肺部结外鼻型NK/T细胞淋巴瘤11例临床病理学分析

Pulmonary extranodal NK/T-cell lymphoma,nasal type:a clinicopathological analysis of eleven cases

目的探讨肺部结外鼻型NK/T细胞淋巴瘤(extranodal NK/T-cell lymphoma,nasal type,ENKTL-NT)的临床病理特点、诊断及鉴别诊断。方法收集郑州大学第一附属医院2013年8月至2018年11月间11例诊断为肺部ENKTL-NT的病例,结合组织形态学、免疫组织化学和原位杂交检测,观察分析其临床病理特点,并结合文献进行讨论。结果11例肺部ENKTL-NT中,男性8例,女性3例,男女之比为8∶3;年龄30~74岁,中位年龄43岁,平均年龄48岁;发生部位:左肺上、下叶各1例,右肺下叶1例,双肺均累及者8例。临床症状主要表现为发热,并常伴咳嗽、咳痰,多数痰中带血。11例标本中10例为粗针穿刺,1例为支气管活检。所有病例均为ⅣE期。组织形态学特点为凝固性坏死和多种炎性细胞混合浸润的背景上,具有明显多形性的肿瘤性淋巴细胞散在或灶性分布,仅少数病例内肿瘤性淋巴细胞弥漫分布,少数病例可见血管中心性和血管破坏性浸润。免疫学标记:肿瘤细胞多数表达CD3ε、CD3、CD43、CD56、T细胞胞质内抗原1、颗粒酶B,不表达CD20、CD79a、CD5,Ki-67阳性指数较高,约40%~90%,EB病毒编码的小mRNA原位杂交均阳性。5例获得随访(1~62个月),其中4例死亡,生存期1周至13个月。结论肺部ENKTL-NT少见,恶性度高,预后差;送检标本多为穿刺活检标本,因坏死较多,细胞成分较杂,极易漏诊或误诊,结合临床病史、免疫组织化学和原位杂交可有效提高肺穿刺标本中ENKTL-NT的诊断。

Objective To investigate the clinicopathological features,diagnosis and differential diagnosis of pulmonary extranodal NK/T-cell lymphoma,nasal type(ENKTL-NT).Methods Eleven cases of newly diagnosed(10 puncture biopsies and 1 transbronchial biopsy),previously untreated pulmonary ENKTL-NT were collected at the First Affiliated Hospital of Zhengzhou University,from August 2013 to November 2018.The clinicopathological features including histomorphology,immunohistochemistry and in situ hybridization were collected and analyzed.Results Among the 11 cases,8 were males and 3 were females,with a male to female ratio of 8∶3.The age range was from 30 to 74 years,with an average of 48 years and a median of 43 years.Tumors involved bilateral lung lobes in 8 cases,the upper left lobe in 1 case,lower left lobe in 1 case,and upper right lobe in 1 case.Main clinical symptoms included fever,often accompanied by cough,and bloody sputum in most cases.All cases were stageⅣE.Histological features included scattered or focal aggregates of marked pleomorphic tumor lymphocytes,accompanied by necrosis and heavy admixture of inflammatory cells.In a few cases,diffuse neoplastic lymphocytes or vascular central and destructive infiltrations were seen.Tumor cells in most cases expressed CD3ε,CD3,CD43,CD56,TIA-1,granzyme B,but did not express CD20,CD79a,and CD5.Ki-67 index ranged from 40%to 90%.All cases were positive for EBER by in situ hybridization.Four of five patients died during follow-up with a survival period of only 1 week to 13 months.Conclusions Pulmonary ENKTL-NT is rare,high grade malignancy with a poor prognosis.Misdiagnosis is common due to lesional necrosis and heterogeneous cell components.Immunohistochemistry and EBER in situ hybridization are essential for accurate diagnosis.