Dong Zeng1, Jianan Cheng1, Zhihua Gong1, Jianghong Chen2, Haixia Long1, Bo Zhu1. 1. Institute of Cancer, Xinqiao Hospital, Third Military Medical University, Chongqing 400037, P.R. China. 2. Department of General Surgery, Xinqiao Hospital, Third Military Medical University, Chongqing 400037, P.R. China.
Abstract
BACKGROUND: 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. METHODS: 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. RESULT: 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. CONCLUSION: 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.
BACKGROUND: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. METHODS: 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. RESULT: 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. CONCLUSION: We presented an overview of the demographics, tumor characteristics and treatment outcomes of the largest number of primary hepatic angiosarcomapatients investigated to date. We highlight the use of routine physical examinations and surgery combined with adjuvant chemotherapy to improve the outcomes in these cases.
Authors: Isidro Machado; Francisco Giner; Javier Lavernia; Julia Cruz; Víctor Traves; Celia Requena; Beatriz Llombart; José Antonio López-Guerrero; Antonio Llombart-Bosch Journal: Histol Histopathol Date: 2020-09-04 Impact factor: 2.303