散发性伯基特及伯基特样淋巴瘤临床病理分析

Clinicopathological analysis of sporadic Burkitt's and Burkitt-like lymphoma

目的:探讨伯基特淋巴瘤(BL)的临床病理特征、免疫表型及鉴别诊断。方法:运用组织病理学、免疫组织化学和EBER原位杂交,对13例BL及7例伯基特样淋巴瘤(BLL)患者的肿瘤组织进行检测,并复习相关文献。结果:20例散发性病例中,病变部位在淋巴结内者,位于颈部淋巴结6例,颌下淋巴结4例,锁骨下淋巴结1例,腋下淋巴结1例;病变部位在淋巴结外者,位于回盲部3例,精索、睾丸、结肠、肌肉、前列腺各1例。光镜下可见瘤组织由单一的、弥漫浸润的中等大小圆细胞组成,其中散在分布着吞噬细胞碎片的组织细胞,呈"星空"样改变。免疫表型检测示,瘤细胞弥漫表达CD20和CD79α,部分表达CD10,>90%的瘤细胞Ki-67阳性,而CD3、CD43、bcl-2和TdT呈阴性。EBER原位杂交发现,3例瘤细胞呈阳性。结论:散发性BL属高度恶性肿瘤,确诊依赖于病理组织学与免疫组化标记。

Objective To investigate the clinicopathological characteristics, immuno-phenotype and differential diagnosis of Burkitt＇s lymphoma （BL）. Methods Thirteen cases of BL and 7 cases of Burkitt-like lymphoma（BLL）were studied by histopathology, immunohistochemistry and in situ hybridization with review of literature. Results Among the 20 sporadic cases, 13 were male and 7 were female, with age ranging from 4-64 years. Of the intranodular cases, sites of involvement included the neck （6 cases）, the submandibular region （4 cases）, the infraclavieular （1 case）, and the axillary （1 case）. The extranodal cases included involvement of the ileocecal junction （3 cases）, the spermatic cord （1 case）, the testis （1 case）, the colon （1 case）, the skeletal muscle （1 case） and the prostate gland （1 case）. Microscopically, the tumor was characterized by uniform diffuse infiltration of medium-sized cells with round nuclei, coarse chromatin, 2-5 basophilic nucleoli, and an appreciable rim of basophilie cytoplasm. A stany-sky pattern imparted by interspersed tangible-body macrophages was highly characteristic. Immunohistoehemieally, the tumor cells were diffusely positive for CD20 and CD79α and some of them were positive for CDIO. Over 95% tumor cells showed expression of Ki-67. The tumor cells were negative for CD3, CD43, bcl-2 and TdT. EBER in situ hybridization showed positivity in 3 cases. Conclusions Sporadic BL is a highly aggressive tumor with extremely poor prognosis. Diagnosis of BL relied on pathological characteristic and immunohistoehemical staining. It is essential to differentiate BL from other types of lymphoma and small cell malignant tumors of non-lymphatic system.