Ovarian dysgerminoma
Synonyms: Dysgerminoma of ovary · Dysgerminomatous germ cell cancer of the ovary
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Adolescent
- Status
- active
A rare malignant germ cell tumor of ovary characterized by a solid, lobulated mass, usually with a diameter of more than 10 cm, generally occurring in children or young women who present with abdominal pain or an abdominal mass and elevated serum LDH. It is bilateral in about 20% of cases, although the contralateral tumor may not be evident on macroscopic examination. Histopathologically, tumor cells are uniform rounded primitive germ cells with clear cytoplasm and macronucleoli, arranged in nests or cords separated by thin fibrous septa containing lymphocytes. Immunohistochemistry should be positive for OCT4 or SALL4, KIT (CD117), and/or D2-40.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Ovarian dysgerminoma | Dysgerminoma of ovary · Dysgerminomatous germ cell cancer of the ovary |
| fr | Dysgerminome de l'ovaire | Cancer germinal dysgerminomateux de l'ovaire · Dysgerminome ovarien |
| de | Dysgerminom des Ovars | Dysgerminom, ovarielles · Dysgerminomatöser Keimzelltumor des Ovars |
| es | Disgerminoma ovárico | Cáncer de células germinales disgerminomatosas de ovario · Disgerminoma de ovario |
| it | Disgerminoma ovarico | Disgerminoma dell'ovaio · Tumore disgerminale dell'ovaio a cellule germinali |
| nl | Dysgerminoom van ovarium | Dysgerminomateuze kiemcelkanker van ovarium · Dysgerminoom van eierstok · Ovarieel dysgerminoom |
| pt | Disgerminoma ovárico | Cancro do ovário disgerminomatoso de células germinativas · Disgerminoma do ovário |
| pl | Złośliwy guz jajnika z komórek rozrodczych | Nowotwór jajnika z komórek rozrodczych · Złośliwy rozrodczak jajnika |
| cs | Maligní dysgerminomatózní nádor ovaria ze zárodečných buněk | Maligní ovariální dysgerminom · Dysgerminomatózní rakovina ovaria ze zárodečných buněk |
| uk | Дисгермінома яєчників | Дисгермінома яєчника · Дисгерміноматозний герміногенний рак яєчника |
| zh | 恶性卵巢无性生殖细胞肿瘤 | 卵巢无性细胞瘤性生殖细胞癌 |
| tr | Overin habis disgerminomatöz germ hücre tümörü | Habis yumurtalık disgerminomu · Yumurtalıkta disgerminomatöz germ hücreli kanser |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C56 | NTBT | attributed | validated |
| ICD-11 | 2C73.1 | E | specific | validated |
| MONDO | 0020538 | E | validated | |
| UMLS | C0346185 | 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:35807Malignant germ cell tumor of ovary
rare neoplastic diseases; rare gynecological and obstetric diseases; rare transplant-related disorders
No children.
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 | <1 / 1 000 000 | 0.04 / 100 000 | Europe | Value and class | 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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/99912
- This record as JSON: /data/d/99912.json