Yolk sac tumor
Synonyms: Endodermal sinus tumor
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult
- Status
- active
A rare germ cell tumor characterized by multiple patterns reflecting endodermal extraembryonal differentiation (secondary yolk sac and allantois) or endodermal somatic tissues (intestine, liver, and mesenchyme). The tumors most commonly occur in the second or third decade of life. They are typically located in the gonads, occasionally also in other regions. Patients present with a pelvic mass and/or abdominal pain (females) or an often painless, unilateral testicular mass (males). Elevated serum alpha fetoprotein is a common laboratory finding.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Yolk sac tumor | Endodermal sinus tumor |
| fr | Tumeur du sac vitellin | Tumeur du sinus endodermique |
| de | Dottersacktumor | Tumor des Sinus endodermalis |
| es | Tumor de saco vitelino | Tumor de seno endodérmico |
| it | Tumore del sacco vitellino | Tumore del seno endodermico |
| nl | Dooierzaktumor | Tumor van endodermale sinus |
| pt | Tumor do saco vitelino | Tumor do seio endodérmico |
| pl | Guz pęcherzyka żółtkowego | guz pęcherzyka żółtkowego |
| cs | Nádor ze žloutkového váčku | Endodermální nádor dutiny nosní |
| uk | Пухлина жовткового мішка | Ендодермальна пухлина пазухи |
| zh | 卵黄囊瘤 | 内胚窦瘤 |
| tr | Yolk kesesi tümörü | Endodermal sinüs tümörü |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 273300 | NTBT | validated | |
| ICD-10 | C56 | NTBT | index | validated |
| ICD-10 | C62.9 | NTBT | index | validated |
| ICD-11 | 2C73.Y | NTBT | index | validated |
| ICD-11 | 2F34 | NTBT | index | validated |
| ICD-11 | XH09W7 | E | specific | validated |
| MONDO | 0005744 | E | validated | |
| MeSH | D018240 | E | validated | |
| UMLS | C0014145 | E | validated | |
| GARD | 348 | E | validated | |
| MedDRA | 10048251 | 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:99913Extragonadal non-dysgerminomatous germ cell tumor
rare neoplastic diseases; rare transplant-related disorders
Children (1):
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
| Annual incidence | 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/876
- This record as JSON: /data/d/876.json