上皮样肉瘤6例临床病理分析

Epithelioid sarcoma:a clinicopathologic analysis of 6 cases

目的:探讨上皮样肉瘤的临床病理特征和鉴别诊断。方法:分析6例上皮样肉瘤的临床病理特征并进行文献复习。结果:6例均为手术切除标本,镜下均呈特征性结节性生长,肿瘤细胞有两种形态:一类为大的多角形或卵圆形细胞,胞质丰富,嗜伊红,类似上皮样细胞;另一类为梭形细胞,丰满似纤维母细胞样,两种细胞逐渐移行。免疫表型:6例瘤细胞vimentin、pan-CK、EMA(+),2例CD34(+),1例S-100(+)。结论:上皮样肉瘤是一种少见的独特的病变,典型病例诊断不难。对取材较少或镜下表现不典型,尤其是结节中心坏死较小甚至无坏死时,易与滑膜肉瘤、上皮样血管内皮瘤、上皮样恶性神经鞘瘤等混淆,需综合临床资料、镜下特征和免疫组化等特点进行鉴别。

Objective To study clinicalpathologic characteristics,diagnostic criteria and differential diagnosis of epithelioid sarcoma.Methods Clinical pathologic features were studied in 6 cases of epithelioid sarcoma with review of the literatures.Results 6 cases were surgical-removed specimens,the tumors showed a markedly nodular mass.Two kinds of tumour cells were identified：one was large polygonal orovate cell with abundant eosinophilic cytoplasm,similar to epithelioid cell,and another spindle cell,or fibroblast-like cell.The two kinds of cells had gradual transition.Immunophenolyping showed that tumor cells were the positive for vimentin,pan-Ck and EMA,CD34 was positive in 3 cases,S-100 was positive in 1 case.Conclusions Epithelioid sarcoma is a rare tumor of soft tissue.It is not difficult to diagnose the tumor with typicalmorphology,but for small biopsy or those with atypical histologic and immunohistochemical features,especially with less or without central necrosis in the neoplastic nodules,are easy to be confused with synovial sarcoma,epithelioid hemangioendothelioma,epithelioid malignant peripheral nerve sheath tumor and malignant melanoma,and diagnosis can be made with caution by combination of clinical data,microscopic characteristics and immunohistochemical findings.