Pulmonary Hamartomas (PHs) are the most common benign lung tumors, constituting around 8% of solitary pulmonary nodules and 77% of benign lung nodules. At Protheragen, we are dedicated to offering comprehensive and state-of-the-art services for the preclinical development of diagnostics and therapeutics for Pulmonary Hamartomas (PHs).
Overview of Pulmonary Hamartomas (PHs)
Pulmonary Hamartomas (PHs) are benign lung tumors that are characterized by an abnormal combination of mature mesenchymal tissues, including cartilage, smooth muscle, fat, and fibromyxoid tissue, as well as entrapped benign epithelium. These tumors are usually found incidentally during imaging studies for other medical conditions or during autopsies. They are predominantly located in the peripheral lung parenchyma and typically appear as solitary nodules, often measuring less than 4 cm in diameter. The incidence of PHs is higher among middle-aged and elderly individuals, with a predominance in males. Radiologically, PHs may display a "popcorn" or "comma-shaped" calcification pattern, which can be helpful in their diagnosis.
Fig.1 Overall image of a PH specimen (HE staining). (Grigoraş A.,
et al., 2022)
Pathogenesis of Pulmonary Hamartomas (PHs)
The development of Pulmonary Hamartomas (PHs) is influenced by multiple factors, including genetic and molecular changes. Research in cytogenetics has pinpointed genetic anomalies in specific chromosomal regions: 6p21 and 12q14-15. Notably, a particular chromosomal translocation, t (6;14) (p21; q24), is often seen. This translocation causes a partial merging of the HMGA1 gene with the RAD51L gene, which in turn triggers abnormal cell growth. Moreover, about 80% of PH cases exhibit genetic rearrangements in the 12q14-q15 region that involve the HMGA2 gene. These genetic disruptions interfere with the normal growth and differentiation of mesenchymal tissues, ultimately leading to the formation of PHs.
Diagnostics Development for Pulmonary Hamartomas (PHs)
Radiological imaging plays a crucial role in the diagnosis of PHs. Computed tomography (CT) scans are particularly effective, often revealing characteristic "popcorn" or "comma-shaped" calcifications within the tumor. These calcifications are highly suggestive of PHs and help differentiate them from other lung lesions. In some cases, positron emission tomography (PET) scans are used to assess metabolic activity, although PHs typically exhibit mild to moderate fluorodeoxyglucose (FDG) uptake.
Histopathological Examination
Histopathological analysis is essential for confirming the diagnosis of PHs. Microscopic examination of surgical specimens reveals a mixture of mature mesenchymal tissues, including hyaline cartilage, adipose tissue, fibromyxoid tissue, and smooth muscle bundles, intermingled with entrapped benign epithelium. Immunohistochemical (IHC) staining is often employed to further characterize the tissue components and rule out other differential diagnoses such as lipoma, chondroma, fibroma, and malignancies.
Therapeutics Development for Pulmonary Hamartomas (PHs)
Targeted Therapies
While surgical intervention remains the standard therapeutics, research is ongoing to develop targeted therapies for PHs. Given the genetic alterations observed in PHs, such as HMGA1 and HMGA2 gene rearrangements, there is potential for developing molecularly targeted therapies. These could include small molecules or biologics designed to inhibit the abnormal signaling pathways driven by these genetic aberrations.
Anti-inflammatory Agents
Given the potential role of chronic inflammation in some benign tumor formations, certain anti-inflammatory drugs might be explored for their potential to slow or prevent PH growth. Preclinical studies would focus on evaluating the efficacy of existing anti-inflammatory compounds or developing novel agents with more selective mechanisms of action.
Our Services
Protheragen offers comprehensive services for the diagnostics and therapeutics development of pulmonary hamartomas (PHs). Our expertise spans from early-stage research to preclinical development, providing clients with a one-stop solution for advancing their PHs projects. Our services are designed to meet the specific needs of each client, ensuring that their research objectives are met with precision and efficiency.
Disease Models
- Primary Lung Tissue-Derived Cell Cultures
- Human Pluripotent Stem Cell (hPSC)-Derived Lung Epithelial Cells
- Lung Bud Organoids
- Monocrotaline-Induced Pulmonary Hypertension Models
Protheragen's preclinical research services for PHs are tailored to support the development of novel diagnostics and therapeutics. Our state-of-the-art facilities and experienced scientific team enable us to conduct thorough preclinical studies, including in vitro and in vivo experiments, to evaluate the efficacy and safety of potential therapeutics. If you are interested in our services, please feel free to contact us.
References
- Grigoraş, Adriana, et al. "The clinicopathological challenges of symptomatic and incidental pulmonary hamartomas diagnosis." Romanian Journal of Morphology and Embryology 63.4 (2022): 607.
- Ulas, Ali Bilal, Yener Aydin, and Atilla Eroglu. "Pulmonary hamartomas: a single-center analysis of 59 cases." The Eurasian Journal of Medicine 54.3 (2022): 270.
All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.
