Angiosarcoma Cases on the Rise as New Model Offers Improved Prognosis Predictions

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The number of angiosarcoma cases has more than doubled over the past four decades, according to a large-scale study that has tracked the disease’s rise and prognosis trends in the US. The findings suggest not only an increase in incidence but also offer clinicians a new method for estimating survival chances more accurately. The findings were published in the journal Medicine.

Researchers used data from over 5,000 patients recorded in the Surveillance, Epidemiology, and End Results (SEER) database between 1975 and 2016. Angiosarcoma, a rare and aggressive cancer that arises from blood or lymphatic vessels, was found to have risen in age-adjusted incidence from 0.13 to 0.33 per 100,000 people during that period. The steepest increases were noted among individuals aged 60 and older.

Despite its rarity, angiosarcoma has shown a consistent upward trend across all stages and grades, with the most pronounced rise observed in tumours classified as grade 4. The disease can appear in a range of organs, with the most commonly affected being soft tissues including the heart, skin, and breast. The prevalence of angiosarcoma has also grown over time, reflecting both improved detection and longer survival.

Survival rates for angiosarcoma remain grim. The study found that only 26 percent of patients were alive five years after diagnosis. Median survival was just 16 months, with outcomes varying widely depending on tumour location. Patients with breast angiosarcoma had the best outcomes, while those with liver involvement fared the worst.

The study went further by developing a prognostic tool known as a nomogram, which outperforms traditional staging methods in predicting overall survival. Based on eight key factors—age, sex, marital status, tumour grade and stage, surgery, tumour size, and site—the model achieved a higher concordance index than previous systems used by the American Joint Committee on Cancer. This index, a measure of predictive accuracy, was 0.74 for the nomogram, compared with 0.66 and 0.61 for the older models.

Among the prognostic factors identified, age and tumour site emerged as especially significant. Older patients, particularly those over 60, faced markedly worse outcomes. Tumour size also played a critical role, with growths larger than five centimetres being associated with shorter survival. Surgical intervention, on the other hand, was linked to significantly improved outcomes, reinforcing its importance as a treatment option where feasible.

While the study enhances the understanding of angiosarcoma’s trajectory and prognosis, it also acknowledges limitations. A substantial portion of patient records lacked tumour grade information, and details on treatment such as chemotherapy and radiotherapy were absent. Furthermore, the model was derived primarily from data on White and Black populations, and its applicability to other ethnic groups remains untested.

Nonetheless, the findings represent one of the most comprehensive overviews of angiosarcoma to date. As clinicians face rising case numbers, tools like the nomogram offer a more tailored approach to managing a disease that remains one of the most challenging in oncology.

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