【摘要】 6例先天性纤维肉瘤均发现于出生后3个月之内,4例见于初生。4例累及四肢远端,另2例分别位于臀部和枕部。肿瘤大多生长快速,局部侵袭明显,但无转移。组织病理学虽以梭形瘤细胞成束交错或青鱼骨状排列为特征,但常有明显的粘液样基质;血管外皮瘤构型;大量微坏死灶和未分化圆形细胞区等。电镜观察见多数瘤细胞符合胚胎性或未成熟纤维母细胞特征,少数类似组织细胞,本瘤似有向其它间叶细胞转化的倾向。更多还原

【Abstract】 This paper reports 6 casses of congenital fibrosarcoma being presented within the first 3months of life.Four tumors affected the distant portion of the extremities and 2 located in buttocks andocciput respectively.Most of the lesions grew rapidly to large size and showed prominent local aggres-sion,but no eviednce of metastasis.Histologically,the fascicular or“herring-bone”pattern was theprinciple feature of the tumors,but it usually showed prominent myxoid stroma,pericytoma-like pat-tern,micronecrotic foci,and areas of undifferentiated round cells.An electron microscopic examinationdemonstrated a majority of tumor cells showing characteristics of embryonal or immature fibroblastsand some of histiocyte-like cells.It is probable that the undifferentiated tumor cells possess potentials oftransformation into other mesenchymal cell types,compared with adult fibrosarcoma.更多还原