Congenital Mesoblastic Nephroma (CMN), sometimes called Bland's tumor or fetal renal hamartoma, is a rare kidney growth that shows up mainly in newborns and very young kids. Protheragen provides comprehensive support for the diagnostics and therapeutics development of Congenital Mesoblastic Nephroma. Our services encompass various stages from initial research to preclinical validation, aiming to accelerate the availability of effective solutions for this rare pediatric cancer.
Overview of Congenital Mesoblastic Nephroma (CMN)
Congenital Mesoblastic Nephroma (CMN) is an uncommon kidney tumor found mainly in newborns and very young children, making up roughly 3 to 10 percent of all tumors in this age group. During the first weeks and months of life, it stands out as the most common kidney growth, and doctors usually spot it within those early weeks. Most of the time, CMN behaves like a benign tumor, yet the cell-dense version can act more aggressively, leading to possible recurrence after therapeutic intervention or spread to other parts of the body. Parents typically notice nothing unusual except for a firm belly lump, or the mass is seen by chance on a prenatal scan.
Fig.1 Abdominal computed tomography shows the left renal mass (corresponding to a volume of 76 mL), and defines its relationships with the surrounding organs and structures. (Serra G.,
et al., 2023)
Pathogenesis of Congenital Mesoblastic Nephroma (CMN)
Although the precise ways that congenital mesoblastic nephroma (CMN) causes disease are still being worked out, researchers have made clear headway in uncovering the gene changes linked to the tumour. One defining genetic marker is the ETV6-NTRK3 fusion; this abnormality shows up in about 70 percent of patients. The rearrangement joins the ETV6 gene on chromosome 12 to the NTRK3 gene on chromosome 15, creating a chimeric protein that has active tyrosine-kinase activity all the time. That runaway signalling spurs cells to multiply too quickly and eventually form a tumour.
In addition to the ETV6-NTRK3 fusion, other genetic abnormalities, such as trisomy 11, have also been reported in CMN cases, suggesting a multifactorial etiology involving multiple genetic events. The precise interplay between these genetic alterations and the tumor microenvironment in promoting CMN development remains an active area of research.
Diagnostics Development for Congenital Mesoblastic Nephroma (CMN)
Prenatal Diagnostics
- Ultrasound: Detects solid or mixed solid-cystic renal masses with hypervascularity ("ring sign").
- Fetal MRI: Provides superior soft-tissue contrast to differentiate CMN from neuroblastoma, Wilms tumor, and adrenal lesions.
- Amniocentesis/Karyotyping: Rarely used but may identify chromosomal abnormalities (e.g., t(12;15)).
Postnatal Diagnostics
- Abdominal Ultrasound: Confirms mass location and vascularity.
- Contrast-Enhanced CT/MRI: Assesses tumor margins, invasion, and metastasis.
- Histopathology & Molecular Testing: Gold standard for subtype classification and ETV6-NTRK3 fusion detection.
Therapeutics Development for Congenital Mesoblastic Nephroma (CMN)
- Adjuvant Chemotherapy
Adjuvant chemotherapy is reserved for cases with aggressive histological subtypes, such as cellular CMN, or high-risk features, including incomplete resection or metastatic disease. Chemotherapeutic agents commonly used in the therapeutic of CMN include vincristine, actinomycin-D, and cyclophosphamide. For example, a case with cellular CMN and evidence of local invasion received adjuvant chemotherapy following nephrectomy, which helped prevent tumor recurrence.
- Targeted Therapy
Targeted therapies against NTRK3 fusion proteins represent a promising avenue for the therapeutic of CMN. TRK inhibitors, such as larotrectinib and entrectinib, have shown efficacy in preclinical and clinical studies for tumors harboring NTRK fusions. Although their use in CMN is still under investigation, these agents offer a potential therapeutic option for cases with refractory or recurrent disease.
Table 1. Therapeutics of Congenital Mesoblastic Nephroma (CMN).
| Therapeutics |
Drug Name |
Mechanism |
Description |
Stage |
| Adjuvant Chemotherapy |
Vincristine + Actinomycin-D + Doxorubicin |
Microtubule disruption, DNA intercalation, and Topoisomerase II inhibition |
Reserved for high-risk cellular/mixed CMN or metastatic disease. |
Approved |
| Adjuvant Chemotherapy |
Furosemide |
Diuretic (promotes calcium excretion) |
Used to manage hypercalcemia and hypertension (paraneoplastic syndromes). |
Approved |
| Targeted Therapy |
NA |
NTRK3 kinase inhibitor |
Potential for ETV6-NTRK3 fusion-positive CMN; not yet reported in CMN cases but used in other NTRK-driven tumors. |
Preclinical |
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's diagnostics and therapeutics development services for CMN are characterized by their integrated and multidisciplinary nature. Our approach combines cutting-edge molecular diagnostics, including advanced genetic sequencing and biomarker identification, with comprehensive preclinical drug discovery and development platforms.
Disease Models
- CMN Primary Cultures
- CMN-Derived iPSC Lines
- Fetal Kidney Organoids
- ETV6-NTRK3 Transgenic Mouse
- Xenograft Model (Subrenal Capsule)
Protheragen offers highly customized services to meet the unique requirements of each CMN research and development project. Recognizing the rarity and heterogeneity of CMN, we tailor our experimental designs, assay development, and analytical approaches to align with specific research objectives. If you are interested in our services, please feel free to
contact us.
References
- Serra, Gregorio, et al. "Report and follow-up on two new patients with congenital mesoblastic nephroma." Italian Journal of Pediatrics 49.1 (2023): 124.
- Zhang, Xiaoxiao, et al. "Prenatal diagnosis and postnatal management of congenital mesoblastic nephroma: a case report and literature review." Frontiers in Pediatrics 10 (2022): 1040304.
All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.
