Primary cardiac angiosarcoma: From pathogenesis to therapeutic strategies.

Primary cardiac angiosarcoma (PCAS) is a rare, highly aggressive malignancy arising from cardiac vascular endothelial cells and carries a dismal prognosis.

• The abstract describes median survival of typically less than one year.
• The tumor predominantly affects young to middle-aged males.
• Most patients present at an advanced or metastatic stage because of nonspecific manifestations and rapid infiltrative growth.

Genomic and transcriptomic studies have identified recurrent molecular drivers in PCAS.

• Recurrent mutations are reported in POT1, KDR/VEGFR2, and PLCG1.
• Loss of CDKN2A is reported.
• Aberrant activation of the angiogenic cascade is described.

PCAS has an immunosuppressive tumor microenvironment that may contribute to progression and therapeutic resistance.

• The microenvironment is described as dominated by exhausted T cells and tumor-associated macrophages.
• The abstract states these features may collectively contribute to tumor progression and therapeutic resistance (framed as possible, not established).

Multimodal imaging plays a central role in detection, tissue characterization, and systemic staging of PCAS.

• Modalities integrated include echocardiography, cardiac magnetic resonance, computed tomography, and PET/CT.

Histopathology with immunohistochemistry remains the diagnostic gold standard for PCAS.

• Imaging supports detection and staging, but tissue diagnosis is the reference standard.

Current treatment relies on multimodal approaches, with targeted therapies and immunotherapies under active investigation.

• Established strategies include surgery, chemotherapy, and radiotherapy.
• Emerging targeted therapies and immunotherapies are under active investigation.
• Despite recent advances, clinical outcomes remain poor.