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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。
Objective To analyze the clinicopathological features of undifferentiated embryonal sarcoma in the elderly and to discuss its diagnosis and differential diagnosis. Methods One case of 72-year-old female patients with undifferentiated embryonal sarcoma was imaged, HE and immunohistochemical staining, and reviewed the relevant literature. Results The elderly undifferentiated embryonal sarcoma was more common in women. The tumors were mostly located in the right lobe of the liver with a diameter of 10-30 cm. The sections were gray and jelly-like, accompanied by hemorrhage and cystic degeneration. Microscopic examination revealed mucoid matrix scattered undifferentiated spindle or astrocytic cells, between the shape of weird, larger pleomorphic cells or multinucleated giant cells, tumor cells can be seen in the cytoplasm of PAS-positive eosinophilic transparent bodies. Immunohistochemistry: tumor cells vimentin and a l-antitrypsin diffuse (+). Conclusions The occurrence of undifferentiated embryonal sarcoma in the elderly is extremely rare and the diagnosis depends on histomorphology and immunohistochemical markers. Surgical resection of the main treatment can be supplemented with chemotherapy, but the prognosis is poor.