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. 2024 Apr 1;7(4):e246235.
doi: 10.1001/jamanetworkopen.2024.6235.

Incidence and Presenting Characteristics of Angiosarcoma in the US, 2001-2020

Michael J Wagner  1   2   3 , Vinod Ravi  4 , Stephanie K Schaub  5 , Ed Y Kim  5 , Jeremy Sharib  6 , Harveshp Mogal  6 , Min Park  7 , Michaela Tsai  7 , Daniela Duarte-Bateman  8 , Anthony Tufaro  8 , Elizabeth T Loggers  1   2 , Lee D Cranmer  1   2 , Bonny Chau  2 , Michael J Hassett  3 , Juneko Grilley-Olson  9 , Kelly G Paulson  7
Affiliations

Incidence and Presenting Characteristics of Angiosarcoma in the US, 2001-2020

Michael J Wagner et al. JAMA Netw Open. .

Abstract

Importance: Angiosarcoma is an aggressive vascular malignant neoplasm presenting either as a primary or secondary cancer, often arising after radiotherapy or in the context of preexisting lymphedema. Comprehensive data describing its incidence and presentation patterns are needed.

Objective: To describe the incidence, presenting characteristics, and change over time of angiosarcoma in the US.

Design, setting, and participants: This retrospective cross-sectional study used data from the US Cancer Statistics (USCS) National Program of Cancer Registries-Surveillance, Epidemiology, and End Results Combined Database, which captures more than 99% of newly diagnosed cancers in the US. The study included all 19 289 patients in the US with a new diagnosis of angiosarcoma between 2001 and 2020 captured in the USCS database. Statistical analysis was performed from June to September 2023.

Main outcomes and measures: Incidence of angiosarcoma, demographics of patients with angiosarcoma, and extent of disease at presentation.

Results: The study included 19 289 patients (median age, 71 years [IQR, 59-80 years]; 10 506 women [54.5%]) with a new diagnosis of angiosarcoma. The US incidence of angiosarcoma doubled between 2001 (657 cases) and 2019 (1312 cases), reflecting both an increase in the adjusted incidence rate of 1.6% per year (P = .001), to 3.3 cases per 1 000 000 person-years (95% CI, 3.1-3.5 cases per 1 000 000 person-years), and an increase in the population at risk. In 2020, the reported incidence rate (3.0 cases per 1 000 000 person-years) and cases of angiosarcoma (n = 1159) were modestly lower than in 2019. Overall, 72.3% of cases of angiosarcoma (n = 13 955) were cutaneous, subcutaneous, or breast angiosarcomas; 24.4% were visceral (n = 4701); and 3.3% were located in unknown or rare primary sites (n = 633). Secondary breast and chest wall angiosarcomas among women represented the largest contribution to increasing incidence. Among breast angiosarcomas, 99.2% (2684 of 2705) were in women and 71.9% (1944 of 2705) were secondary. A total of 80.4% of chest wall or thorax cases among women (1861 of 2316) were secondary vs 26.5% among men (112 of 422), and 63.9% of upper extremity cases among women (205 of 321) were secondary vs 26.8% (56 of 209) among men (P = .001). Rates of secondary angiosarcoma in the abdomen and lower extremities were similar between men and women. The incidence rate of visceral angiosarcoma was also found to be increasing (1.5% per year; P = .001).

Conclusions and relevance: This cross-sectional study describes angiosarcoma presentation patterns and incidence rates in the US over a 20-year period and shows that the number of cases in men and women increased, with the greatest increase among women with secondary angiosarcoma of the chest, breast, and upper extremity. These data increase awareness of a rare but highly morbid disease and highlight the need for improved early detection of angiosarcoma among patients at high risk, such as women with a history of breast cancer.

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Conflict of interest statement

Conflict of Interest Disclosures: Dr Wagner reported receiving personal fees from Adaptimmune, Deciphera, Epizyme, Aadi Bioscience, and PharmaEssentia outside the submitted work. Dr Cranmer reported receiving grants to institution from Merck, Exelixis, Eli Lilly, Avacta, AADi Biosciences, Philogen, InhibRX, Zentalis, Boehringer Ingelheim; and personal fees from AADi Biosciences and Boehringer Ingelheim outside the submitted work. No other disclosures were reported.

Figures

Figure 1.
Figure 1.. Incidence of Angiosarcoma in the United States (N = 19 289)
A, Cases per year of incident (newly diagnosed) angiosarcoma in the US; the black horizontal line indicates 1000 newly diagnosed cases per year, and the orange bar indicates year 2020, when reported incidence was impacted by COVID-19 pandemic. B, Reported cases of angiosarcoma by month and year, 2016-2020. C, Incidence rate of angiosarcoma in the US Cancer Statistics (USCS) dataset, adjusted for population age and sex. Error bars indicate 95% CIs. D, Age distribution at time of diagnosis. APC indicates annual percentage change.
Figure 2.
Figure 2.. Cutaneous, Subcutaneous, and Breast Angiosarcoma (N = 13 955)
A, US-reported incidence rate, 2001-2020. Error bars indicate 95% CIs. B, Breakdown by body site, sex, and first or second or later malignant neoplasm. C, Changes in incidence rate for breast angiosarcoma among women based on whether the angiosarcoma was the first reported malignant neoplasm or second or later reported malignant neoplasm. Error bars indicate 95% CIs. D, Percentage by site and sex that are the second or later malignant neoplasm for the patient. APC indicates annual percentage change. aP < .001. bP = .006.
Figure 3.
Figure 3.. Incidence Rates of Angiosarcoma With Visceral Primary Sites
Error bars indicate 95% CIs. APC indicates annual percentage change.
See this image and copyright information in PMC

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