Ovarian fibrothecoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Ovarian fibrothecoma is a rare, benign, sex cord-stromal neoplasm, with a typically unilateral location in the ovary, characterized by mixed features of both fibroma and thecoma. Patients may be asymptomatic or may present with pelvic/abdominal pain and/or distension and, occasionally, with post-menopausal bleeding. Large tumors (>10cm) are often associated with pleural effusion and ascites (the Meigs syndrome triad).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Ovarian fibrothecoma | |
| fr | Fibrothécome ovarien | |
| de | Fibrothekom des Ovars | |
| es | Fibrotecoma ovárico | |
| it | Fibrotecoma ovarico | |
| nl | Ovarieel fibrothecoom | |
| pt | Fibrotecoma ovárico | |
| pl | Fibrotekoma jajnika | |
| cs | Ovariální fibrothékom | |
| uk | Фіброміома яєчників | |
| zh | 卵巢纤维膜瘤 | |
| tr | Over fibrotekom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D27 | NTBT | attributed | validated |
| ICD-11 | 2F32.Y | NTBT | index | validated |
| MONDO | 0017802 | E | validated | |
| UMLS | C4707356 | 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:97293Rare benign ovarian tumor
rare neoplastic diseases; rare gynecological and obstetric diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
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
15 annotations · validated 2016-01-20| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0003270 | Abdominal distention | |
| HP:0002027 | Abdominal pain | |
| HP:0000137 | Abnormality of the ovary | |
| HP:0100608 | Metrorrhagia | |
| HP:0010618 | Ovarian fibroma | |
| HP:0002202 | Pleural effusion | |
| Frequent (79-30%) | ||
| HP:0001541 | Ascites | |
| HP:0002586 | Peritonitis | |
| Occasional (29-5%) | ||
| HP:0003117 | Abnormality of circulating hormone level | |
| HP:0030126 | Abnormality of the endometrium | |
| HP:0006756 | Diffuse leiomyomatosis | |
| HP:0100244 | Fibrosarcoma | |
| HP:0008703 | Gonadal calcification | |
| HP:0001007 | Hirsutism | |
| HP:0030088 | Increased serum testosterone level | |
Run the differential on the 6 obligate / very frequent terms
Annotation source: 16844153[PMID]_10193334[PMID]_11426907[PMID]_24670054[PMID]_8629912[PMID]_18501324[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/314478
- This record as JSON: /data/d/314478.json