去分化脂肪肉瘤（dedifferentiated liposarcoma，DDLPS）有很多形态学生长模式，其组织学可与其他梭形细胞间叶性肿瘤形态类似已被广泛认知。但伴上皮样形态的DDLPS却罕见报道。这里，我们报道6例伴有显著上皮样/上皮特征的DDLPS。患者男性5例，女性1例，中位年龄61岁。所有病例均发生于躯干深部。随访时间1~41个月，4例局部复发，2例远处转移，4例因肿瘤死亡。肿瘤除了有高分化脂肪肉瘤成分、传统DDLPS的高级别梭形细胞形态，所有病例均局灶出现小的或者大的上皮样细胞弥漫或片状生长。2例出现横纹肌样细胞。5例做了MDM2基因检测，均显示扩增。5例行免疫组化检查，细胞角蛋白和/或上皮膜抗原均见至少局灶阳性。有1例局灶出现未见报道的异源性上皮分化，表现为腺泡状结构、免疫组化细胞角蛋白、MOC31、Claudin-4弥漫阳性，同时H3K27me3表达缺失。伴有上皮样/上皮特征的DDLPS易误诊为癌或间皮肉瘤，需要基于临床病理特征和分子检测结果的综合评判，再作出病理诊断。本小组病例显示，伴有上皮样特征的DDLPS预示侵袭性生物学行为。此外，本文还报道了2例发生于躯干深部伴有上皮样特征的MDM2基因扩增的未分化肿瘤，但组织学上与高分化脂肪肉瘤无关，临床病程进展迅速，此2例是否属于伴有上皮样/上皮特征的DDLPS还有待进一步研究。
Am J Surg Pathol 2017;41:1523–1531
美国外科病理学杂志中文版2018年第一期摘要No.7
（贡其星 翻译/审校）
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