raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:464359

Benign metanephric tumor

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
All ages
Status
active

A rare renal tumor characterized by a benign epithelial (metanephric adenoma), biphasic (metanephric adenofibroma) or renal stromal (metanephric stromal tumor) neoplasm. Metanephric adenoma mostly occurs in the fifth to sixth decade of life with distinct female predominance. It may be asymptomatic or present with abdominal pain, hematuria, and/or polycythemia. Metanephric adenofibroma has been described from infancy to young adulthood, potentially causing polycythemia or hematuria. Metanephric stromal tumor typically presents in infancy or childhood as an abdominal mass, sometimes manifestations of extrarenal vasculopathy such as hypertension or hemorrhage.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enBenign metanephric tumor
frTumeur métanéphrique bénigne
deMetanephrogener Tumor, benigner
esTumor metanéfrico benigno
itTumore metanefrico benigno
nlGoedaardige metanefrogene tumor
ptTumor metanéfrico benigno
plŁagodny guz metanefryczny
csBenigní metanefrický nádor
ukДоброякісна метанефрична пухлина
zh良性肾肿瘤
trSelim metanefrik tümör

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10D30.0NTBTattributedvalidated
ICD-112F35NTBTattributedvalidated
MONDO0018738Evalidated
UMLSC5681098Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

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Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyValidatedORPHANET_26870216[PMID]_27227914[PMID]

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

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HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

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Sources for this card