ORPHA:99916
Malignant Sertoli-Leydig cell tumor of the ovary
Synonyms: Androblastoma · Arrhenoblastoma · Ovarian Sertoli-Leydig cell cancer · Ovarian malignant Sertoli-Leydig cell tumor · Virilizing ovarian tumor
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
A rare malignant sex cord stromal tumor of ovary occuring typically in young women and characterized by manifestations of androgen excess (hirsutism, hair loss, amenorrhea, or oligomenorrhea), when functional.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Malignant Sertoli-Leydig cell tumor of the ovary | Androblastoma · Arrhenoblastoma · Ovarian Sertoli-Leydig cell cancer · Ovarian malignant Sertoli-Leydig cell tumor · Virilizing ovarian tumor |
| fr | Tumeur maligne de Sertoli-Leydig de l'ovaire | Androblastome · Arrhénoblastome · Cancer de Sertoli-Leydig de l'ovaire · Tumeur ovarienne maligne de Sertoli-Leydig · Tumeur virilisante de l'ovaire |
| de | Maligner Sertoli-Leydig-Zell-Tumor des Ovars | Androblastom · Arrhenoblastom · Ovarialtumor, virilisierender · Sertoli-Leydig Zell-Tumor, ovarieller · Sertoli-Leydig-Zell-Tumor, maligner ovarieller · Sertoli-Zell-Karzinom des Ovars |
| es | Tumor maligno de las células de Sertoli-Leydig de ovario | Androblastoma · Arrenoblastoma · Cáncer ovárico de células de Sertoli-Leydig · Tumor ovárico maligno de células de Sertoli-Leydig · Tumor ovárico virilizante |
| it | Tumore maligno dell'ovaio a cellule del Sertoli-Leydig | Androblastoma · Arrenoblastoma · Cancro ovarico a cellule del Sertoli-Leydig · Tumore ovarico maligno a cellule del Sertoli-Leydig · Tumore ovarico virilizzante |
| nl | Maligne Sertoli-Leydigceltumor van ovarium | Androblastoom · Arrhenoblastoom · Kwaadaardige Sertoli-Leydigceltumor van eierstok · Ovariële Sertoli-Leydigcelkanker · Ovariële maligne Sertoli-Leydigceltumor · Viriliserende ovariumtumor |
| pt | Tumor ovárico de células de Sertoli-Leydig | |
| pl | Złośliwy guz jajnika z komórek Sertoliego i Leydiga | Androblastoma · Arrenoblastoma/Jądrzak · Nowotwór jajnika z komórek Sertoliego i Leydiga · Wirilizujący guz jajnika |
| cs | Maligní nádor ovaria ze Sertoliho-Leydigových buněk | Androblastom · Arhenoblastom · Rakovina vaječníku ze Sertoliho-Leydigových buněk · Maligní nádor ze Sertoliho-Leydigových buněk · Virilizující nádor vaječníků |
| uk | Злоякісна Сертолі-Лейдіга-клітинна пухлина яєчника | Андробластома · Артенобластома · Злоякісна клітинна пухлина яєчників Сертолі-Лейдіга · Клітинний рак яєчників Сертолі-Лейдіга · Вірилізуюча пухлина яєчника |
| zh | 卵巢恶性支持-间质细胞瘤 | 卵巢恶性支持间质细胞肿瘤 |
| tr | Overin habis Sertoli-Leydig hücre tümörü | Androblastoma · Arenoblastom · Virilize yumurtalık tümörü · Yumurtalık Sertoli-Leydig hücre kanseri · Yumurtalık habis Sertoli-Leydig hücre tümörü |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C56 | NTBT | attributed | validated |
| ICD-11 | 2C73.Y | NTBT | index | validated |
| MONDO | 0020542 | E | validated | |
| UMLS | C5574973 | E | validated | |
| GARD | 5495 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:35808Malignant sex cord stromal tumor of ovary
rare neoplastic diseases; rare gynecological and obstetric diseases
No children.
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Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| DICER1 | Major susceptibility factor in | Assessed | HGNC:17098 | ENSG00000100697 | 606241 | 14q32.13 | dicer 1, ribonuclease III | 24761742[PMID]_21205968[PMID]_21882293[PMID] |
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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.
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HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/99916
- This record as JSON: /data/d/99916.json