Minami Koji, Sakoda Masahiko, Iino Satoshi, Hiwatashi Kiyokazu, Maemura Kosei, Mataki Yuko, Kurahara Hiroshi, Kawasaki Yota, Tsuruda Yusuke, Setoyama Tetsuro, Okumura Hiroshi, Maenohara Shigeho, Natsugoe Shoji
Dept. of Surgery, Kagoshima Kouseiren Hospital.
Gan To Kagaku Ryoho. 2018 Apr;45(4):721-724.
A 24-year-old woman was admitted to the hospital for abdominal pain. Abdominal contrast-enhanced computed tomography( CT)revealed a cystic mass measuring 11×8 cm in the left lobe of the liver with extravasation. Vascular embolization was performed, but extravasation remained on CT images. She was then transferred to our hospital. We performed an emergency extended left hepatectomy. Histopathological examination revealed solid proliferation of spindle-shaped cells. Immunohistochemical staining showed that the tumor cells were positive for vimentin and negative for AE1/AE3. Thus, a diagnosis of undifferentiated sarcoma was confirmed. Multiple recurrent tumors were recognized on CT images of the lung and right atrium taken 1 year and 10 months post-surgery. Partial resection of the tumor was performed for the right atrial mass, the left lingular segment, the left inferior lobe, and the right middle lobe. Pathological diagnosis confirmed metastasis of undifferentiated sarcoma from the liver. Chemotherapy consisting of vincristine, actinomycin D, and cyclophosphamide(VAC) was not effective, and the patient died 31 months after the primary surgery. Undifferentiated sarcoma of the liver is a rare malignant mesenchymal tumor, whose occurrence is extremely rare in adults. Although surgical treatment is the first choice, outcomes remain poor. Multimodality treatment should be used to improve the outcome.
一名24岁女性因腹痛入院。腹部增强计算机断层扫描（CT）显示肝左叶有一个11×8厘米的囊性肿块并伴有造影剂外渗。进行了血管栓塞术，但CT图像上仍有造影剂外渗。随后她被转至我院。我们实施了急诊扩大左肝切除术。组织病理学检查显示梭形细胞呈实性增生。免疫组织化学染色显示肿瘤细胞波形蛋白阳性，AE1/AE3阴性。因此，确诊为未分化肉瘤。术后1年零10个月的肺部和右心房CT图像上发现了多个复发性肿瘤。对右心房肿块、左舌段、左下叶和右中叶的肿瘤进行了部分切除。病理诊断证实为肝脏未分化肉瘤转移。由长春新碱、放线菌素D和环磷酰胺（VAC）组成的化疗无效，患者在初次手术后31个月死亡。肝脏未分化肉瘤是一种罕见的恶性间叶肿瘤，在成人中极为罕见。尽管手术治疗是首选，但预后仍然很差。应采用多模式治疗来改善预后。
