In a comprehensive retrospective study spanning over two decades, researchers at Sahlgrenska University Hospital, Gothenburg, have illuminated the bleak prognosis faced by patients diagnosed with angiosarcoma (AS), an aggressive and rare vascular malignancy. The investigation delved deeply into a cohort of 64 patients treated between 2000 and 2020, offering critical insights into this enigmatic cancer’s clinical behavior, therapeutic challenges, and survival outcomes, underscoring the urgent need for refined treatment strategies and heightened clinical awareness.
Angiosarcoma is a rare type of cancer originating from the endothelial cells lining blood vessels, notorious for its highly malignant nature and poor clinical outcomes. Manifesting either spontaneously as primary angiosarcoma (pAS) or as secondary angiosarcoma (sAS) induced by prior radiation therapy, chemical exposure, or Stewart-Treves syndrome, this tumor presents a multifaceted clinical picture. This dual classification aligns with the diverse pathogenetic mechanisms and clinical demographics observed, demanding nuanced understanding for effective management.
The study at Sahlgrenska University Hospital identified 64 patients with AS over a 21-year period, meticulously reviewing medical records to characterize clinical presentation, treatment modalities, and outcomes including local recurrence, metastasis, and overall survival. Patient ages ranged extensively from 18 to 96 years, with a mean age of 67, reflecting the disease’s predilection for older adults but also its capacity to affect a broader age spectrum.
A notable demographic split emerged between primary and secondary AS cases. Among those with pAS, both genders were represented nearly equally, whereas secondary AS predominantly affected women, primarily correlated with prior breast cancer treatments. This stratification highlights the distinct etiological pathways and risk factors inherent in the disease, with sAS largely arising in the thoracic region attributable to radiotherapy sequelae in breast cancer patients.
Therapeutically, surgery remains the cornerstone intervention, with 84% of patients undergoing surgical resection; however, the high local recurrence rates—65.4% in pAS and 63% in sAS—reflect the tumor’s aggressive local invasiveness and the limited efficacy of surgery alone. Adjuvant treatments incorporating chemotherapy, radiotherapy, or both were employed in approximately 63% of cases, yet even multimodal approaches failed to substantially curb recurrence or improve long-term survival significantly.
The dismal survival statistics from this cohort underscore the daunting prognosis inherent to angiosarcoma. Only 23% of patients were alive at the conclusion of the study’s follow-up period. The mortality analysis revealed that over 60% succumbed directly to AS progression, while an additional 15.6% died from other ailments or causes that could not be determined with certainty, emphasizing the relentless nature of the disease and the vulnerability of this patient population.
Five-year overall survival rates delineated a stark contrast between pAS and sAS patient groups. Specifically, pAS patients demonstrated a meager 10.2% survival rate at five years, while those with sAS fared somewhat better with a 43.5% survival in the same timeframe. These figures not only reveal the aggressive biology of primary AS but also hint at possible differential responses to treatment or inherent biological variability between the two forms of the disease.
Clinically, primary angiosarcomas presented at a variety of anatomical sites, implicating a heterogeneous disease course, whereas secondary angiosarcomas predominantly involved the thoracic region. This localization mirrors the underlying causal factors for sAS, chiefly prior radiation exposure for breast cancer treatment, which induces carcinogenesis in susceptible vascular endothelial cells. Such geographic predilection necessitates heightened surveillance in high-risk post-therapeutic cohorts.
The high recurrence rates recorded in this study reflect the intrinsic challenges navigated by oncologists and surgeons managing angiosarcoma. The tumors’ aggressive angiogenic and infiltrative properties complicate complete resection and render local control elusive despite contemporary surgical and adjuvant techniques. This signifies a pressing demand for innovative therapeutic modalities capable of eradicating microscopic disease and curtailing regrowth.
Moreover, the retrospective nature of this investigation, while comprehensive, highlights the limitations encountered when studying rare malignancies. Small sample sizes, heterogeneity in treatment protocols, and variable follow-up make drawing definitive conclusions challenging but nonetheless establish a vital foundation for future prospective trials and translational research aimed at elucidating molecular targets and optimizing therapy.
The notable survival disparity between pAS and sAS also posits intriguing biological questions. Potential differences in genetic alterations, tumor microenvironment, and host immune responses might underpin the variable clinical courses observed. Investigating these facets could unlock precision medicine approaches tailored to disease subtypes, pushing beyond the one-size-fits-all treatment paradigm currently prevailing for AS.
In light of the suboptimal outcomes observed, the study reinforces the necessity of early diagnosis and multidisciplinary management involving surgical oncologists, radiation oncologists, and medical oncologists. Enhanced diagnostic vigilance, especially in post-radiotherapy breast cancer patients, combined with aggressive staging and individualized treatment planning, could potentially improve prognosis in this otherwise lethal disease.
Furthermore, research efforts must prioritize molecular characterization and therapeutic innovation, encompassing targeted therapies, immunotherapies, and novel chemotherapeutic regimens. It is increasingly clear that conventional treatments yield limited success, and breakthroughs in understanding AS pathobiology will be pivotal in ameliorating patient outcomes.
In conclusion, the Sahlgrenska University Hospital cohort study paints a sobering portrait of angiosarcoma’s clinical trajectory, marked by a 60% local recurrence rate and strikingly low five-year survival, particularly among primary AS patients. These findings call for augmented clinical awareness, robust research endeavors, and therapeutic advancement to confront the formidable challenge posed by this rare yet devastating malignancy.
By delineating the distinct clinical and pathological features of primary and secondary angiosarcomas, this research furnishes invaluable epidemiological and prognostic data that can shape future clinical guidelines and inform patient counseling. It also underscores the critical role of long-term follow-up in capturing the full spectrum of disease behavior and outcomes in AS.
As oncologists continue to grapple with angiosarcoma’s unpredictability and resistance to standard treatments, the need for collaborative multicenter studies and clinical trials escalates. Sharing data and resources globally may facilitate accrual of sufficient patient numbers to validate emerging therapies and deepen understanding of this rare cancer’s underlying mechanisms.
Ultimately, confronting the high mortality and recurrence associated with angiosarcoma demands a concerted, multidisciplinary approach integrating clinical acumen, patient-centered care, and cutting-edge scientific investigation. Only through such collective efforts can the dismal prognosis currently tethered to AS be transformed into more hopeful and survivable clinical outcomes.
Subject of Research: Clinical presentation, management, treatment, and survival outcomes of patients diagnosed with primary and secondary angiosarcoma.
Article Title: Low overall survival after treatment for angiosarcoma: a single-centre retrospective observational cohort study of 64 patients.
Article References:
Kjartansdóttir, S., Malchau, E., Berger, C. et al. Low overall survival after treatment for angiosarcoma: a single-centre retrospective observational cohort study of 64 patients. BMC Cancer 25, 1378 (2025). https://doi.org/10.1186/s12885-025-14642-7
Image Credits: Scienmag.com
