ORPHA:35808
Malignant sex cord stromal tumor of ovary
Synonyms: Malignant ovarian SCST · Malignant ovarian sex cord-stromal tumor
- Type
- Category
- Group
- Group of disorders
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
Malignant sex cord stromal tumor (SCST) of ovary is a rare ovarian cancer arising from granulosa, theca, sertoli and leydig cells or stromal fibroblasts, occurring at any age and presenting with abdominal or pelvic mass, and characterized (with the exception of fibroma) by the production of sex steroids resulting in manifestations of hormone excess, with a relatively favorable prognosis.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Malignant sex cord stromal tumor of ovary | Malignant ovarian SCST · Malignant ovarian sex cord-stromal tumor |
| fr | Tumeur maligne des cordons sexuels et du stroma de l'ovaire | SCST ovarienne maligne · Tumeur ovarienne maligne stromale des cordons sexuels |
| de | Maligne Keimstrang-Stroma-Tumoren des Ovars | OSCST |
| es | Tumor maligno del estroma de los cordones sexuales de los ovarios | SCST maligno ovárico · Tumor estromal maligno de los cordones sexuales ováricos |
| it | Tumore maligno dello strome e dei cordoni sessuali dell'ovaio | Cancro ovarico dello stroma e dei cordoni sessuali · SCST ovarico maligno · Tumore ovarico maligno dello stroma e dei cordoni sessuali |
| nl | Maligne sex cord-stromale tumor van ovarium | Kwaadaardige sex cord-stromale tumor van eierstok · Maligne ovariële SCST · Maligne ovariële sex cord-stromale tumor |
| pt | Tumor maligno do estroma dos cordões sexuais do ovário | SCST ovárico maligno |
| pl | Złośliwy guz jajnika z komórek sznurów płciowych i podścieliska | Złośliwy SCST jajnika |
| cs | Maligní sex-cord stromální tumor ovaria | Maligní ovariální SCST · Maligní ovariální sex cord-stromální tumor |
| uk | Злоякісна стромальна пухлина статевого канатика яєчника | Злоякісний рак яєчника · Злоякісна пухлина статевого канатика-строми яєчника |
| zh | 恶性卵巢性索间质肿瘤 | 恶性卵巢性索-间质瘤 |
| tr | Yumurtalıkların habis cinsiyet kord stromal tümörü | Habis yumurtalık SCST · Habis yumurtalık cinsiyet kord stromal tümörü |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 0018172 | E | validated | |
| MONDO | 18172 | E | validated | |
| UMLS | C1334609 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:398940Malignant non-epithelial tumor of ovary
rare neoplastic diseases; rare gynecological and obstetric diseases
4
Genes
0 directOrphanet records no gene directly on this code.
5
Prevalence and incidence
27 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 1.85 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.13 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.1 / 100 000 | United States | Value and class | Validated | 15863154[PMID] |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
| Annual incidence | <1 / 1 000 000 | 0.05 / 100 000 | Finland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.043 / 100 000 | Iceland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.074 / 100 000 | Ireland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.069 / 100 000 | Italy | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.094 / 100 000 | Malta | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.045 / 100 000 | Portugal | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.063 / 100 000 | Slovenia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.039 / 100 000 | Spain | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.073 / 100 000 | Switzerland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.093 / 100 000 | Netherlands | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.089 / 100 000 | United Kingdom | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.133 / 100 000 | Austria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.102 / 100 000 | Belgium | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.367 / 100 000 | Bulgaria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.152 / 100 000 | Croatia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.314 / 100 000 | Czech Republic | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.296 / 100 000 | Estonia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.135 / 100 000 | Germany | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.401 / 100 000 | Latvia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.236 / 100 000 | Lithuania | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.12 / 100 000 | Norway | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.232 / 100 000 | Poland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.212 / 100 000 | Slovakia | 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.
6
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
7
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/35808
- This record as JSON: /data/d/35808.json