Key result
Primary hepatic angiosarcoma carries a poor prognosis with a median overall survival of ~6 months.
Why the study?
Primary hepatic angiosarcoma is an extremely rare and aggressive malignant neoplasm, and clinical knowledge regarding the disease is limited.
Systematic Review (n=219)
Primary hepatic angiosarcoma is an extremely rare and aggressive tumor with a median survival of 6 months, for which surgery combined with adjuvant chemotherapy is recommended to improve outcomes.
Underscores need for multimodal therapy and novel agents in primary hepatic angiosarcoma; confirms poor outcomes and extends the evidence base for this rare tumor.
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.
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Zeng et al. (2020) conducted a systematic review in Primary hepatic angiosarcoma (n=219). Primary hepatic angiosarcoma was evaluated on Overall survival. Primary hepatic angiosarcoma is associated with a poor prognosis, with a median overall survival of 6 months and 1- and 2-year survival rates of 30.4% and 17.3%, respectively.
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