Ikeda Masaki, Suenaga Masaya, Sugiyama Keiji, Iwakoshi Akari, Shiraishi Kazuhiro, Tashiro Mitsuru, Hattori Masashi, Murayama Mutsumi, Hirashima Noboru, Nishimura Rieko, Shimada Masaaki, Kitagawa Chiyoe, Takeda Shin, Kataoka Masato
Dept. of Surgery, National Hospital Organization Nagoya Medical Center.
Gan To Kagaku Ryoho. 2024 Jul;51(7):771-773.
Undifferentiated sarcoma of the liver is rare, especially in adults, and is an aggressive malignancy that originates from the primary mesenchymal tissues. A 53-year-old man was referred to our hospital for further evaluation of a low-grade fever. Contrast-enhanced CT revealed an 18-cm tumor in the right lobe of the liver. The tumor was characterized by low-density areas suspected of cystic components, a high-density area suspected of hemorrhage, and contrast enhancement in the thickened marginal and internal septa. MRI revealed a high-intensity tumor with a heterogeneous structure on T2-weighted images. Angiosarcoma of the liver with intratumoral hemorrhage was suspected, and right hepatectomy was performed. The pathological diagnosis was an undifferentiated sarcoma based on the presence of undifferentiated mesenchymal tumor cells with a stellate to spindle-shaped pleomorphism. Following a multidisciplinary discussion, 4 courses of the AI regimen (doxorubicin and ifosfamide)were administered as adjuvant chemotherapy, and no recurrence was confirmed at 2 years and 6 months follow-up. Our case suggests that radical resection followed by adjuvant chemotherapy may contribute to a favorable prognosis for undifferentiated sarcoma of the liver.
肝未分化肉瘤罕见，尤其在成人中，是一种起源于原始间充质组织的侵袭性恶性肿瘤。一名53岁男性因低热被转诊至我院进一步评估。增强CT显示肝脏右叶有一个18厘米的肿瘤。该肿瘤的特征为存在疑似囊性成分的低密度区、疑似出血的高密度区，以及增厚的边缘和内部间隔有强化。MRI显示在T2加权图像上有一个结构不均一的高强度肿瘤。怀疑是伴有肿瘤内出血的肝血管肉瘤，遂行右肝切除术。病理诊断为未分化肉瘤，依据是存在具有星状至梭形多形性的未分化间充质肿瘤细胞。经过多学科讨论，给予4个疗程的AI方案（多柔比星和异环磷酰胺）辅助化疗，在2年6个月的随访中未证实有复发。我们的病例表明，根治性切除后辅助化疗可能有助于肝未分化肉瘤获得良好预后。
