Cardiac Angiosarcoma
Cardiac angiosarcoma, a form of primary cancer with occurrence in the heart, is an infrequent and highly invasive tumor that arises from the endothelial lining of blood vessels in the heart. The diagnosis and management of cardiac angiosarcoma is particularly difficult due to its infiltrative growth pattern and tendency for early metastatic spread. At Protheragen, we focus on pushing forward the development of transformative therapies for rare diseases of the cardiovascular system, including the intricate issue of primary cardiac angiosarcoma, and offer comprehensive pharmaceutical research and development services.
Introduction to Cardiac Angiosarcoma
The occurrence of primary cardiac neoplasms is exceptionally rare, with an autopsy incidence ranging from 0.0001% to 0.030%, approximately one in 500 cardiovascular surgical individuals. Angiosarcomas, which are the most prevalent form of primary cardiac malignancy, typically develop within the right side of the heart, particularly the right atrium. The defining feature of cardiac angiosarcoma is the aggressive proliferation of neovascularization, which progressively invades the myocardium, resulting in the obliteration of normal tissue.

Pathogenesis of Cardiac Angiosarcoma

The development of primary cardiac angiosarcoma is associated with aberrant angiogenesis as well as distinct molecular changes. KDR gene mutations, occurring in 7% to 10% of cases, fuel excessive angiogenesis. PLCG1 mutations disrupt phosphoinositide signaling and confer resistance to therapies targeting VEGF. Mutations in the protection of telomeres 1 (POT1) gene associated with familial and sporadic primary cardiac angiosarcoma are thought to allow for the immortalization of tumor cells.
Therapeutics Development for Cardiac Angiosarcoma
Drug Name | Mechanism of Action | Targets | Research Phase |
---|---|---|---|
Sorafenib | Inhibits several intracellular (RAF kinase) and cell surface kinases involved in tumor cell proliferation and angiogenesis. | RAF kinase | Phase IV |
Bevacizumab | Monoclonal antibody that binds to and neutralizes vascular endothelial growth factor A (VEGF-A), thereby inhibiting angiogenesis (new blood vessel formation) that tumors need to grow. | VEGF-A | Phase IV |
Pazopanib | Inhibits several receptor tyrosine kinases important for angiogenesis and tumor growth. | VEGFR, PDGFR | Phase IV |
Propranolol | A non-selective beta-adrenergic receptor blockade that proposed anti-cancer effects by inhibiting catecholamine-driven tumor growth and angiogenesis. | Beta-adrenergic receptors | Phase IV |
Disclaimer: Protheragen focuses on providing preclinical research services. This table is for information exchange purposes only. This table is not a therapy plan recommendation. For guidance on therapy options, please visit a regular hospital.
Our Services
Appreciate the scope and the challenges presented by rare cardiovascular diseases pathology, from the limited population of potential individuals to complex disease mechanisms, we provide integrated approaches spanning diagnostics and therapeutics to advanced modeling of the disease. This offers the most rapid and effective route to innovative therapeutic development and preclinical research for cardiac angiosarcoma, including pharmacokinetics and drug safety evaluation.
Therapeutic Development Services
Diverse Platforms

Animal Model Development for Cardiac Angiosarcoma
At Protheragen, we understand the importance of rare preclinical animal models in the early stages of cardiovascular disease research. We offer comprehensive services in developing animal models for primary cardiac angiosarcoma to accompany your research from its initial stages to confirmation. Our animal model development expertise will provide you with human-disease-relevant models, which will expedite your drug discovery and development processes.
These models utilize specific chemical carcinogens to induce tumor development in susceptible animal strains, which can then be adapted to study cardiac involvement.
- 1,3-butadiene-induced model
- Other models

Construct genetic engineering animal models by introducing specific genetic alterations, offering insights into the molecular drivers of the disease.
- POT1R117C mutation model
- Other models
Selecting Protheragen means partnering with a team of specialists committed to addressing the issues posed by uncommon cardiovascular diseases. The core of our competitive benefits lies in the integration of different domains, promoting synergy between diagnostics and therapeutic development. This comprehensive perspective streamlines your research, shortens timelines, and augments the probability of a successful outcome. Should you be interested in our services, please reach out.
References
- Fang, Xin, and Song Zheng. "Primary cardiac angiosarcoma: a case report." The Journal of international medical research 49.8 (2021): 3000605211033261.
- Kumari, Naina et al. "Primary Cardiac Angiosarcoma: A Review." Cureus 15.7 (2023): e41947.
For research use only, not for clinical use.