Institute of Cancer, Xinqiao Hospital, Third Military Medical University, Chongqing 400037, P.R. China.
Department of General Surgery, Xinqiao Hospital, Third Military Medical University, Chongqing 400037, P.R. China.
Jpn J Clin Oncol. 2020 May 5;50(5):556-567. doi: 10.1093/jjco/hyaa017.
Angiosarcoma is an aggressive and malignant neoplasm. Primary hepatic angiosarcoma is extremely rare and accounts for only approximately 5% of all angiosarcomas. Therefore, many doctors do not know enough about this disease; this lack of knowledge motivated us to perform this study.
We carried out a systematic review of the literature published worldwide from 1990 to 2019 to study the main characteristics, demographics, treatment and prognosis of primary hepatic angiosarcoma.
A total of 219 patients were included in this study. Patients were mainly middle-aged and elderly at diagnosis, with an average age at onset of 56.7 years. The vast majority of patients (61.5%) presented with abdominal pain or distension. Of 143 patients with clear records of metastasis, 31.5% (45 patients) had distant metastasis. The median overall survival time was only 6 months, and the 1- and 2-year survival rates were 30.4 and 17.3%, respectively. Sex, age, tumor size and metastasis at diagnosis showed no correlation with survival rate. Hepatic rupture was a significant predictor of survival. Surgery is a major treatment choice, and adjuvant chemotherapy can improve the prognosis of patients. Hepatic artery embolization is mainly used in cases of tumor rupture. However, liver transplantation is not advised.
We presented an overview of the demographics, tumor characteristics and treatment outcomes of the largest number of primary hepatic angiosarcoma patients investigated to date. We highlight the use of routine physical examinations and surgery combined with adjuvant chemotherapy to improve the outcomes in these cases.
血管肉瘤是一种侵袭性和恶性肿瘤。原发性肝血管肉瘤极为罕见，约占所有血管肉瘤的 5%。因此，许多医生对这种疾病了解不足；这种知识的缺乏促使我们进行了这项研究。
我们对 1990 年至 2019 年全球发表的文献进行了系统回顾，以研究原发性肝血管肉瘤的主要特征、人口统计学、治疗和预后。
本研究共纳入 219 例患者。患者诊断时主要为中老年人，平均发病年龄为 56.7 岁。绝大多数患者（61.5%）以腹痛或腹胀为首发症状。在 143 例有明确转移记录的患者中，31.5%（45 例）有远处转移。中位总生存时间仅为 6 个月，1 年和 2 年生存率分别为 30.4%和 17.3%。性别、年龄、肿瘤大小和诊断时的转移与生存率无关。肝破裂是生存的显著预测因素。手术是主要的治疗选择，辅助化疗可以改善患者的预后。肝动脉栓塞主要用于肿瘤破裂的情况。但是，不建议进行肝移植。
我们总结了迄今为止调查的最大数量的原发性肝血管肉瘤患者的人口统计学、肿瘤特征和治疗结果。我们强调了常规体检和手术联合辅助化疗在改善这些病例结局方面的应用。
