Malignant granulosa cell tumor of the ovary
Synonyms: Granulosa cell cancer · Granulosa cell malignant tumor
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial
- Age of onset
- Adolescent, Adult, Elderly
- Status
- active
A rare malignant sex cord stromal tumor of ovary arising from the granulosa cells of the ovary, which occurs in peri and post menopausal women, and that presents with abnormal vaginal bleeding, abdominal pain and distension. The tumor is frequently unilateral, estrogen secreting, and has a slow natural history and a tendency to relapse long after the initial diagnosis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Malignant granulosa cell tumor of the ovary | Granulosa cell cancer · Granulosa cell malignant tumor |
| fr | Tumeur maligne de la granulosa de l'ovaire | Cancer de la granulosa · Tumeur maligne de la granulosa |
| de | Maligner Granulosazelltumor des Ovars | Granulosazellkarzinom · Granulosazelltumor, maligner |
| es | Tumor maligno de células de la granulosa de ovario | Cáncer de células de la granulosa · Tumor maligno de células de la granulosa |
| it | Tumore ovarico maligno a cellule della granulosa | Carcinoma a cellule della granulosa · Tumore a cellule della granulosa · Tumore maligno delle cellule della granulosa |
| nl | Maligne granulosaceltumor van ovarium | Granulosacelkanker · Kwaadaardige granulosaceltumor · Kwaadaardige granulosaceltumor van eierstok · Maligne granulosaceltumor |
| pt | Tumor de células granulosas | |
| pl | Złośliwy ziarniszczak jajnika | Ziarniszczak · Ziarniszczak złośliwy |
| cs | Maligní nádor ovaria z buněk granulózy | Rakovina z buněk granulózy · Maligní nádor z buněk granulózy |
| uk | Злоякісна гранульозоклітинна пухлина яєчника | Гранулезоклітинний рак · Гранулезоклітинна злоякісна пухлина |
| zh | 卵巢恶性颗粒细胞瘤 | 颗粒细胞恶性肿瘤 |
| tr | Overin maligant granüloza hücre tümörü | Granüloza hücreli kanser · Granüloza hücreli habis tümör |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C56 | NTBT | attributed | validated |
| ICD-11 | 2C73.2 | E | specific | validated |
| MONDO | 0020541 | E | validated | |
| MeSH | D006106 | E | validated | |
| UMLS | C0346175 | 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:35808Malignant sex cord stromal tumor of ovary
rare neoplastic diseases; rare gynecological and obstetric diseases
No children.
Genes
2 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| FOXL2 | Disease-causing somatic mutation(s) in | Assessed | HGNC:1092 | ENSG00000183770 | 605597 | 3q22.3 | forkhead box L2 | 19516027[PMID] |
| DICER1 | Major susceptibility factor in | Assessed | HGNC:17098 | ENSG00000100697 | 606241 | 14q32.13 | dicer 1, ribonuclease III | 24761742[PMID]_21205968[PMID]_21882293[PMID] |
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.12 / 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/99915
- This record as JSON: /data/d/99915.json