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

Thoracic angiosarcoma is a very rare and malignant disease. This study examined the incidence and survival of thoracic angiosarcomas with special focus on primary and secondary angiosarcomas.

Data from the population-based North Rhine-Westphalia (NRW) cancer registry in Germany covering the years 2008-2023 were analyzed, including primary and secondary thoracic angiosarcoma (ICD-O-3: morphology 9120/3, topography C34, C38, C44.51, C49.3, C50), and reported age-standardized incidence rates (using the Old European Standard population) and survival analyses (Kaplan-Meier curves).

A total of 421 cases of thoracic angiosarcoma were analyzed, and 90.0% of the patients were female (n = 379). Age-standardized incidence rates of thoracic angiosarcoma were 0.25 per million per year for male patients (SE 0.0) and 1.5 per million per year for female patients (SE 0.1). All male patients had primary angiosarcoma (n = 42). Among female patients, the majority of cases were second primary tumors (n = 262, 69.1%). The 5-year overall survival (OS) for the cohort study was 38.5% (SE 2.6). Stratified by sex, OS was 41.1% (SE 2.8) for women and 12.0% (SE 5.4) for men. Among female patients, OS was 40.9% (SE 4.1) for primary and 41.8% (SE 3.8) for second primary angiosarcoma. The worst OS occurred among patients with angiosarcoma of the lung (men 20.0% (SE 12.7)) and of the 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.

Thoracic angiosarcoma is a rare condition with a poor prognosis. Irrespective of whether their disease is classified as primary or second primary, women with angiosarcoma have a better prognosis than men. Topography seems to be the most determining prognostic factor in this disease.