未放化疗而长期无瘤生存的原发扁桃体弥漫性大B细胞淋巴瘤的临床和病理学特征

Clinicopathological features of long-term tumor-free survival of patients with untreated primary tonsillar diffuse large B-cell lymphoma

目的探讨未放化疗而长期无瘤生存原发扁桃体弥漫性大B细胞淋巴瘤(DLBCL)的临床和病理学特征。方法收集首都医科大学附属北京友谊医院病理科2006年4月至2017年7月间,原发扁桃体DLBCL的会诊病例共80例,其中未放化疗而长期无瘤生存者10例为实验组,已治疗患者70例为对照组,对患者的临床资料、病理组织学、免疫组织化学染色及分子生物学检测结果进行回顾性分析。结果患者年龄6~79岁,平均年龄46.5岁,男性37例,女性43例。未放化疗而长期无瘤生存的DLBCL,病变大部分局限在扁桃体,切除活检显示结构仅部分破坏,病变比较新鲜,EB病毒编码的小RNA(EBER)检测和荧光原位杂交(FISH)检测t(14;18)结果均为阴性;基因重排检测为单克隆。未放化疗组在年龄、bcl-2表达、CMYC蛋白表达以及CMYC和bcl-2共表达方面与已治疗组相比,差异具有统计学意义(P<0.05);在性别、部位、分型、临床分期等方面,两者间差异无统计学意义(P>0.05);未放化疗组和已治疗组中位总生存期分别为81及20个月,差异无统计学意义(P>0.05)。在年龄<40岁的患者中,未放化疗组在部位和CMYC蛋白表达方面与已治疗组相比,差异有统计学意义(P<0.05),其他各方面差异均无统计学意义(P>0.05)。结论未放化疗而长期无瘤生存的原发扁桃体DLBCL具有相对独特的临床和病理学特征。生存分析结果显示未放化疗组可长期无瘤生存,可以使患者免受放疗和化疗带来的副作用。

Objective To investigate the clinicopathological features of long-term tumor-free survival in patients with untreated primary diffuse large B-cell lymphoma (DLBCL) of the tonsil. Methods The study included 80 consultation cases of primary tonsillar DLBCL from April 2006 to July 2017 in the Department of Pathology, Beijing Friendship Hospital, Capital Medical University. The patients were divided into two groups: experimental groups of 10 untreated patients with long-term tumor-free survival, and 70 patients who had been treated (control group). The clinical data, histopathological features, immunohistochemical staining, and molecular biology test results of the patients were analyzed retrospectively. Results Patients who had long-term tumor-free survival with untreated primary diffuse large B-cell lymphoma had the disease mostly confined to the tonsil. Biopsy showed that the tonsil structure was only partially effaced and the lesions were relatively "fresh". EBER and FISH test for t (14;18) results were negative. Gene rearrangement detection showed monoclonality. There was statistically significant difference between the age, bcl-2 expression, CMYC protein expression and co-expression of CMYC and bcl-2 between the untreated group and the treated group(P<0.05). Patient gender, tumor site, histological type and clinical stage showed no difference between the untreated group and the treated group (P>0.05);The median overall survival of the untreated group and treated group was 81 months and 20 months, respectively, and the difference was not statistically significant (P>0.05).In patients younger than 40 years of age, the untreated group had a statistically significant difference in primary site and CMYC protein expression compared with the treated group (P<0.05), and there was no statistical significance in other aspects. Conclusions Long-term tumor-free survival patients with untreated tonsillar primary DLBCL have relatively unique clinical characteristics. There is no significant difference in the prognosis between the untreated and treated groups, indicating radiotherapy and chemotherapy may not be required and therefore, avoiding related side effects.