内容紹介
Summary
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.
要旨
症例は24歳,女性。上腹部痛を主訴に近医を受診した。CTで肝左葉に11×8 cm大の囊胞性腫瘍と内部のextravasation所見を認めた。塞栓術を実施するもCTで出血残存が疑われ当院に転院した。準緊急的に肝拡大左葉切除術を実施した。病理所見で星芒状,紡錘形の細胞質を有する異型細胞の増殖を認め,免疫染色でvimentin陽性,AE1/AE3は陰性であり肝未分化肉腫の診断であった。その後,定期的に経過観察していたが,初回手術1年10か月後左胸背部痛が出現し,胸部CTで左肺下葉に結節,右心房内に腫瘤影を認めた。右心房腫瘍摘出,左肺舌区・左肺下葉・右肺中葉部分切除術を実施し,肝未分化肉腫転移の診断であった。再手術後vincristine+actinomycin D+cyclophosphamide(VAC)療法実施もPDの判断となり,患者本人・家族の希望でbest supportive care(BSC)となった。肝未分化肉腫は,成人にはまれな肝原発間葉系悪性腫瘍である。外科切除が第一選択となるが予後は不良である。さらなる症例の蓄積,治療の検討が必要である。
目次
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.
要旨
症例は24歳,女性。上腹部痛を主訴に近医を受診した。CTで肝左葉に11×8 cm大の囊胞性腫瘍と内部のextravasation所見を認めた。塞栓術を実施するもCTで出血残存が疑われ当院に転院した。準緊急的に肝拡大左葉切除術を実施した。病理所見で星芒状,紡錘形の細胞質を有する異型細胞の増殖を認め,免疫染色でvimentin陽性,AE1/AE3は陰性であり肝未分化肉腫の診断であった。その後,定期的に経過観察していたが,初回手術1年10か月後左胸背部痛が出現し,胸部CTで左肺下葉に結節,右心房内に腫瘤影を認めた。右心房腫瘍摘出,左肺舌区・左肺下葉・右肺中葉部分切除術を実施し,肝未分化肉腫転移の診断であった。再手術後vincristine+actinomycin D+cyclophosphamide(VAC)療法実施もPDの判断となり,患者本人・家族の希望でbest supportive care(BSC)となった。肝未分化肉腫は,成人にはまれな肝原発間葉系悪性腫瘍である。外科切除が第一選択となるが予後は不良である。さらなる症例の蓄積,治療の検討が必要である。