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[一例成人中酷似肝门部胆管癌的未分化(胚胎性)肝肉瘤病例]

[A case of undifferentiated (embryonal) liver sarcoma mimicking klatskin tumor in an adult].

作者信息

Lee Ji Ae, Kim Tae Wan, Min Jae Hoon, Byon Sun Jung, Jang Seung Hoon, Choi Seung Yeon, Kim Hong Joo

机构信息

Department of Internal Medicine, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea.

出版信息

Korean J Gastroenterol. 2010 Feb;55(2):144-8. doi: 10.4166/kjg.2010.55.2.144.

Abstract

Undifferentiated sarcoma is an uncommon primary malignant tumor of the liver typically occurring in older children. It is also referred to as malignant mesenchymoma, fibromyxosarcoma, or mesenchymal sarcoma. We experienced a case of undifferentiated sarcoma in 72-year-old male. Contrast enhanced liver CT scan revealed a 3.4 cm heterogeneously enhancing, ill-defined, and low attenuated mass in the left liver and subtle intrahepatic duct dilatation. And, in tubogram, there were segmental stenosis and occlusion from the hilum to the proximal common bile duct. We did ultrasonography guided liver biopsy. The pathologic finding revealed infiltrative growth of atypical cells with rhabdoid features. Some atypical cells showed clear cytoplasm, but no organoid pattern was identified. The stroma around atypical cells was filled with eosinophilic hyaline material. These tumor cells were positive for vimentin only, and the tumor was consistent with undifferentiated sarcoma of the liver.

摘要

未分化肉瘤是一种罕见的肝脏原发性恶性肿瘤,通常发生于大龄儿童。它也被称为恶性间叶瘤、纤维黏液肉瘤或间叶肉瘤。我们遇到一例72岁男性的未分化肉瘤病例。肝脏CT增强扫描显示左肝有一个3.4厘米大小的肿块,强化不均匀、边界不清且呈低密度,同时肝内胆管有轻微扩张。并且,在胆管造影中,从肝门到近端胆总管存在节段性狭窄和闭塞。我们进行了超声引导下肝脏活检。病理结果显示具有横纹肌样特征的非典型细胞呈浸润性生长。一些非典型细胞胞质清亮,但未发现器官样结构。非典型细胞周围的间质充满嗜酸性透明物质。这些肿瘤细胞仅波形蛋白呈阳性,该肿瘤符合肝脏未分化肉瘤。

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