Polyembryoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult
- Status
- active
A rare malignant germ cell tumor characterized by predominant composition of embryoid bodies consisting of a central core of embryonal carcinoma cells, an amnion-like cavity, and a yolk sac tumor component. The tumor usually occurs as the dominant component of a mixed germ cell tumor, with teratoma being the most common associated element. It may manifest as an abdominal mass or with abdominal pain, menstrual irregularities, or precocious puberty in women, while men typically present with testicular enlargement. Serum alpha-fetoprotein and/or beta-human chorionic gonadotropin can be elevated.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Polyembryoma | |
| fr | Polyembryome | |
| de | Polyembryom | |
| es | Poliembrioma | |
| it | Poliembrioma | |
| nl | Polyembryoom | |
| pt | Poliembrioma | |
| pl | Wielozarodkowiak | |
| cs | Polyembryom | |
| uk | Поліембріома | |
| zh | 多胚瘤 | |
| tr | Poliembriyom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C80.9 | NTBT | attributed | validated |
| MONDO | 0015863 | E | validated | |
| UMLS | C0334518 | E | validated | |
| GARD | 9621 | 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
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | — | Europe | Class only | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
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
19 annotations · validated 2019-06-27| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0030338 | Abnormal circulating gonadotropin level | |
| HP:0010785 | Gonadal neoplasm | |
| HP:0030061 | Neuroectodermal neoplasm | |
| Occasional (29-5%) | ||
| HP:0031500 | Abdominal mass | |
| HP:0002027 | Abdominal pain | |
| HP:0045026 | Abnormal mediastinum morphology | |
| HP:0040231 | Abnormal onset of bleeding | |
| HP:0005107 | Abnormal sacrum morphology | |
| HP:0002585 | Abnormality of the peritoneum | |
| HP:0006254 | Elevated alpha-fetoprotein | |
| HP:0012288 | Neoplasm of head and neck | |
| Very rare (<4-1%) | ||
| HP:0003270 | Abdominal distention | |
| HP:0000818 | Abnormality of the endocrine system | |
| HP:0001945 | Fever | |
| HP:0003144 | Increased serum serotonin | |
| HP:0030088 | Increased serum testosterone level | |
| HP:0000858 | Irregular menstruation | |
| HP:0008236 | Isosexual precocious puberty | |
| HP:0000053 | Macroorchidism | |
Annotation source: 24819795[PMID]_29065103[PMID]_73579[PMID]_28429716[PMID]_26389316[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/180229
- This record as JSON: /data/d/180229.json