Diedhiou Abdoulaye, Genin Pascal, Terris Benoît, Sauvanet Alain, Belghiti Jacques, Degott Claude
Service d'Anatomie Pathologique, 100 Blvd. du General Leclerc, 92118, Clichy Cedex, France.
Ann Pathol. 2002 Apr;22(2):134-6.
Undifferentiated embryonal sarcoma of the liver is rare. It is usually observed in children and adolescents. We report one case of embryonal sarcoma of the liver arising in patient without any antecedent. The only symptom was right scapular pain. The liver scan showed a multicystic lesion suspicious for infectious origin or a tumor. Serologies for ecchinococcus, schistosomiasis and brucellosis were negative. The treatment was a right hepatectomy. On gross examination, the tumor was unencapsulated, multicystic and contained large areas of necrosis admixed with gelatinous areas. Microscopically, there were epithelioid and spindle tumor cells in a myxoïd stroma. Lipoblastic-like or rhabdomyoblastic-like, giant cells and PAS positive hyaline globules in the cell cytoplasm were present. The tumor cells expressed vimentin, cytokeratin (KL1), alpha-1-antitrypsin and smooth muscle actin. This observation shows that embryonal sarcoma of the liver may develop in adult patients and should be taken into consideration in any differential diagnosis of cystic hepatic tumor.
肝脏未分化胚胎性肉瘤较为罕见。它通常见于儿童和青少年。我们报告一例无任何既往病史的患者发生肝脏胚胎性肉瘤的病例。唯一症状是右肩胛疼痛。肝脏扫描显示一个多囊肿块,怀疑为感染性病因或肿瘤。棘球蚴病、血吸虫病和布鲁氏菌病的血清学检查均为阴性。治疗方法是右半肝切除术。大体检查时,肿瘤无包膜,呈多囊性,包含大片坏死区域并混有胶冻样区域。显微镜下,在黏液样基质中有上皮样和梭形肿瘤细胞。细胞胞质中存在脂母细胞样或横纹肌母细胞样、巨细胞以及PAS阳性透明小球。肿瘤细胞表达波形蛋白、细胞角蛋白(KL1)、α1抗胰蛋白酶和平滑肌肌动蛋白。该病例表明肝脏胚胎性肉瘤可发生于成年患者,在对囊性肝肿瘤进行任何鉴别诊断时均应予以考虑。