Placental site trophoblastic tumor
Synonyms: PSST
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare gestational trophoblastic neoplasm characterized histologically by invasion of myometrium by intermediate trophoblastic cells without chorionic villi and containing human placental lactogen hormone (hPL). Tissue necrosis is usually absent and hemorrhage is mild. The tumor develops from the placental implantation site and always occurs following pregnancy, voluntary termination of pregnancy (VTP) or miscarriage. Indicative signs are irregular metrorrhagia some time after spontaneous miscarriage or VTP, presence of metastasis or unexplained metrorrhagia in the weeks and months following normal childbirth or ectopic pregnancy.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Placental site trophoblastic tumor | PSST |
| fr | Tumeur du site d'implantation | Tumeur trophoblastique placentaire |
| de | Plazentabett-Tumor | PSST · Trophoblastentumor am Plazentaansatz |
| es | Tumor trofoblástico del sitio placentario | |
| it | Tumore trofoblastico della placenta | |
| nl | Trofoblasttumor aan implantatiezijde van placenta | = en |
| pt | Tumor trofoblástico do local da placenta (PSST) | |
| pl | Guz trofoblastyczny miejsca łożyskowego | = en |
| cs | Cytotrofoblastický choriokarcinom | = en |
| uk | Трофобластична пухлина плацентарної ділянки | = en |
| zh | 胎盘部位滋养细胞肿瘤 | = en |
| tr | Plasental bölge trofoblastik tümör | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D39.2 | NTBT | index | validated |
| ICD-11 | 2F31.2 | NTBT | index | validated |
| ICD-11 | 2F76 | NTBT | index | validated |
| ICD-11 | 2F96 | NTBT | index | validated |
| ICD-11 | XH1RM5 | E | specific | validated |
| MONDO | 0020552 | E | validated | |
| MeSH | D018245 | E | validated | |
| UMLS | C0206666 | E | validated | |
| GARD | 7403 | 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:59305Gestational trophoblastic neoplasm
rare neoplastic diseases; rare gynecological and obstetric diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.02 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Lifetime Prevalence | 1-9 / 1 000 000 | 0.86 / 100 000 | Europe | Value and class | Validated | 22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER] |
| Point prevalence | <1 / 1 000 000 | — | Europe | 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
4 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0000141 | Amenorrhea | |
| HP:0011434 | Low maternal serum chorionic gonadotropin | |
| HP:0100608 | Metrorrhagia | |
| HP:0005268 | Spontaneous abortion | |
Run the differential on the 4 obligate / very frequent terms
Annotation source: 14614893[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/99928
- This record as JSON: /data/d/99928.json