Watanabe Akira, Enokida Yasuaki, Suzuki Masaki, Takada Takahiro, Yoshida Tomonori, Imaizumi Jun, Sohda Makoto, Iijima Misa, Ojima Hitoshi, Shirabe Ken
Department of Gastroenterological Surgery, Gunma Prefectural Cancer Center, Ohta, Gunma, Japan.
Department of Pathology, Gunma Prefectural Cancer Center, Ohta, Gunma, Japan.
Surg Case Rep. 2025;11(1). doi: 10.70352/scrj.cr.25-0200. Epub 2025 Jul 29.
Undifferentiated embryonal sarcoma (UES) of the liver (UESL) is a tumor that is rare in adults. UESL is composed of undifferentiated mesenchymal tumor cells, with characteristic normal bile duct cells found at the tumor periphery. We present a rare case of UESL containing an adenocarcinoma component in one part of the tumor.
A 68-year-old male had a tumor with an 8-cm diameter in the left lateral liver segment. CT showed a cystic tumor with a solid component and a heterogeneous area. In the solid component, the contrast effect was weak in the arterial phase and uniform in the late phase. Imaging suggested that the internal tumor area was necrotic or hemorrhagic. We diagnosed it as hemorrhagic hepatocellular carcinoma or combined hepatocellular-cholangiocellular carcinoma. The patient exhibited normal liver function. Left lateral segmentectomy was performed. Macroscopically, the tumor was cystic, with hemorrhagic/necrotic deposits and a solid portion composed of tumor cells. Histopathologically, the tumor had 2 components. One was a nonepithelial tumor comprising immature spindle-shaped and stellate plaques, with positivity for the mesenchymal markers vimentin and CD68 and negativity for myogenic, neurogenic, and other markers. This component was diagnosed as UES. The other component comprised epithelial and adenocarcinoma tissues with differentiated bile duct cells. The tumor was mainly composed of UES cells, with the adenocarcinoma component located predominantly in the periphery.
We present a unique case of UESL containing UES cells and adenocarcinoma cells at the periphery. A combination of UESL and adenocarcinoma is extremely rare, even among hepatic carcinosarcomas.
肝未分化胚胎性肉瘤（UESL）是一种在成人中罕见的肿瘤。UESL由未分化的间充质肿瘤细胞组成，在肿瘤周边可见特征性的正常胆管细胞。我们报告一例罕见的UESL病例，该肿瘤的一部分含有腺癌成分。
一名68岁男性在肝左外叶有一个直径8厘米的肿瘤。CT显示为一个有实性成分和不均匀区域的囊性肿瘤。在实性成分中，动脉期造影效果较弱，延迟期均匀。影像学提示肿瘤内部区域为坏死或出血性。我们诊断为出血性肝细胞癌或肝细胞-胆管细胞癌合并。患者肝功能正常。进行了左外叶切除术。大体上，肿瘤为囊性，有出血/坏死灶以及由肿瘤细胞组成的实性部分。组织病理学检查显示，肿瘤有两个成分。一个是非上皮性肿瘤，由未成熟的梭形和星状细胞组成，间充质标志物波形蛋白和CD68呈阳性，肌源性、神经源性和其他标志物呈阴性。该成分被诊断为UES。另一个成分包括上皮和腺癌组织，有分化的胆管细胞。肿瘤主要由UES细胞组成，腺癌成分主要位于周边。
我们报告了一例独特的UESL病例，其周边含有UES细胞和腺癌细胞。UESL与腺癌的组合极为罕见，即使在肝肉瘤样癌中也是如此。
