Cardiac Fibromas
Cardiac fibroma is a rare, non-encapsulated, benign tumor of the heart. It is most commonly observed among infants and in children. It can have a lot of growth, which can lead to multiple heart complications, including sudden death. Protheragen provides innovative drug research solutions for rare cardiac fibromas and other challenging cardiovascular diseases. We plan to bridge the gap between the "bench and bedside" for therapy development through integrated solutions, and ultimately offer seamless, comprehensive services across the drug development continuum.
Introduction to Cardiac Fibromas
The occurrence of primary cardiac tumors is known to range between 0.03% and 0.32%, and 75% of cardiac neoplasms are benign. After rhabdomyoma, cardiac fibromas are the second most common primary cardiac tumor in children, and are made up of fibroblasts and connective tissue. While these are benign tumors, they can cause severe symptoms and complications, including intracavitary obstruction, coronary artery compromise, and conduction disturbances up to and including sudden death.
Fig.1 Diagnosis and management of cardiac fibromas in adult individuals. (Finke, K., et al., 2024)Pathogenesis of Cardiac Fibromas
Cardiac fibroma commonly occurs as a sporadic tumor, although it does occur in association with the familial disorder Gorlin syndrome, or nevoid basal cell carcinoma syndrome. Gorlin syndrome is an autosomal dominant, multisystem disease resulting from mutations in PTCH1 or SUFU tumor suppressor genes located on chromosome 9q22.3. The genes encode a protein known to inhibit tumor growth by inhibiting the Hedgehog signaling mechanism.
Fig.2 Semicircular cardiac mass in a cardiac fibroma individual. (Finke, K., et al., 2024)Therapeutics Development for Cardiac Fibromas
Surgery has been the mainstay of cardiac fibromas therapy for a potential cure. An appreciation of a tumor's molecular nature is increasingly important as researchers explore non-surgical modes of therapy. Certain antiarrhythmic drugs (e.g., amiodarone and beta-blockers for treating irregular heart rates) have shown effectiveness.
Our Services
As your dedicated partner in change for rare cardiovascular disease therapy, we provide rare cardiovascular disease therapeutics development, advanced diagnostic development, as well as supporting and understanding disease mechanisms, and developing state-of-the-art disease models. Furthermore, we offer extensive drug safety assessment and pharmacokinetic analysis for an accurate profile of the drug before additional studies.
Therapeutic Development Services
Diverse Platforms
Animal Model Development for Cardiac Fibromas
Recognizing the role of animal models in the discovery and development of therapeutics for rare cardiovascular diseases of the heart, like cardiac fibromas, we offer complete animal model development services customized for cardiac fibromas. This will guarantee that you receive a model with correct and reproducible disease phenotypes that mimic those observed in human disease, for you to accelerate your research and drug development.
Genetically Engineered Animal Models
Genetic engineering involves the targeted manipulation of genes implicated in the development of fibrotic conditions and tumors.
Optional models: TSC1/TSC2 knockout models, other models.
To collaborate with Protheragen is to have a strategic advantage in the challenging area of rare cardiovascular disease drug development. We are excited to offer our established preclinical development platform, which brings together deep scientific knowledge with cutting-edge facilities and a relentless focus on therapy innovation for cardiac fibroma. If you would like to know our services, don't hesitate to contact us.
Reference
- Finke, Karl et al. "Cardiac fibromas in adult patients: a case series focusing on rhythmology and radiographic features." European heart journal. Case reports 8.8 (2024): ytae410.
For research use only, not for clinical use.