近端型上皮样肉瘤3例临床病理分析  

Proximal-type epithelioid sarcoma:A clinicopathological analysis of 3 cases

作　　者：范宜娟[1] 杨滢瑜[1] 陈荣[1] 章林培[1] 

机构地区：[1]上海复旦大学附属华山医院宝山分院病理科,上海2000431

出　　处：《现代肿瘤医学》2009年第5期938-941,共4页Journal of Modern Oncology

摘　　要：目的:探讨近端型上皮样肉瘤的临床病理特征、诊断要点、免疫表型、鉴别诊断及生物学行为。方法:分析3例上皮样肉瘤的临床病理资料,观察其组织病理学形态及免疫表型特征,对其进行随访。结果:女2例,男1例。发病年龄24-34岁。位于头部2例,肩部1例。临床均表现为无痛性生长的单一结节状肿块。肿瘤细胞呈圆形、卵圆形及梭型,含有均质状嗜酸性胞质,细胞核空泡状,核仁明显,一个或多个,见肉芽肿样改变及出血囊性变。肿瘤细胞波形蛋白(Vimentin)、细胞角蛋白(AE1/AE3)、CD34阳性表达,上皮膜抗原(EMA)部分阳性表达。阴性表达的是CD31、FⅧ因子、Mac387、CD68、S-100蛋白、SMA、HMB-45、desmin、CD240。1例死于发病后14个月,1例于发病6个月后出现复发,另1例随访个5月无复发和转移。结论:近端型上皮样肉瘤的组织病理学特征是:肿瘤细胞呈结节状分布,有上皮样结构,肿瘤细胞有弥漫性嗜酸性胞质,核大,空泡状,核仁明显,核分裂相多见。瘤细胞共同表达Vimentin、AE1/AE3、CD34,部分病例瘤细胞表达EMA;需与上皮样血管肉瘤、滑膜肉瘤、上皮样恶性神经鞘瘤鉴别诊断。其生物学行为高度恶性。Objective ：To investigate the pathological charcateristics,clinical and differential dignosis of proximal - type epithelioid sarcoma. Methods： Three cases of proximal - type epithelioid sarcoma were studied with the pathologic and immunophenotypic features and all the cases were followed up. Results： Two of the patients were female and 1 was male. The age of the patients ranged from 24 to 33. Two cases tumor was on the head , another on shoulder. The tumors were painless subcutaneous growing solitary nodule. The tumor cells were round or ovoid, containing eosinophilic cytoplasm. The nucleus was obvious one or two nucleoli. Mitotis figures were showed. The tumor ceils stained positively for vimentin, AE1/AE3, CD34, EMA,while negatively for CD31, FⅧ, Mac387, CD68, S - 100 protein, SMA,CD240, HMB - 45 and desmin. One patient died after fourteen months, one patient was found local recurrence after six months, and one patient was still alive with no local recurrence and mecastatsis for five months. Conclusion：The histopathological characteristics of proximal - type epithelioid sarcoma are as follows ： the tumor cells show diffuse and densely eosinophilic cytoplasm, enlarged vesicular nuclei and prominent nucleoli. All the tumor cells express vimentin and AE1/AE3 and CD34. The tumor cells express EMA in part of the cases. More attention should be paid to differentiate this tumor from epithelioid angiosarcoma synovial sarcoma, epithelioid malignant peripheral nerve sheath tumor, synovial sarcoma, malignant rhabdoid tumor. The biological behavior of epithelioid sarcoma is highly maliganante.

关 键 词：软组织肿瘤 上皮样肉瘤 免疫组织化学 

分 类 号：R730.61[医药卫生—肿瘤]