软组织及骨的小细胞型间变性大细胞淋巴瘤1例报道

Small cell variant anaplastic large cell lymphoma: report of one case in soft and bone tissue

目的 :阐述罕见的小细胞型间变性大细胞淋巴瘤 (ALCL)的病理形态学特点。方法 :对 1例发生于双侧肩胛区、累及骨和周围软组织的小细胞型ALCL进行了光镜、免疫组织化学观察和PCR基因分析。结果 :病变以小到中体积的恶性淋巴细胞为主 ,局部可见大的间变性细胞 ,部分肿瘤细胞围绕小血管呈花环状排列 ,肿瘤间有较多的中性粒细胞和组织细胞。肿瘤细胞表达CD30和EMA ,并具有T细胞表型和TCR β基因重排。 结论 :此型ALCL具有特殊的组织学表现和侵袭性的生物学行为 ,应注意与炎症和嗜酸性肉芽肿等疾病进行鉴别。

Purpose To report histopathologic characteristics of small cell variant of anaplastic large cell lymphoma (ALCL), which is a rare subtype of ALCL. Methods One case of small cell variant ALCL involving bone and soft on the bilateral scapular region was observed by light microscopy, immunohistochemical staining and PCR technique. Results In the case histologic sections showed a predominantly small to medium sized lymphocytes and locally some large anaplastic cells, of which some tumor cells mimcked flower petals around the small vessels, with a lot of neutrophils and histocytes. Both the small and large tumor cells expressed CD30 and EMA, and they were of T cell phenotype as well as a clonal TCR beta gene rearrangement. Conclusion The small cell variant ALCL has specific histopathologic appearance and aggressive biological behavior. We suggest that the small variant of ALCL should be differentiated from inflammatory and eosinophilic granuloma.