Presentation, management, and outcomes of malignant vascular sarcomas: a National Cancer Database comparison of pediatrics and adults

Vascular sarcomas, including angiosarcoma (AS) and epithelioid hemangioendothelioma (EHE), are rare neoplasms for which there do not exist standardized treatment guidelines. This study retrospectively analyzed the National Cancer Database to identify a total of 12,640 malignant vascular sarcoma cases, including 11,311 AS and 1329 EHE cases. We then characterized differences in presentation, treatment, and survival across pediatric, young adult, and older adult cohorts. Pediatric AS patients were more likely to present with metastatic and visceral disease than adults. Metastatic AS had the highest mortality across all age groups. Despite their higher-risk presentation, pediatric patients paradoxically exhibited improved survival compared to adults. M0 status was associated with surgical management, but surgery and chemotherapy were less consistently applied. The findings underscore the clinical heterogeneity of these tumors and the need for standardized, age-specific treatment protocols to improve outcomes in this rare tumor population.