Incidence and Survival of Thoracic Angiosarcoma. Epidemiologic Evidence from a Population-Based Cancer Registry

Background/Objectives: Angiosarcoma of the thorax is a very rare and malignant disease. We studied the incidence and survival of thoracic angiosarcomas with special focus on primary and secondary angiosarcoma. Methods: We analyzed data from the population-based cancer registry North Rhine-Westphalia (NRW), Germany, of the years 2008–2023. We included primary and secondary angiosarcoma of the thorax (ICD-O-3: morphology 9120/3, topography C34, C38, C44.51, C49.3, C50) and report age-standardized (Old European Standard population) incidence rates and survival (Kaplan-Meier Curves). Results: We analyzed 421 cases of thoracic angiosarcoma. 90.0% were female (n = 379). The age-standardized incidence rates of angiosarcoma of the thorax were 0.25 per million and year for male patients (SE 0.0) and 1.5 per million and year for female patients (SE 0.1). All male patients had primary angiosarcoma (n = 42). The majority of thoracic angiosarcoma among females were second primary tumors (n=262, 69.1%). The 5-year overall survival (OS) was 38.5% (SE 2.6). OS for women was 41.4% (SE 2.8) and for men was 12.0% (SE 5.4). OS for female patients was 40.9% (SE 4.1) and 41.8% (SE 3.8) for primary and second primary angiosarcoma respectively. The worst OS had patients with angiosarcoma of the lung (men 20.0% (SE 12.7) and mediastinum, heart and pleura (men 4.7% (SE 4.5). The OS for women was 0%, all females died within 2.2 years after diagnosis of angiosarcoma with these topographies. Conclusions: Angiosarcoma of the thorax is a rare condition with poor prognosis. Irrespective of the classification into primary and second primary, women with angiosarcoma have a better prognosis than men. Topography seems to be the most determining prognostic factor in this disease.