Mucinous tubular and spindle cell renal carcinoma
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Elderly
- Status
- active
Mucinous tubular and spindle cell renal carcinoma is a rare subtype of renal cell carcinoma characterized, histologically, by tubular architecture and sheets of spindle cells embedded in a mucinous/myxoid stroma and, macroscopically, by a solid, generally well-circumscribed, partially encapsulated tumor of variable size, with a homogenously colored, bulging cut surface, occassionally containing areas of hemorrhage or necrosis, usually located in the cortex. Patients can present abdominal/flank pain, adbominal mass and/or hematuria, however most are asymptomatic and tumor is discovered incidentally. Indolent behavior is frequent and association with nephrolithiasis and end-stage kidney disease has been noted.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Mucinous tubular and spindle cell renal carcinoma | |
| fr | Carcinome rénal tubulo-mucineux à cellules fusiformes | |
| de | Muzinöses tubuläres und spindelzelliges Karzinom | |
| es | Carcinoma renal mucinoso tubular y de células fusiformes | |
| it | Carcinoma renale tubulare mucinoso a cellule fusiformi | |
| nl | Mucineus tubulair en spindelcel renaal carcinoom | |
| pt | Carcinoma tubular mucinoso e de células fusiformes | |
| pl | Rak śluzowo-cewkowy i wrzecionowatokomórkowy | |
| cs | Mucinózní tubulární a vřetenobuněčný karcinom | |
| uk | Муцинозний тубулярний і веретеноклітинний рак нирки | |
| zh | 肾脏黏液小管状和梭形细胞癌 | |
| tr | Müsinöz tübüler ve iğsi hücreli renal 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 |
| MONDO | 0003011 | E | validated | |
| UMLS | C4707257 | 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/319322
- This record as JSON: /data/d/319322.json