皮肤原发性上皮样肉瘤病理及临床的误诊探讨(1)
【摘要】目的:探讨皮肤上皮样肉瘤的临床病理特点，免疫组化表型及生物学行为。方法:收集5例皮肤ES患者的资料，运用显微镜观察、免疫组化等方法观察和分析其临床、细胞学涂片和病理学组织学特征。结果:本组肿瘤全部原发于皮肤，肿瘤呈结节状浸润性生长，瘤细胞又由两种细胞组成，大部分为多角形或卵圆形，胞浆丰富，嗜酸性，少部分呈胖梭形，象纤维母细胞样，常围绕在结节周围，结节中央常有坏死。免疫组化示：上皮膜抗原(EMA)，细胞角蛋白(CK)，波形蛋白(Vimentin)均强阳性，CD34 及CD68弱阳性，其余多项免疫组化结果均呈阴性。结论:ES少见且形态复杂多变，只凭光镜容易误诊，肿瘤细胞学对其诊断无特异性，需结合免疫组化结果才能做出正确的诊断。ES预后差，患者常需行肿物扩大甚至根治术。
【关键词】皮肤肿瘤；上皮样肉瘤；免疫组化
A Clinicopathologic misdiagnosis Study of Primary Epithelioid Sarcoma of Skina
, 百拇医药
Zhao Jun Sun Qian Wang xue li
【Abstract】Objective To explore the pathoclinical characteristics immunophonetype and its biological behaviour of primary epithelial sarcoma.Methods Five cases of primary epithelial sarcoma of skin were studied by using microscope observation and immunohistochemistry and analyse their characterstics of clinic、cytological smear and pathohistology.Results The group of tumors were all located in skin.Microscopically，tumor showed an invasive growth and nodulous arrangement and was made up of two kinds of cells，relativepart of cells were characterized by multangular or oval and round cell with richful eosinophilic cytoplasm，other cells were fatty and spindle just like fibroblast.The tumor nodule's central zone that was always degenerative and necrosive that surrounded by the two kinds cells.Immunohistochemically，tumor cells showedintense positive staining for EMA、Cytokeratin and Vimentin，only showed weak positive staining for CD34and CD68，other results of immnuohistochemistry were all negative.ConclusionThe tumor is quite difficult to diagnose only by microscope for its infrequency and various morphological features.No diagnostic particularity only bycelluar smear.Only based on histopathological characteristics with the results of immunohistochemistry，a definite pathological diagnosis of the tumor can be got.Its prognosis is very poor and a patient needs to have a marginal or even a radical resection.
, 百拇医药
【Key words】Skin cancer；Epithelioid sarcoma；Immunohistochemistry
【中图分类号】R730.26【文献标识码】A【文章编号】1008-6455(2011)08-0011-02
上皮样肉瘤(epithelioid sarcome，ES)是一种表现为缓慢生长的皮内或皮下结节，是少见的起源未定的肿瘤，临床和病理医师都很容易误诊，该肿瘤预后较差，多需广泛切除。我院自1995年～2008年期间共收治5例经临床确诊的病人，结合病理形态特点、穿刺细胞学和免疫组化技术以及生物学行为对此病进行探讨，现报告如下。
1 方法
5例HE染色并行即用型二步法免疫组化检查，所用EMA、Vimetin、CK、CD68、CD34、 HMB45等试剂均购自广州中山生物技术有限公司，均设阳性对照，并用PBS代替一抗作为阴性对照。
, 百拇医药
2 结果
2.1 大体观察:4例均为带皮肤组织一块，另一例未见皮肤，呈结节状或不整组织，皮肤中间可见浅溃疡，3例溃疡表面有脓性分泌物，切面呈灰白、灰红色，一例可见灶性灰黄色坏死区，均未见明显包膜，界限不清。
2.2 细胞学穿刺镜检:穿刺涂片见呈聚集的梭形细胞，部分细胞核较大，含颗粒状的染色质、胞浆略嗜伊红，还可见核偏位的蝌蚪形的细胞。
2.3 组织学镜检:肿瘤组织均位于皮下至真皮深部，上皮样瘤细胞作结节状排列，界限不甚清晰，3例镜下结节中央呈退变及坏死，坏死灶呈“地图样”，另2例合并出血和囊性变，周围瘤细胞呈多边形，肥胖梭形，胞浆深伊红色或淡染。此3例肿瘤细胞呈梭形，胞体比较肥胖，少量上皮样瘤细胞作结节状排列并沿着神经血管束方向生长。另2例肿瘤均由两种细胞构成，结节中间部分细胞为比较大的类似上皮样细胞的圆形或多角性的细胞，细胞排列密切，核仁清晰，异型性明显，胞浆丰富呈嗜酸性；结节周围可见另一种梭形细胞成分，细胞肥胖，似纤维母细胞，两种肿瘤细胞之间无明显界限，核分裂象常少见(<5个/10HPF)。
, 百拇医药
2.4 免疫组化:肿瘤细胞CK、Vimetin及EMA均弥漫(++)，CD68弥漫弱(+)，CD34弱(+)，其余鉴别诊断平滑肌、神经等标记物均阴性。
3 讨论
3.1 组织发生:ES的组织来源至今未定，现普遍认为肿瘤来源于原始的间叶组织，具有多项分化的功能[1]，结合以上5例免疫组化结果，肿瘤细胞表达CK、EMA、Vimetin弥漫阳性，CD68弥漫弱阳性，CD34弱阳性这些结果支持这一关观点，本组结果均显示：在同一病例中，部分区域以上皮样分化为主，另外区域以梭形的间叶细胞分化为主，两种细胞形态之间无明显过渡，以上皮样分化的肿瘤组织部分CK、EMA染色(++)，梭形间叶分化成分EMA染色(++)，CD68(+)这样可以提示肿瘤的分化趋势。, 百拇医药(赵军 孙倩 王雪利)
【关键词】皮肤肿瘤；上皮样肉瘤；免疫组化
A Clinicopathologic misdiagnosis Study of Primary Epithelioid Sarcoma of Skina
, 百拇医药
Zhao Jun Sun Qian Wang xue li
【Abstract】Objective To explore the pathoclinical characteristics immunophonetype and its biological behaviour of primary epithelial sarcoma.Methods Five cases of primary epithelial sarcoma of skin were studied by using microscope observation and immunohistochemistry and analyse their characterstics of clinic、cytological smear and pathohistology.Results The group of tumors were all located in skin.Microscopically，tumor showed an invasive growth and nodulous arrangement and was made up of two kinds of cells，relativepart of cells were characterized by multangular or oval and round cell with richful eosinophilic cytoplasm，other cells were fatty and spindle just like fibroblast.The tumor nodule's central zone that was always degenerative and necrosive that surrounded by the two kinds cells.Immunohistochemically，tumor cells showedintense positive staining for EMA、Cytokeratin and Vimentin，only showed weak positive staining for CD34and CD68，other results of immnuohistochemistry were all negative.ConclusionThe tumor is quite difficult to diagnose only by microscope for its infrequency and various morphological features.No diagnostic particularity only bycelluar smear.Only based on histopathological characteristics with the results of immunohistochemistry，a definite pathological diagnosis of the tumor can be got.Its prognosis is very poor and a patient needs to have a marginal or even a radical resection.
, 百拇医药
【Key words】Skin cancer；Epithelioid sarcoma；Immunohistochemistry
【中图分类号】R730.26【文献标识码】A【文章编号】1008-6455(2011)08-0011-02
上皮样肉瘤(epithelioid sarcome，ES)是一种表现为缓慢生长的皮内或皮下结节，是少见的起源未定的肿瘤，临床和病理医师都很容易误诊，该肿瘤预后较差，多需广泛切除。我院自1995年～2008年期间共收治5例经临床确诊的病人，结合病理形态特点、穿刺细胞学和免疫组化技术以及生物学行为对此病进行探讨，现报告如下。
1 方法
5例HE染色并行即用型二步法免疫组化检查，所用EMA、Vimetin、CK、CD68、CD34、 HMB45等试剂均购自广州中山生物技术有限公司，均设阳性对照，并用PBS代替一抗作为阴性对照。
, 百拇医药
2 结果
2.1 大体观察:4例均为带皮肤组织一块，另一例未见皮肤，呈结节状或不整组织，皮肤中间可见浅溃疡，3例溃疡表面有脓性分泌物，切面呈灰白、灰红色，一例可见灶性灰黄色坏死区，均未见明显包膜，界限不清。
2.2 细胞学穿刺镜检:穿刺涂片见呈聚集的梭形细胞，部分细胞核较大，含颗粒状的染色质、胞浆略嗜伊红，还可见核偏位的蝌蚪形的细胞。
2.3 组织学镜检:肿瘤组织均位于皮下至真皮深部，上皮样瘤细胞作结节状排列，界限不甚清晰，3例镜下结节中央呈退变及坏死，坏死灶呈“地图样”，另2例合并出血和囊性变，周围瘤细胞呈多边形，肥胖梭形，胞浆深伊红色或淡染。此3例肿瘤细胞呈梭形，胞体比较肥胖，少量上皮样瘤细胞作结节状排列并沿着神经血管束方向生长。另2例肿瘤均由两种细胞构成，结节中间部分细胞为比较大的类似上皮样细胞的圆形或多角性的细胞，细胞排列密切，核仁清晰，异型性明显，胞浆丰富呈嗜酸性；结节周围可见另一种梭形细胞成分，细胞肥胖，似纤维母细胞，两种肿瘤细胞之间无明显界限，核分裂象常少见(<5个/10HPF)。
, 百拇医药
2.4 免疫组化:肿瘤细胞CK、Vimetin及EMA均弥漫(++)，CD68弥漫弱(+)，CD34弱(+)，其余鉴别诊断平滑肌、神经等标记物均阴性。
3 讨论
3.1 组织发生:ES的组织来源至今未定，现普遍认为肿瘤来源于原始的间叶组织，具有多项分化的功能[1]，结合以上5例免疫组化结果，肿瘤细胞表达CK、EMA、Vimetin弥漫阳性，CD68弥漫弱阳性，CD34弱阳性这些结果支持这一关观点，本组结果均显示：在同一病例中，部分区域以上皮样分化为主，另外区域以梭形的间叶细胞分化为主，两种细胞形态之间无明显过渡，以上皮样分化的肿瘤组织部分CK、EMA染色(++)，梭形间叶分化成分EMA染色(++)，CD68(+)这样可以提示肿瘤的分化趋势。, 百拇医药(赵军 孙倩 王雪利)