【摘要】 目的分析老年人肝未分化胚胎性肉瘤的临床病理学特点,探讨其诊断和鉴别诊断。方法对1例72岁女性患者肝未分化胚胎性肉瘤进行影像学、HE及免疫组化染色分析,并复习相关文献。结果老年人肝未分化胚胎性肉瘤以女性多见,肿瘤多位于肝右叶,直径10~30 cm,切面灰白色、胶冻样,伴有出血、囊性变。镜检示黏液基质中散在分布未分化梭形或星形细胞,间有形态怪异、体积较大的多形细胞或多核巨细胞,肿瘤细胞胞质内可见PAS阳性嗜酸性透明小体。免疫组化:瘤细胞vimentin和a l-抗胰蛋白酶弥漫(+)。结论发生于老年人的肝未分化胚胎性肉瘤极为罕见,确定诊断依靠组织形态学和免疫组化标记。治疗以手术切除为主,可辅以化疗,但预后极差。更多还原

【Abstract】 Objective To analyze the clinicopathological features of undifferentiated embryonal sarcoma of liver( UES)in old-aged adults and to investigate the diagnosis,differential diagnosis,therapy and prognosis. Methods A case of undifferentiated embryonal sarcoma of liver in an old female was analyzed by studying clinical history,imageology,HE and immunohistochemical stains and the related literatures were reviewed. Results Of UESs in the old-aged adults,most patients were females. More than half of the tumours were located in the right liver lobe. The size of the tumours was 10 cm to 30 cm. The cut surface revealed a white,gelatinous soft mass with massive necrosis,hemorrhage and always showed cystic degeneration. Histologically,the mass consisted primarily of loose myxoid stroma admixed with undifferentiated spindle,stellated cells and markedly atypical cells( including multi-nucleated forms) were also present. Some tumor cells contained eosinophilic hyaline globules that were diastase resistant and periodic acid-Schiff positive.Immunohistochemically,the tumor cells showed diffusely positive staining for vimentin and alpha-1-antitrypsin.Conclusions Undifferentiated( embryonal) sarcoma of the liver is a rare pediatric liver malignancy that is extremely uncommon in elderly. Definite diagnosis depends upon histomorphological findings and immunohistochemical staining.Surgical resection combined with postoperative chemotherapy is the main treatment method. The prognosis is very poor.更多还原