raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:319319

Renal medullary carcinoma

Type
Disease
Group
Disorder
Age of onset
Adult
Status
active

Renal medullary carcinoma is a rare, aggressive subtype of renal cell carcinoma characterized by a large, white or tan, firm, infiltrative tumor with microabscess-like foci centered in the renal medulla, typically presenting with hematuria, abdominal/flank pain, weight loss and fever. It is associated with sickle cell trait and disease and metastasis to the bones and lungs is common at time of diagnosis.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enRenal medullary carcinoma
frCarcinome rénal médullaire
deNierenkarzinom, medulläres
esCarcinoma medular renal
itCarcinoma della midollare renale
nlRenaal medullair carcinoom
ptCarcinoma medular renal
plRak rdzeniasty nerki
csMedulární karcinom ledviny
ukМедулярна карцинома нирки
zh肾髓样癌
trRenal medüller karsinom

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10C64NTBTattributedvalidated
ICD-112C90.0NTBTattributedvalidated
MONDO0006260Evalidated
UMLSC4049328Evalidated
GARD13175Evalidated
MedDRA10064886Evalidated

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

3

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
Annual incidence<1 / 1 000 000—EuropeClass onlyValidatedRARECARE surveillance of rare cancers in Europe[OTHER]

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