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Start free with EleplanNephroblastoma
ORPHA:654Disease
Also called Renal embryonic tumor · Wilms tumor
What it is
A rare malignant renal tumor, typically affecting the pediatric population, characterized by an abnormal proliferation of cells that resemble the kidney cells of an embryo (metanephroma), leading to the term embryonal tumor.
Key facts
- Prevalence
- 1-9 / 100 000 (Europe)
- Age of onset
- Childhood
- Inheritance
- Autosomal dominant, Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Sometimes5–29%
20- Abnormal uterus morphology
- Anemia
- Aniridia
- Cryptorchidism
- Ectopic kidney
- Fever
- Hemihypertrophy
- Horseshoe kidney
and 12 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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