Multiloculated renal cyst
Synonyms: Multilocular cyst of the kidney · Multilocular renal cyst
- Type
- Morphological anomaly
- Group
- Disorder
- Age of onset
- Adult, Elderly, Infancy
- Status
- active
A rare benign renal tumor characterized by a typically unilateral, solitary, multiloculated cystic mass consisting of small, non-communicating cysts with flat, cuboidal, or hobnail epithelial lining, separated by fibrous septa which may have an ovarian stroma-like appearance or be paucicellular. The tumor is surrounded by a thick fibrous capsule and does not contain solid areas or necrosis. Patients may be asymptomatic or present with a palpable abdominal mass and/or abdominal or flank pain. Age distribution is bimodal, the typical age of onset being either below five or between 40 and 70 years of age.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Multiloculated renal cyst | Multilocular cyst of the kidney · Multilocular renal cyst |
| fr | Kyste multiloculaire du rein | Kyste rénal multiloculaire · Néphrome kystique |
| de | Nierenzysten, septierte | Septierte Zysten der Niere |
| es | Quiste renal múltiple | Quiste multilocular del riñón · Quiste renal multilocular |
| it | Cisti renale multiloculare | Cisti multiloculare del rene |
| nl | Multiloculaire renale cyste | Multiloculaire cyste van de nier · Multiloculaire niercyste |
| pt | Quisto renal, multiloculado | |
| pl | Wielokomorowa torbiel nerki | Wielokomorowa torbiel nerki · Wielokomorowa torbiel nerki |
| cs | Mnohočetné renální cysty | Mnohočetné cysty ledvin |
| uk | Мультилокулярна кіста нирки | Мультилокулярна кіста нирки · Мультилокулярна кіста нирки |
| zh | 多囊性肾囊肿 | 肾脏多房囊肿 |
| tr | Multiloküle böbrek kisti | Multiloküler böbrek kisti · Böbreğin multiloküler kisti |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D30.0 | NTBT | attributed | validated |
| MONDO | 0019983 | E | validated | |
| UMLS | C0431719 | 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 (2):
- ORPHA:506213Rare disorder potentially indicated for kidney transplant
rare transplant-related disorders - ORPHA:93619Rare renal tumor
rare renal diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/97366
- This record as JSON: /data/d/97366.json