Cutaneous angiosarcoma (cAS) is an aggressive vascular tumor that originates from vascular or lymphatic epithelial cells. To date, the cAS literature has been limited in a small number with single-center experiences or reports due to its rarity and the optimal treatment strategy is still in dispute. This study aimed to conduct a systematic review and compare the effect of available treatments retrieved from observational studies and Surveillance, Epidemiology, and End Results (SEER) program.
The authors performed a systematic review in the PubMed, Embase and MEDLINE database identifying the researches assessing the treatment for cAS patients. Clinical and treatment information of patients who had been diagnosed with a primary cAS were also obtained from the SEER program.
Thirty-two studies were eligible but only 5 of which with 276 patients were included in meta-analysis since the unclear or unavailable information. The risk ratio of 5-year death for surgery, surgery with radiotherapy and surgery with chemotherapy were 0.84, 0.96, and 0.69. Meanwhile, in SEER database, there are 291 metastatic and 437 localized patients with cAS. The localized patients receiving surgery showed a significantly worse overall survival result when compared with the surgery combined with RT: hazard ratio: 1.6, 95% confidential interval: 1.05, 2.42, P = 0.03.
In conclusion, our study provided a detailed picture of the effectiveness of present treatments for localized and metastatic cAS patients. The CT could be inappropriate in localized patients. For metastatic patients, the surgery combined RT was recommended compared with surgery alone since its enhanced OS prognosis. Yet, more novel-designed clinical trials with specific targeted populations and rigorous conducting are needed for a solid conclusion on which would be a better treatment strategy.