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。 Objective: To elucidate the pathomorphological features of a rare small cell type of anaplastic large cell lymphoma (ALCL). Methods: One case of small cell type ALCL involving in the bilateral scapular region and involving the bone and surrounding soft tissue was observed by light microscopy, immunohistochemistry and PCR gene analysis. Results: The lesion was mainly small to medium volume of malignant lymphocytes. Localized large anamorphic cells were found. Some of the tumor cells were arranged in a circle around the small blood vessels with more neutrophils and tissue cells in the tumor. Tumor cells express CD30 and EMA and have a T cell phenotype and TCR beta gene rearrangement. Conclusion: This type of ALCL has special histological and invasive biological behavior, and should pay attention to the identification of diseases such as inflammation and eosinophilic granuloma.