Primitive neuroectodermal tumor of the corpus uteri
Synonyms: Malignant peripheral neuroectodermal tumor of the corpus uteri · Peripheral neuroectodermal cancer of the corpus uteri
- Type
- Disease
- Group
- Disorder
- Status
- active
Primitive neuroectodermal tumor of the corpus uteri is a rare cancer of corpus uteri derived from neural crest cells, characterized by small, round neoplastic cells with variable degree of neural, glial and ependymal differentiation. Macroscopically, the tumor is often a large, poorly circumscribed polypoid mass with necrotic areas and hemorrhage. It usually presents with lower abdominal or pelvic pain, irregular vaginal bleeding or discharge, pelvic mass and uterine enlargement.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primitive neuroectodermal tumor of the corpus uteri | Malignant peripheral neuroectodermal tumor of the corpus uteri · Peripheral neuroectodermal cancer of the corpus uteri |
| fr | Tumeur neuroectodermique primitive du corps de l'utérus | Cancer neuroectodermique périphérique du corps de l'utérus · Tumeur neuroectodermique périphérique maligne du corps de l'utérus |
| de | Primitiver neuroektodermaler Tumor des Corpus uteri | Maligner peripherer neuroektodermaler Tumor des Corpus uteri · PNET des Uterus |
| es | Tumor neuroectodérmico primitivo del cuerpo uterino | Cáncer neuroectodérmico periférico del cuerpo uterino · Tumor neuroectodérmico periférico maligno del cuerpo uterino |
| it | Tumore neuroectodermico primitivo del corpo dell'utero | Cancro neuroectodermico periferico del corpo uterino |
| nl | Primitieve neuro-ectodermale tumor van corpus uteri | Maligne perifere neuro-ectodermale tumor van baarmoederlichaam · Maligne perifere neuro-ectodermale tumor van corpus uteri · Perifere neuro-ectodermale kanker van baarmoederlichaam · Perifere neuro-ectodermale kanker van corpus uteri · Primitieve neuro-ectodermale tumor van baarmoederlichaam |
| pt | Tumor maligno neuroectodérmico periférico do corpo uterino | |
| pl | Pierwotny guz neuroektodermalny trzonu macicy | Obwodowy guz neuroektodermalny trzonu macicy · Złośliwy obwodowy guz neuroektodermalny trzonu macicy |
| cs | Maligní periferní neuroektodermální nádor těla děložního | Periferní neuroektodermální rakovina těla děložního |
| uk | Примітивна нейроектодермальна пухлина тіла матки | Злоякісна периферична нейроектодермальна пухлина тіла матки · Периферичний нейроектодермальний рак тіла матки |
| zh | 子宫体原始神经外胚层瘤 | 子宫体恶性外周神经外胚层肿瘤 |
| tr | Korpus uteri'nin primitif nöroektodermal tümörü | Korpus uteri'nin periferik nöroektodermal kanseri · Korpus uteri'nin habis periferik nöroektodermal tümörü |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C54.0 | BTNT | attributed | validated |
| ICD-10 | C54.1 | BTNT | attributed | validated |
| ICD-10 | C54.2 | BTNT | attributed | validated |
| ICD-10 | C54.3 | BTNT | attributed | validated |
| ICD-10 | C54.8 | BTNT | attributed | validated |
| ICD-11 | 2B5F.0 | NTBT | index | validated |
| MONDO | 0016263 | E | validated | |
| UMLS | C4707724 | 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:213620Sarcoma of the corpus uteri
rare neoplastic diseases; rare gynecological and obstetric diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/213630
- This record as JSON: /data/d/213630.json