ORPHA:2665
Congenital mesoblastic nephroma
Publications
897
89.8th percentile
Trials
0
Interventional, condition-specific
Researchers
1,253
Distinct authors in sample
Gene link
—
Readiness
1/6
Stages with a signal
Clinical definition (Orphanet)
A rare renal tumor characterized by a unilateral, solitary, well demarcated, mesenchymal/myofibroblastic neoplasm occurring in very young children. Histopathologically, three subtypes (classic, cellular, and mixed) can be distinguished. The tumor most commonly involves the renal sinus and is typically discovered as a palpable abdominal mass. Patients may also present with hypertension or hematuria, rarely with hypercalcemia or hyperreninemia. presentation, usually with polyhydramnios, is not infrequent. The most important prognostic factor is completeness of surgical resection. Overall, malignant potential is low and clinical outcome favorable.
Cross-references
Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.
- MONDO:0017043
- UMLS:C1332965
- NCIT:C6569
Additional Mondo synonyms (4)
CMN · CMn · congenital mesoblastic nephroma · stromal nephroma, malignant
Research stages
Trial readiness signals
Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”
1/6 stages with a signal
Research-stage checklist from open sources (GenCC, literature, Monarch when enriched, ClinicalTrials.gov). Not a prognosis or care recommendation.
- Gene identifiedNot found
No GenCC disease–gene assertion in this build
- LiteraturePresent
897 matched papers (474 in last 10 years) Source
- Phenotype characterisedNot checked
Not yet enriched from Monarch / HPO
- Animal modelNot checked
Not yet enriched from Monarch / Alliance
- Orphan designationNot checked
FDA/EMA orphan-drug designation not enriched yet
- Interventional trialNot found
No matched interventional trial under our ClinicalTrials.gov rules
Biology
Genes and phenotypes
Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.
Do we know what causes it?
Not yet — the cause hasn't been pinned down in GenCC.
No strong gene–disease assertion joined for this Orphanet entity.
Phenotypes (Monarch / HPO)
Not enriched in this build — Monarch phenotype joins were not run for this record.
Animal models (Monarch / Alliance)
Not enriched in this build.
Literature
Is anyone studying this?
897
897 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.
897 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).
474 in the last 10 years · medium confidence · 89.8th percentile (publications denominator)
Phrase hits: 897 · MeSH hits: 0
Who's working on it?
1,253
Distinct author names in 200 sampled papers — named people below.
Who's working on it?
No interventional trial matched this name; these authors publish on it in the sampled literature — a practical starting point for contact.
- 01van den Heuvel-Eibrink MM9 papers · 2026
Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Papers in Europe PMC - 02Graf N7 papers · 2026
Department of Pediatric Oncology & Hematology, Saarland University Medical Center and Saarland University Faculty of Medicine, Homburg, Germany.
Papers in Europe PMC - 03de Krijger RR6 papers · 2025
Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
Papers in Europe PMC - 04Rudzinski ER6 papers · 2025
Department of Laboratories, Seattle Children's Hospital, Seattle, WA, USA.
Papers in Europe PMC - 05Wang H6 papers · 2026
Department of Urology, the Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China.
Papers in Europe PMC - 06Alaggio R5 papers · 2026
Pathology Unit, Bambino Gesù Children's Hospital, Rome, Italy.
Papers in Europe PMC - 07Davis JL5 papers · 2022
Department of Pathology, Oregon Health & Science University, Portland, OR, USA. davisjes@ohsu.edu.
Papers in Europe PMC - 08Furtwängler R5 papers · 2026
Department of Pediatric Hematology and Oncology, University Medical Center Homburg/Saar, Homburg, Germany.
Papers in Europe PMC - 09Chang KTE4 papers · 2025
Department of Pathology and Laboratory Medicine, KK Women's and Children's Hospital, Singapore.
Papers in Europe PMC - 10Chen L4 papers · 2022
Department of Radiology, The First Affiliated Hospital, Sun Yat-sen University, 58th, The Second Zhongshan Road, Guanzhou, China.
Papers in Europe PMC
Clinical research
Is a treatment being tested?
0
interventional trials for this specific condition
No interventional trial testing a treatment matched this specific condition name. 3 observational studies did — shown below because natural-history and cohort work can be an important step toward a trial.
Data as of 27 July 2026
No matched interventional trials. This is true for 73% of diseases in the trials denominator (5114 of 7003). Here are the researchers publishing on it.
medium confidence · 36.5th percentile (trials denominator)
Recruiting interventional trials
From the matched ClinicalTrials.gov set
No interventional trial testing a treatment was found for this specific condition name on ClinicalTrials.gov.
Broader category mesoblastic nephroma also has no matched interventional trial. See who's working on it — people publishing on this disease are often the practical next contact when no trial is listed.
Broader category: mesoblastic nephroma
0
Interventional trials for the parent category, exclusive of NCT IDs already counted above. Eligibility for this subtype is not guaranteed.
Parent-category matching found a broader label but no interventional trials under it. How we count trials.
Observational and natural-history studies
3 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.
Recruiting or not-yet-recruiting
- NCT02402244·RECRUITING·Project: Every Child for Younger Patients With Cancer
Conditions: Adrenal Gland Pheochromocytoma · Carcinoma In Situ · Central Nervous System Neoplasm · Childhood Immature Teratoma·Matched via name phrase
Where to find support
We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:
India — NPRD
Last verified 2026-07-26This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.
Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.
How we counted this
Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.
"Congenital mesoblastic nephroma" OR "stromal nephroma, malignant"
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Congenital mesoblastic nephroma" OR "stromal nephroma, malignant"
Study-type breakdown: 0 interventional · 3 observational · 0 expanded access. Only interventional studies enter the trial headline.
Parent-category trials query:
"mesoblastic nephroma"
Query health: ok — strategies attempted: phrase; with hits: phrase
Run this search on ClinicalTrials.gov
Synonyms dropped by stoplist: CMN
Confidence reasoning
- Preferred label is multi-word and distinctive
- 1 synonym(s) dropped by stoplist (may under-count)
- No label/synonym collisions with other diseases in this corpus
Ingested 2026-07-26T20:47:53.787Z