摘要
目的探讨近端型上皮样肉瘤(PES)的临床病理特点、免疫表型、诊断及鉴别诊断。方法收集5例PES患者的临床资料,观察其组织病理学形态、免疫组化特征并进行随访。结果 5例PES中男性3例,女性2例,发病年龄28～54岁,分别位于头部、臀部、腹股沟、胸壁、胸椎。临床均表现为无痛性生长的结节状肿块。镜下肿瘤由上皮样细胞、梭形细胞混合并相互过渡组成,呈结节状或结节融弥漫合成片状,中央常见坏死;其中2例周边可见肉芽肿性改变。细胞间见明显的胶原纤维沉积。细胞呈上皮样或梭形,胞质丰富,嗜酸性;细胞核卵圆形、空泡状,核仁明显,一个或多个,核分裂象多见,常见横纹肌样细胞。免疫表型:5例瘤细胞vimentin、CKpan、EMA和β-catenin(+),3例CD34(+),1例desmin(+),2例CD99(+),而S-100、HMB45、melanA、SMA、myogenin、CD31、FⅧ、HHF35和bcl-2均(-)。5例PES均行肿物局部切除或扩大切除,其中3例辅以放疗或化疗,5年的随访中均有复发和转移。结论 PES因肿瘤组织主要由上皮样细胞、横纹肌样细胞及梭形细胞构成,形态复杂多样,极易与具有类似形态的多种肿瘤混淆,故其鉴别诊断显得尤为重要。
Objective To investigate the clinicopathological features,immunophenotype and differential diagnosis of proximal-type epithelioid sarcoma（PES）.Methods The pathologic and immunophenotypic features were collected and analyzed with follow-up in 5 cases of PES.Results Three of the patients were male and another two were female.The age of patients ranged from 28 to 54 years.The 5 tumors located at head,buttock,groin,chest wall and dorsal vertebrae,respectively.Clinically,the tumors were painless firm and subcutaneous nodules.Under the microscope,the tumors were composed of epithelioid cells,spindle cells or transitional cells between each other.The tumor cells arranged in nodular,diffusely nodular or sheet patterns.There was always necrosis at the centre and 2 cases showed granulomatous change around or increased collagen deposition between trabeculae of tumor cells.Cells were mainly epithelioid or spindle,with abundant and eosinophilic cytoplasm;nucleus appeared oval and vesicular,with one or more prominent nucleoli and mitosis was common.Rhabdoid cells were observed frequently.Immunophenotyping showed that tumor cells were positive for vimentin,pan-CK,EMA and β-catenin.CD34 was positive in 3 cases,positive CD99 in two cases and positive desmin in one case.S-100,HMB45,Melan A,SMA,myogenin,CD31,FⅧ,HHF35 and bcl-2 all were negative.As for follow-up,all 5 patients underwent tumor resection or extended excision,and 3 patients with radiotherapy or chemotherapy.For those 2 cases without adjuvant therapy,1 case appeared metastasis after 2 months,another case had multiple recurrence within 5 years and widespread metastases after 5 years.For those 3 cases with adjuvant radiotherapy or chemotherapy,all showed recurrence and metastasis within 9 months to 2 years.Conclusions PES can be distinguished according to its clinical and morphologic features,combined with the results of immunohistochemical staining.The tumor is composed of epithelioid cells,rhabdoid cells as well as spindle cells and demonstrates combined forms or complex and diverse patterns,which are easily confused with other tumors,so that the differential diagnosis is very important.
出处
《诊断病理学杂志》
CSCD
2011年第2期94-97,共4页
Chinese Journal of Diagnostic Pathology
关键词
上皮样肉瘤
近端型
免疫组化
鉴别诊断
Epithelioid sarcoma
Proximal type
Immunohistochemistry
Differential diagnosis
