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目的探讨黏液炎症性纤维母细胞肉瘤(MIFS)的临床病理学特征、免疫学表型及鉴别诊断。方法对2例四肢的MIFS进行光镜观察和免疫组化标记,并文献复习。结果MIFS可发生于任何年龄段,多发于40～50岁,肿块位于肢端皮下组织,境界不清,生长缓慢,偶伴疼痛。镜检:病变呈多结节状,玻璃样和黏液样间质相间的背景中有大量急慢性炎症细胞浸润。病变中肿瘤细胞呈3种典型形态:梭形细胞、神经节样细胞或RS细胞样细胞和脂母细胞样细胞。免疫组化:瘤细胞vimentin、CD34弥漫(+),CD68和Mac387灶性(+),SMA、CK、HHF-35、S-100、CD30和CD15均(-)。结论MIFS是一种罕见的低度恶性软组织肉瘤,瘤细胞由变异的纤维母细胞衍化而来。该瘤在局部呈侵袭性生长,具有较高的复发率,偶有转移。推荐局部扩大切除治疗。
Objective To investigate the clinicopathological, immunological phenotype and differential diagnosis of mucinous inflammatory fibroblastic sarcoma (MIFS). Methods MIFS in 2 limbs were observed with light microscope and immunohistochemically, and the literature review. Results MIFS occurred in any age group, occurred in 40 to 50 years old, the mass located in the extremity subcutaneous tissue, the state is not clear, slow growth, accompanied by pain. Microscopic examination: lesions were nodular, glassy and mucus-like interstitial phase in a large number of acute and chronic inflammatory cell infiltration. Tumor cells showed three typical morphological changes: spindle cells, ganglion-like cells or RS cell-like cells and adipocyte-like cells. Immunohistochemistry: The tumor cells vimentin, CD34 diffuse (+), CD68 and Mac387 foci (+), SMA, CK, HHF-35, S-100, CD30 and CD15 were all (-). Conclusions MIFS is a rare low-grade soft tissue sarcoma derived from variant fibroblasts. The tumor was locally invasive growth, with a high recurrence rate, occasionally transfer. Recommended local expansion of excision treatment.