梭形细胞变异型非特指性弥漫性大B细胞性淋巴瘤2例临床病理观察

Spindle cell variant of diffuse large B cell lymphoma: a cliniopathologic analysis of two cases

目的探讨梭形细胞变异型非特指性弥漫性大B细胞性淋巴瘤(DLBCL)的临床病理特点及鉴别诊断。方法对2例梭形细胞变异型非特指性DLBCL进行组织形态学观察及免疫组化、原位杂交、基因重排检测并文献复习。结果 2例均为女性,年龄分别为15岁和22岁。例1发生于腹腔肠系膜及大网膜,例2发生于前上纵隔。镜下肿瘤细胞肉瘤样生长,交错呈束状排列,间质有明显胶原纤维;肿瘤细胞胞质较少,核呈梭形或卵圆形,染色质粗颗粒状。免疫组化显示肿瘤细胞vimentin、CD45、CD20、CD79а、Pax5和MUM-1弥漫(+),例1 CD5(+),例2CD5(-),而其他抗体2例均(-)。IgH基因重排可见较强克隆性重排条带。结论梭形细胞变异型非特指性DLBCL非常罕见,需要与多种梭形细胞肿瘤鉴别。准确的诊断需要病理形态学观察与免疫组化和基因重排检测相结合,治疗以放、化疗为主,预后较好。

To investigate the clinical pathological feature and differential diagnosis of variant of spindle cell non-specific diffuse large B cell lymphoma （DLBCL）.Methods We carried out a study of 2 cases of DLBCL on histomorphology,immunohistochemistry,genomic in situ hybridization,gene rearrangement examinations,and literature review.Results 2 cases were both female,who were 15 and 22 year old,respectively.The DLBCL of one case occurred in celiac mesentery and omentum majus,another case of DLBCL occurred in anterior superior mediastinum.Tumor cells under microscope showed granulomatous growth,fascicular and intertwined arrangement,obvious collagen fiber in mesenchyma,less cytoplasm in tumor cells and showed fusiform or ovoid cell nucleus and coarse granular chromatin.Immunohistochemistry examination showed diffuse expression of vimentin,CD45,CD20,CD79a,Pax5; MUM-1 positive; CD5 positive in case one; CD5 negative in case two; other antibody expression was negative.The gene rearrangements of IgH showed visible strong clonal rearrangement bands.Conclusion Variant non-specific diffuse large B cell lymphoma of spindle cell is very rare and needs to differentiate from variety of spindle cell tumors.Accurate diagnosis requires a combination of histomorphology,immunohistochemistry and gene rearrangement examinations.Chemoradiotherapy is the first line treatment option with excellent prognosis.