肝未分化(胚胎性)肉瘤的临床病理分析

Undifferentiated(embryonal) sarcoma of the liver

目的 :探讨肝未分化 (胚胎性 )肉瘤的临床病理特征及其鉴别诊断。方法 :对 2例肝未分化 (胚胎性 )肉瘤标本通过光镜、电镜及免疫组织化学等方法进行病理组织学观察。结果 :组织学特征为 :肿瘤与正常肝组织之间隔以纤维性假包膜 ;肿瘤细胞大小不等 ,形态多样 ,呈星芒状和 (或 )梭形 ,细胞界限不清 ,有明显的黏液样背景 ;在肿瘤外周可见卷入的胆管样结构 ;瘤细胞内外可见一些PAS阳性的嗜酸性球形小体 ;有明显的出血、坏死及囊性变、免疫组化及超微结构没有明显的形态学分化特征。结论 :肝未分化 (胚胎性 )肉瘤的组织发生仍然不清楚 ,免疫组化和电镜研究支持是间叶起源 ,但没有一定的分化模式 ,是一种罕见的肝原发肿瘤 ,根据其组织学和病理形态学特征 ,结合腹部包块、CT等临床表现 。

Purpose To investigate the clinicopathological characteristics of primary hepatic undifferentiated (embryonal) sarcoma and its differential diagnosis from other primary malignant tumors of liver. Methods Two cases of primary hepatic undifferentiated (embryonal) sarcoma were studied by light microscopy, electron microscopic examination and immunohistochemical analysis. Results The histological characteristics showed a fibrous pseudocapsule separated the normal liver tissue from tumor cells. The tumor cells varied in size and shape and had vague boundary,showed spindle cell and/or stellate cells with prominent myxoid background. The peripheral area of tumor was surrounded by entrapped bile duct like structures. Some PAS positive eosinophilous globules were found within and adjacent to tumor cells. Areas of necrosis, hemorrhage and cystic degeneration were prominent. Immunohistochemical and ultrastructureal study showed no evidence of the histological differentiate characteristic of the tumor. Conclusions The histogenesis of the undifferentiated (embryonal) sarcoma of the liver is still unclear. Immunohistochemical and electron microscopic studies support a mesenchymal origin,but do not reveal a consistent pattern of differentiation. It was rare of primary hepatic neoplasm. By histopathological characteristics and clinical manifestations such as tumor in abdomen,CT,etc,a definite pathologcal diagnosis of the tumor can be made.