ORPHA:2665
Congenital mesoblastic nephroma
Publications
897
82.8th 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 found
No HPO disease–phenotype associations via Monarch for these Mondo IDs
- Animal modelNot found
No Alliance genotype “model of” associations via Monarch for these Mondo IDs
- Orphan designationNot found
No FDA or EMA orphan-drug designation matched this disease via UMLS or preferred name Source
- 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)
None returned for this Mondo ID. That often means “not linked under this ID,” not “no clinical features.”
Animal models (Monarch / Alliance)
None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.
Monarch fetch 2026-07-29
Therapies
Designations, candidates, and chemicals
FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.
Orphan designation (FDA · EMA)
No designation matched this disease via UMLS or preferred name on the FDA OOPD mirror or EMA orphan register. Absence here is not proof that none exists under another wording.
Open Targets candidates
No drugs or clinical candidates returned for this Mondo ID on Open Targets.
CTD chemicals (MyDisease.info)
No CTD chemical associations returned for this Mondo ID.
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=3967) is 59.
897 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).
474 in the last 10 years · medium confidence · 82.8th 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 11 September 2026 · last trial check 11 September 2026
No matched interventional trials. This is true for 77.2% of diseases in the trials denominator (5501 of 7126). Here are the researchers publishing on it.
medium confidence · 38.6th 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
Other registries (secondary)
Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.
raw 60 · after dedupe 60 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 60 · dropped 0 · fetched 2026-07-29
Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri
No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).
Uncertain / not reviewed (60)
- ctis·2026-526821-16-00·Authorised·Melanoma-TBK1-inhibitor enhanced Immuno Therapy with Checkpoint Inhibitors: a Phase IB/II Trial in Unresectable Stage III or Stage IV Cutaneous Melanoma
skipped — LLM skipped (--skip-llm)
- ctis·2025-523067-38-01·Authorised·Phase 1/1b Study of BMS-986463 in Advanced Malignant Tumors
skipped — LLM skipped (--skip-llm)
- ctis·2025-523482-23-00·Authorised·PITALC. Pilot study on the effect of PITAC (Pressurized IntraThoracic Aerosol Chemotherapy) by videothoracoscopy in patients with malignant pleural effusion in the light of the results of a retrospective historical cohort treated with talc pleurodesis by videothoracoscopy
skipped — LLM skipped (--skip-llm)
- ctis·2025-524403-80-00·Authorised·A Phase 3, Randomized, Open-Label, Multicenter Study Evaluating the Efficacy of KITE-753 Versus Axicabtagene Ciloleucel in Participants with Relapsed or Refractory Large B-Cell Lymphoma After First-Line Therapy
skipped — LLM skipped (--skip-llm)
- ctis·2025-523923-22-00·Authorised·A Phase 2, Open-Label, Single-Arm Study of Lirafugratinib in Patients With Previously Treated, Unresectable, Locally Advanced or Metastatic Solid Tumors (Excluding Cholangiocarcinoma) With FGFR2 Fusion or Rearrangement
skipped — LLM skipped (--skip-llm)
- ctis·2024-510793-24-00·Authorised·Feasibility of fluorescence imaging with bevacizumab-800CW during bronchoscopy in patients with a pulmonary lesion considered to be malignant - DFLECT I
skipped — LLM skipped (--skip-llm)
- ctis·2026-526388-39-00·Authorised·Calcium Electroporation for Urinary Bladder Tumors: A First-in-Human Feasibility, Safety, and Early Response Trial - The CALCIFER Trial
skipped — LLM skipped (--skip-llm)
- ctis·2025-524623-50-00·Authorised·A Phase 1/2 Open-Label, Multicenter Study of RAS(ON) inhibitors in Combination with Ivonescimab with or without Other Anti-Cancer Agents in Patients with Solid Tumors
skipped — LLM skipped (--skip-llm)
- ctis·2025-524299-44-00·Authorised·First study evaluating the safety and dosage of YG-01 in patients with a malignant brain tumor
skipped — LLM skipped (--skip-llm)
- ctis·2025-524687-38-00·Authorised·Phase 1/1b Study of RNDO-564 in Participants with Advanced Urothelial Cancer
skipped — LLM skipped (--skip-llm)
- ctis·2025-523432-39-01·Authorised·A Phase 1/2, Open-label Study of VS-7375, a KRAS G12D (ON/OFF) Inhibitor, as Monotherapy and in Combination, in Patients with Advanced KRAS G12D-Mutant Solid Tumors
skipped — LLM skipped (--skip-llm)
- ctis·2025-522845-23-00·Authorised·An open-label, non-randomized, single center, single dose, exploratory phase II trial of FG001 (an imaging agent) in combination with transoral robotic surgery for oropharyngeal squamous cell carcinoma and neck dissection surgery for oral squamous cell carcinoma with regional lymph node metastases
skipped — LLM skipped (--skip-llm)
- ctis·2025-523590-42-00·Authorised, recruiting·A Phase 1/2, Multicenter, Open-label, Dose-escalation, and Dose-expansion Study to Evaluate the Safety, Pharmacokinetics, Pharmacodynamic, and Antitumor Activity of CR-001 in Adult Participants with Locally Advanced or Metastatic Solid Tumors.
skipped — LLM skipped (--skip-llm)
- ctis·2025-523276-23-00·Authorised·A Phase I/II open label study of DJI136, a DLL3-targeted CAR-T therapy, in adult patients with ES-SCLC
skipped — LLM skipped (--skip-llm)
- ctis·2025-522339-32-00·Authorised, recruiting·LIGHTBEAM-U01 Substudy 01D: A Phase 1b/2 Substudy to Evaluate the Safety and Efficacy of Ifinatamab Deruxtecan in Pediatric Participants With Relapsed or Refractory Solid Tumors
skipped — LLM skipped (--skip-llm)
- ctis·2025-524620-22-00·Authorised, recruiting·A 2-part phase 1/2 open-label trial evaluating the safety, tolerability, pharmacokinetics, pharmacodynamics, and efficacy of ODM-212 in combination with anti-cancer therapy in participants with advanced solid tumours
skipped — LLM skipped (--skip-llm)
- ctis·2025-524192-24-00·Authorised, ongoing·A Phase I, Open-label, Multi-center, First-in-human Trial to Investigate E303, in Participants with Advanced Refractory Solid Tumors
skipped — LLM skipped (--skip-llm)
- ctis·2025-521891-78-00·Authorised·IMMUNOTIME « EVALUATION OF THE EFFECT OF TIME OF ADMINISTRATION ON THE ACTIVITY ON FIRST LINE OF PEMBROLIZUMAB IN ASSOCIATION WITH CARBOPLATIN PEMETREXED IN METASTATIC NON-SQUAMOUS LUNG CANCER. A RANDOMISED MULTICENTER PHASE III TRIAL»
skipped — LLM skipped (--skip-llm)
- ctis·2024-514089-37-00·Authorised, ongoing·GLO-Surgery: Indocyanine Green and near infrared fLuorescence in paediatric Oncology surgery
skipped — LLM skipped (--skip-llm)
- ctis·2025-523760-21-00·Authorised, ongoing·A Study of GEN1106 in Participants with Solid Tumors
skipped — LLM skipped (--skip-llm)
- ctis·2025-524814-27-00·Authorised·Phase I, first-in-human clinical trial to evaluate safety of infusion of STAb-T19 cells: genetically modified T lymphocytes secreting αCD19xαCD3 bispecific antibodies for malignant B-cell neoplasms
skipped — LLM skipped (--skip-llm)
- ctis·2025-523474-16-00·Authorised, ongoing·A Phase 1/2, Open-label Study of Oral S241656 (BDTX-4933) as Monotherapy and in Combination with Other Anti-Cancer Therapies in Patients with KRAS, BRAF and Other Selected RAS/MAPK Mutation-Positive Malignancies
skipped — LLM skipped (--skip-llm)
- ctis·2025-521911-38-00·Authorised, recruiting·An open-label, multi-center, phase I/II study of GVV858 as a single agent and in combination with endocrine therapy in patients with advanced hormone receptor positive, HER2-negative breast cancer and other advanced solid tumors
skipped — LLM skipped (--skip-llm)
- ctis·2025-521000-22-00·Authorised, recruiting·A clinical trial to learn about the effects of DAK539 on the blood levels of repaglinide, midazolam, drospirenone, and ethinyl estradiol in people with advanced cancer
skipped — LLM skipped (--skip-llm)
- ctis·2025-521571-30-00·Authorised·PREV-CART - A multicentric phase III randomized clinical trial open-label, comparing immunoglobulin replacement therapy (IgRT) and antibiotic prophylaxis (AP) in patients treated by CD19-targeted Chimeric Antigen Receptor (CAR)T Cells for a B cell acute lymphoblastic leukemia or a B cell lymphoma
skipped — LLM skipped (--skip-llm)
Where to find support
Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.
Orphanet entry for Congenital mesoblastic nephroma — check Associations / patient organisations on that page.
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