involved in 14 (24. 1% ) and 10 ( 17.2% ) patients, respectively. Immunophenotype analysis showed predoufinant non-GCB type of DLBCL (48/58, 82. 8% ) by Hans classification. No EBV infection was detected. Follow-up data were available in 48 (82. 8% ) patients. Twenty eight patients (58.3%) died of the disease. One-year, 3-year, and 5-year overall survivals were 55.7% , 31.6% and 27.6% , respectively. Age ( older than 60 years) , B-symptoms, high serum level of LDH, advanced Ann Arbor stage as well as lack of combination of therapy were associated with a poor prognosis. Conclusions This large series of testicular DLBCL mainly present with local disease at diagnosis. Most cases show non-GCB inmmnophenotype. Despite early clinical stage at presentation, the prognosis is poor. Combined chemotherapy postoperation may prolong survival of the patients.