Associate professor of clinical oncology Ain Shams University Faculty of Medicine Cairo, Al Qahirah, Egypt
Objective: Angiosarcoma (AS) is a rare, malignant tumor of vascular mesenchymal origin accounting for less than 1% of all sarcomas. It can be classified as primary type without a known precursor or secondary type with association to a history of irradiated tissue. Additionally, it may classified into cutaneous, visceral, and soft tissue subtypes. The purpose of this study is to examine epidemiologic trends and outcomes in Cutaneous Angiosarcoma (CAS).
Methods: In this retrospective, population-based study, we identified patients with AS from the Surveillance Epidemiology and End Results database. Age, sex, and race-standardized incidence rates (IRs) were calculated. We used MP-SIR session with multiple outcome analysis to calculate the standardized incidence ratio (SIR), as observed/expected (O/E) and was considered significant if P< 0.05, Excess Absolute Risk (EAR) per 10,000, and 95% confidence interval (CI). SPSS version 23 was used for data analysis.
Results: We identified a total of 5127 patients with Angiosarcoma; 60% had first primary malignancy (n=3,3111). Primary Angiosarcoma involving the skin of scalp and neck comprised 4.6% (n=236) of all Angiosarcomas. The risk of multiple primary malignancies in Angiosarcoma not involving the skin of the scalp was increased in the 0-11 months’ interval, with most tumors (22%) arising from the digestive system and O/E ratio of 3.83 (p< 0.05, 95% CI: 2.04-6.55, EAR=80.93). Non-scalp primary first Angiosarcoma also had an increased overall risk of second primaries in other soft tissues, including the heart, with O/E of 23.71 (p< 0.05, 95% CI: 12.25-41.42, EAR=18.73). On the other hand, Angiosarcoma involving skin of the scalp and neck had an increased risk of multiple primary malignancies in the lung and bronchus with overall O/E of 3.91 (p< 0.05, 95% CI: 1.27-9.12, EAR=84.70).
Conclusion: Angiosarcoma is a grave tumor with serious need for continuous monitoring and screening. Endoscopic screening for patients with first primary non- scalp Angiosarcoma is recommended, especially during the first year after diagnosis. In patients with scalp Angiosarcoma, extra attention should be given to lesions in the lung and bronchus.
