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

TL;DR

This study examines the incidence and survival rates of thoracic angiosarcoma, a rare and aggressive cancer, using data from a German cancer registry.

Contribution

The study provides population-based epidemiologic evidence on thoracic angiosarcoma, highlighting sex differences and the impact of tumor location on survival.

Findings

Thoracic angiosarcoma incidence is significantly higher in women, largely due to secondary tumors following prior cancer treatment.

Survival is strongly influenced by tumor location, with the worst outcomes for angiosarcomas in the lung, heart, mediastinum, or pleura.

Women with thoracic angiosarcoma have better survival rates than men, regardless of whether the tumor is primary or secondary.

Abstract

Thoracic angiosarcoma is a very rare, highly aggressive malignant vascular tumor that may involve the lung, breast, chest wall, heart, or adjacent thoracic tissues. Using data from a large population-based cancer registry in Germany, we analyzed the incidence and post-diagnosis survival of this disease. A markedly higher incidence of thoracic angiosarcoma was observed in women than in men, largely attributable to cases arising as secondary malignancies following prior cancer treatment, with longer survival observed in women than in men for both primary and second primary angiosarcomas. Survival was strongly dependent on tumor location, with particularly poor outcomes observed for angiosarcoma of the lung, heart, mediastinum, or pleura. These findings highlight the aggressive nature of thoracic angiosarcoma and the importance of tumor location in determining patient prognosis.

Introduction: 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. Methods: 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). Results: 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. Conclusions: 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.

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