Tubulocystic renal cell carcinoma
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult, Elderly
- Status
- active
Tubulocystic renal cell carcinoma is an extremely rare subtype of renal cell carcinoma most frequently characterized by a small, solitary, well-circumscribed, unencapsulated renal tumor composed of multiple small to medium-sized cysts with a white or gray, spongy ("bubble wrap-like") cut surface. Patients are usually asymptomatic or could manifest with abdominal pain, abdominal distension and/or hematuria. Progression, recurrence and metastasis rarely occur although lymph node, bone, pleura and liver metastases have been reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Tubulocystic renal cell carcinoma | |
| fr | Carcinome rénal tubulokystique | |
| de | Nierenzellkarzinom, tubulozystisches | |
| es | Carcinoma renal túbulo-quístico | |
| it | Carcinoma tubulocistico | |
| nl | Tubulocystisch niercelcarcinoom | |
| pt | Carcinoma de células renais tubuloquístico | |
| pl | Rak cewkowo-torbielowaty | |
| cs | Tubulocystický karcinom | |
| uk | Тубулоцистозний нирковоклітинний рак | |
| zh | 管状囊性肾癌 | |
| tr | Tubulosistik renal hücreli karsinom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C64 | NTBT | attributed | validated |
| ICD-11 | 2C90.0 | NTBT | attributed | validated |
| ICD-11 | XH7K79 | E | specific | validated |
| MONDO | 0017890 | E | validated | |
| UMLS | C4288091 | 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/319325
- This record as JSON: /data/d/319325.json