Adult Cystic Nephroma (CN) is an uncommon, non-hereditary form of a benign cystic neoplasm of the kidney that occurs primarily in women during their middle-aged years. Protheragen provides complete preclinical therapeutic development for Adult Cystic Nephroma, which includes diagnosis, drug discovery, and therapy development.
Overview of Adult Cystic Nephroma (CN)
Cystic nephroma is an uncommon benign renal neoplasm that falls under the umbrella of adult neoplasms. It is characterized by a multilocular cystic structure containing cysts lined with epithelium and stroma similar to the ovary. It constitutes less than half a percent of all renal tumors, has a striking female predominance (8:1), and peaks in the perimenopausal age group. Due to some overlapping histological as well as molecular features, CN is now categorized within Mixed Epithelial and Stromal Tumors (MEST) genetically due to grouped Epithelial-Stromal Tumors. From a clinical standpoint, CN manifests as flank pain, hematuria, or other vague symptoms, but more often goes unnoticed until imaging for something else reveals it. Although uncommon, malignant transformation undergoing rare sarcomatous change has been reported.
Fig.1 A case study of pathological analysis of Adult Cystic Nephroma (CN). (Debnath A.,
et al., 2021)
Pathogenesis of Adult Cystic Nephroma (CN)
Although the precise pathogenic mechanisms of Adult Cystic Nephroma (CN) are not fully understood, several working hypotheses have been developed. One theory suggests a possible developmental genesis, perhaps linked to remnants of the ureteric bud or metanephric blastema. Estrogen and progesterone, especially during hormonal shifts like menopause, have been considered CNS-activating due to higher rates in perimenopausal women and the presence of hormone receptors in the CN stroma. Some genetic aspects, such as DICER1 mutations, have been documented in children, but less so for adult CNs. The collaborative interactions between these deviant developments alongside hormonal influences and certain inherited factors seem to form the likely framework for CN's pathogenesis.
Diagnostics Development for Adult Cystic Nephroma (CN)
Imaging-Based Diagnostics
Computed Tomography (CT) and Magnetic Resonance Imaging (MRI): These modalities are pivotal in the initial detection and characterization of CN. CT scans typically reveal a well-circumscribed, multicystic mass with variable septation and occasional calcifications. MRI offers superior soft tissue contrast, aiding in the differentiation of cystic from solid components and the assessment of lesion enhancement.
Histopathological Diagnostics
Histopathological Examination: Definitive diagnosis of Adult Cystic Nephroma (CN) relies on histopathological examination, which reveals a multiloculated cystic lesion lined by cuboidal to hobnail epithelium and separated by fibrous septae. Immunohistochemical staining for markers such as CD10, estrogen receptor (ER), and progesterone receptor (PR) aids in confirming the diagnosis.
Therapeutics Development for Adult Cystic Nephroma (CN)
Although no specific targeted therapies are currently approved for Adult Cystic Nephroma (CN), ongoing research explores the potential of hormonal manipulation, given the expression of ER and PR in Adult Cystic Nephroma (CN) stroma. Additionally, the role of angiogenesis inhibitors and mTOR inhibitors is being investigated in preclinical models.
Table 1. Therapeutics of Adult Cystic Nephroma (CN).
| Therapeutics |
Target |
Description |
Stage |
| Hormonal Modulation |
ER/PR (stromal component) |
Selective estrogen receptor modulator; theoretical use in ER/PR-positive stroma. |
Preclinical |
| Hormonal Modulation |
Estrogen synthesis |
Blocks estrogen production; potential role in hormone-sensitive stroma. |
Preclinical |
| CD10 Inhibitors |
CD10 |
Monoclonal antibodies or small-molecule inhibitors targeting CD10-positive stromal cells. |
Preclinical |
| PD-1/PD-L1 Inhibitors |
PD-1/PD-L1 |
Checkpoint inhibition for rare malignant transformation cases. |
Preclinical |
| Gene Therapy |
DICER1 |
Gene editing to restore DICER1 function (pediatric CN focus). |
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
At Protheragen, we are at the forefront of preclinical research, committed to advancing therapeutic and diagnostic solutions for challenging conditions like Adult Cystic Nephroma. Our comprehensive service portfolio is designed to address the unique complexities of rare diseases.
Disease Models
- N-Butyl-N-(4-hydroxybutyl)nitrosamine (BBNU) Induced Models
- Ferric Nitrilotriacetate (Fe-NTA) Induced Models
- VHL Gene Knockout Models
- FLCN Gene Knockout Models
- Transplantation Tumor Models
Protheragen offers an integrated suite of services spanning the entire preclinical development pipeline for Adult Cystic Nephroma. Recognizing the current reliance on surgical intervention and the unmet need for pharmaceutical solutions, our focus extends to discovering novel therapeutic targets and developing advanced diagnostic tools. If you are interested in our services, please feel free to contact us.
References
- Debnath, Abhik, et al. "Adult cystic nephroma masquerading urothelial carcinoma of a calyceal diverticulum." BMJ Case Reports 14.10 (2021): e245270.
- Demır, Hale, et al. "Mixed epithelial and stromal tumor family of kidney (adult cystic nephroma, mixed epithelial and stromal tumor): Retrospective clinicopathological evaluation." Turkish Journal of Pathology 38.3 (2022): 251.
All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.
