甲状腺原发性Burkitt淋巴瘤临床病理观察

Primary thyroid Burkitt lymphoma:clinicopathological analyses

目的甲状腺原发性Burkitt淋巴瘤非常罕见,对其临床病理特点进行探讨。方法对1例患有甲状腺Burkitt淋巴瘤的9岁男童进行详细的临床资料和病理形态学观察,并采用MaxVision法进行免疫组化染色、EBER原位杂交和c-myc基因FISH检测。结果甲状腺组织内见中等大小异型淋巴细胞弥漫浸润,可见明显"星空"现象。肿瘤细胞CD20、CD10、bcl-6、CD43和CD38(+),CD3、CD5、bcl-2和TDT均(-),Ki-67阳性率>95%。FISH检测有c-myc基因位点的染色体断裂重组。EBER分子原位杂交(-)。结论本例为原发于甲状腺的散发型Burkitt淋巴瘤,与EBV感染无关。需要与弥漫性大B细胞性淋巴瘤、结外黏膜相关淋巴组织边缘区淋巴瘤和B淋巴母细胞性淋巴瘤/白血病进行鉴别。

Objective To investigate the clinicopathological features of a rare case of primary thyroid Burkitt lymphoma （BL）. Methods A 9-year-old-boy with Burkitt lymphoma in the thyroid was analyzed by clinicopathological observation, immunohistochemistry （CD20, CD10, bcl-6, CD43, CD38, CD3, CD5, bcl-2, TDT and Ki-67）, in situ hybridization for Epstein-Barr virus, and FISH for c-myc breakpoint. Results The tumor was composed of monomorphic medium-sized lymphoid cells infiltrating diffusely in thyroid tissue, and had a prominent starry-sky pattern. The tumor cells expressed CD20, CD10, bcl-6, CIM3 and CD38 but not CD3, CD5, bcl-2 and TDT. The Ki-67 labeling index was more than 95% in this tumor with c-myc breakpoint （ ＋ ） and EBER（ - ）. Conclusion This case is a sporadic BL involving primarily the thyroid without EBV infection. The differential diagnosis of this BL includes diffuse large B-cell lymphoma, extranodal marginal lymphoma of mucosa-associated lymphoid tissue and B lymphoblastic lymphoma/leukaemia.