Review maps mechanisms, diagnosis and treatment of primary cardiac angiosarcoma

Primary cardiac angiosarcoma is a rare, highly aggressive malignancy arising from cardiac vascular endothelial cells, with a median survival typically shorter than one year, according to a 2026 review in Medical Oncology . The review examines the tumor’s pathogenesis, diagnosis, staging and current and emerging treatment strategies. PCAS predominantly affects young to middle-aged males.

Its nonspecific manifestations and rapid infiltrative growth mean that most patients present with advanced or metastatic disease. The review identifies recurrent mutations in POT1 , KDR /VEGFR2 and PLCG1 , as well as loss of CDKN2A . It also highlights aberrant activation of angiogenic signaling.

The tumor microenvironment may also be relevant to progression and treatment resistance. The review describes an immunosuppressive environment dominated by exhausted T cells and tumor-associated macrophages. These observations are presented as contributors that may collectively support tumor progression and resistance, rather than as quantified clinical effects.

Diagnosis and staging use a multimodal imaging approach involving echocardiography, cardiac magnetic resonance, computed tomography and PET/CT. These methods help detect the tumor, characterize tissue features and assess systemic spread. Histopathology with immunohistochemistry remains the diagnostic gold standard.

Treatment currently relies on combined approaches involving surgery, chemotherapy and radiotherapy. Despite advances in molecular analysis, imaging and systemic treatment, clinical outcomes remain poor; the review concludes that improved mechanistic understanding and more effective personalized therapeutic strategies are urgently needed.

Subject of Research: Pathogenesis, diagnosis and treatment of primary cardiac angiosarcoma

Article Title: Primary cardiac angiosarcoma: From pathogenesis to therapeutic strategies

Article References: Gao, H., Zhang, H., Zhu, D., Zhang, Y., Guo, M., Zhu, M., Wang, Y., & Yang, M. (2026). Primary cardiac angiosarcoma: From pathogenesis to therapeutic strategies. Medical Oncology, 43(11), Article 346. https://doi.org/10.1007/s12032-026-03467-4

Image Credits: AI Generated

DOI: 10.1007/s12032-026-03467-4

Keywords: primary cardiac angiosarcoma, cardiac cancer, vascular endothelial cells, cancer genomics, tumor microenvironment, cancer immunotherapy, cardiac imaging, targeted therapy, multimodal treatment, Primary, cardiac, angiosarcoma