Acquired cystic disease-associated renal cell carcinoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare subtype of renal cell carcinoma, ocurring in the context of end-stage kidney disease and acquired cystic kidney disease, characterized by a usually well circumscribed, solid, multifocal, bilateral tumor with inter- or intracellular microlumen formation (leading to cribiform architecture). Tumors are often diagnosed incidentally in early stages, although complications caused by renal cysts (dull flank or abdominal pain, fever) or renal parenchymal bleeding may mask the underlying neoplastic process. Most have an indolent behavior.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acquired cystic disease-associated renal cell carcinoma | |
| fr | Carcinome rénal associé à une maladie kystique acquise | |
| de | Erworbene Nierenkrankheit mit assoziiertem Nierenzellkarzinom | |
| es | Carcinoma de células renales adquirido asociado con una enfermedad quística | |
| it | Malattia cistica acquisita associata al carcinoma delle cellule renali | |
| nl | Niercelcarcinoom geassocieerd met verworven cystische ziekte | |
| pt | Carcinoma de células renais associado a doença renal quística adquirida | |
| pl | Nabyta choroba torbielowata nerek towarzysząca rakowi nerkowokomórkowemu | |
| cs | Získané onemocnění ledvin asociované s renálním karcinomem | |
| uk | Набута нирковоклітинна карцинома, асоційована з кістозною хворобою | |
| zh | 获得性囊性疾病相关性肾细胞癌 | |
| tr | Edinsel kistik hastalık-ilişkili renal hücreli karsinom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C64 | NTBT | attributed | validated |
| MONDO | 0018449 | E | validated | |
| UMLS | C4707179 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:217071Renal cell carcinoma
rare renal diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/404514
- This record as JSON: /data/d/404514.json