Metanephric Stromal Tumor (MST) is a distinct entity that lies in the intersection of pediatric oncology and renal pathology, requiring specific strategies for MST multidisciplinary evaluation regarding diagnostics and therapy. Protheragen offers MST comprehensive diagnostic and therapeutic development services using advanced technologies and interdisciplinary collaboration.
Overview of Metanephric Stromal Tumor (MST)
Metanephric Stromal Tumor (MST) is an uncommon, non-cancerous tumor of the kidney that most often affects children, although it may present in adults as well. It falls within the metanephric tumor family, which contains metanephric adenoma (MA) and metanephric adenofibroma (MAF). Other than abdominal pain, hematuria, hypertension, and an observable mass, which are congenital signs of MST, accompanied by myxoid stroma with attached renal tubules and blood vessels oscillating towards concentric structures like onions typically form between the ages of 1 to 3 years old. Robotics surgery diagnosis relies heavily on IHC-commonly positive CD34. Lately conducted molecular studies have identified that recurrent BRAF V600E mutations found in MST could be remarkably promising from a diagnostic perspective.
Fig.1 Histological characteristics of adult metanephric stromal tumor with BRAF V600E mutation. (Fan Y.,
et al., 2022)
Pathogenesis of Metanephric Stromal Tumor (MST)
The BRAF V600E mutation is an MST pathogenesis caused by genetic mutations that activate the MAPK signaling pathway, causing uncontrolled cell proliferation. Metanephric stromal cells that participate in renal development are hypothesized to give rise to MST. Tumorigenesis may result from a developmental or differentiation abnormality of these cells. In certain instances, juxtaglomerular cell hyperplasia with increased renin secretion can be present, which indicates some of these tumors may lead to hypertension due to the entrapment of cortical glomeruli by the tumor.
Diagnostics Development for Metanephric Stromal Tumor (MST)
Imaging Techniques
For the primary diagnosis of MST, ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MRI) are critical. Canonically, MST manifests as a sharply demarcated hypovascular mass with varying degrees of solid and cystic composition. In addition, MRI reveals various critical features regarding the tumor such as its signal intensity and enhancement patterns.
Histopathological Examination
MST is histologically defined by the presence of spindle or stellate-shaped cells within a fibrous or myxoid stroma. This stroma contains renal tubules and blood vessels, which are frequently enclosed by concentric layers reminiscent of onion skins. For MST, immunohistochemistry (IHC) analysis is essential for accurate diagnosis; CD34 tends to be a common positive marker. Additionally, estrogen and progesterone receptors may also show positivity.
Molecular Testing
With targeted next-generation sequencing (NGS), it is possible to identify BRAF V600E mutations which aid in differentiating Metanephric Stromal Tumor (MST) from other renal tumors. Other genetic rearrangements can be eliminated through testing with Fluorescence in situ Hybridization (FISH) testing. These advanced molecular tests improve diagnostic precision and may refine future therapeutics.
Therapeutics Development for Metanephric Stromal Tumor (MST)
- Molecular Targeted Therapies: With the identification of BRAF V600E mutations, there exists a possibility of considering BRAF or MAPK pathway targeting therapies for cases where surgical intervention is impractical or in the setting of recurrent tumors. These targeted therapies could provide prior therapeutic alternatives in MST cases.
- Immunotherapy: While immunotherapy is rarely applied for MST, it might be considered in the future. The implementation of immune checkpoint inhibitors or other immunomodulatory agents could be looked into to strengthen the body's immune response toward MST cells.
Table 1. Therapeutics of Metanephric Stromal Tumor (MST).
| Therapeutics |
Drug Name |
Target |
Description |
Stage |
| Surgical Resection |
N/A |
N/A |
Complete removal of the tumor with negative resection margins. |
Approved |
| Targeted Therapy |
BRAF inhibitors |
BRAF V600E |
Targeting the BRAF V600E mutation to inhibit tumor growth. |
Approved |
| Chemotherapy |
Vincristine, Dactinomycin |
N/A |
Used in cases of incomplete resection or suspicion of malignancy. |
Approved |
Disclaimer: Protheragen focuses on providing preclinical research services. This table is for information exchange purposes only. This table is not a treatment plan recommendation. For guidance on treatment options, please visit a regular hospital.
Our Services
Protheragen has full-service capabilities for MST diagnostics and therapeutics and provides advanced imaging, histopathological, and molecular testing as part of the accurate diagnosis workflow. Furthermore, we support MST by applying our expertise on molecular targeted therapies in order to develop effective therapeutics.
Disease Models
- BRAF V600E Mutation Models
- WT1 Mutation Models
- PAX2 Mutation Models
- Transgenic Models with Renal Stromal-Specific Promoters
- Patient-Derived Tumor Xenografts (PDTX)
Protheragen's preclinical research services related to MST cover a wide array of studies from basic science to translational research. Our staff employs sophisticated technologies to study the disease mechanisms of MST, determine potential therapeutic targets, as well as design and develop new therapeutic methods. We ensure every project receives maximum precision by providing bespoke services tailored directly to client requirements. If you are interested in our services, please feel free to contact us.
References
- Fan, Yuqian, Jingjing Yu, and Ming Zhao. "Metanephric stromal tumor with BRAF V600E mutation in an adult patient: Case report and literature review." Frontiers in Oncology 12 (2022): 993414.
- Riese, Ronja, et al. "Metanephric stromal tumor as a rare differential diagnosis of a renal mass in children–a case report." Pediatric Radiology (2025): 1-4.
All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.
