Pigmented Villonodular Synovitis (PVNS)
Pigmented villonodular synovitis (PVNS), a subtype of tenosynovial giant cell tumor (TGCT), is a rare, benign, proliferative disorder affecting the lining of joints, bursae, and tendon sheaths. At the forefront of rare disease research, our company offers a comprehensive array of services to bolster your PVNS research.
Overview of PVNS
PVNS is a synovial tissue tumor-like proliferative disease, the incidence of PVNS stands at approximately 1.8 cases per 1 million and can manifest in two distinct forms - the localized type (LPVNS) and the more invasive diffuse type (DPVNS). Predominantly found in joints such as the knee, hip, and shoulder, PVNS may trigger joint swelling, pain, stiffness, limited range of motion, recurrent joint effusions, and tenderness. Severe cases might culminate in joint destruction and functional impairments.
Fig.1 Preoperative imaging of PVNS individual. (Chipman, D. E., et al., 2024)Pathogenesis of PVNS
The etiology of PVNS is believed to stem from abnormal synovial tissue growth driven by chronic inflammation or trauma. Translocations of chromosome 1p13 have been identified in most PVNS cases, prompting the overexpression of colony-stimulating factor 1 (CSF1) and attracting non-neoplastic inflammatory cells, predominantly macrophages expressing the CSF1 receptor (CSF1R), to form tumor masses.
Fig.2 Chromosomal aberrations of the CSF1 gene lead to an overexpression of CSF1. (Spierenburg, G., et al., 2022)Diagnostics Development of PVNS
In addition to conventional imaging modalities like X-rays, MRI, and ultrasound, specific biomarkers hold promise for accurate PVNS diagnosis. Here are some potential biomarkers that are highly correlated with PVNS:
- IL-6
- TNF
- CD163
- KRAS
- FN1
- HMOX1
- PTPRC
- MAPK1
- CCR2
- NFKBIA
- IL-1β
- Others
Therapeutics of PVNS
Conventional Therapy
Small Molecule Drug Therapy
Monoclonal Antibody Therapy
Our Services
Our company boasts seasoned expertise in rare disease research and a proficient workforce poised to aid your PVNS research. Leveraging our animal models and therapeutic development platform, we offer a comprehensive suite of services catering to the exploration of PVNS pathogenesis and the development of novel therapies.
Platforms of PVNS Therapy Development
Animal Models of PVNS
Animal models play a pivotal role in deciphering the disease pathogenesis and evaluating therapy efficacy. We proffer transplant models and genetically engineered models to illuminate PVNS pathogenesis and assess therapy outcomes effectively.
| Xenograft Models | ||
| This animal model involves transplanting tumor tissue into the synovial membrane of the ankle joint in mice or rats, mirroring the abnormal synovial tissue growth in human PVNS. | ||
| Optional Models |
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| Genetically Engineered Models | ||
| This animal model involves the manipulation of specific genes or proteins in mice by transgenic or gene editing technology to induce the development of PVNS-like lesions. | ||
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| Optional Species | Mice, Rats, Non-Human Primates, Others | |
With an innovative approach and a wealth of experience, our company can provide comprehensive services for PVNS research, encompassing pharmacokinetics analysis and drug safety evaluation. If you are interested in our services, please feel free to contact us for more details and quotation information of related services.
References
- Chipman, Danielle E et al. "Pigmented villonodular synovitis/giant cell tumor in the knee." Current opinion in pediatrics 36.1 (2024): 78-82.
- Spierenburg, Geert et al. "Tenosynovial giant cell tumors (TGCT): molecular biology, drug targets and non-surgical pharmacological approaches." Expert opinion on therapeutic targets 26.4 (2022): 333-345.
All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.