Primitive neuroectodermal tumor of the cervix uteri
Synonyms: Cervical malignant peripheral neuroectodermal tumor · Cervical peripheral neuroectodermal cancer · Malignant peripheral neuroectodermal tumor of the cervix uteri · Peripheral neuroectodermal cancer of cervix uteri
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
Primitive neuroectodermal tumor of the cervix uteri is a rare cancer of cervix uteri derived from neural crest cells, histologically composed of small, round neoplatic cells with variable degree of neural, glial and ependymal differentiation. Macroscopically, the tumor is often a large, soft, poorly circumscribed mass with infiltrative borders and necrotic areas. It presents with dysfuntional vaginal bleeding or discharge, lower abdominal pain and uterine enlargement.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primitive neuroectodermal tumor of the cervix uteri | Cervical malignant peripheral neuroectodermal tumor · Cervical peripheral neuroectodermal cancer · Malignant peripheral neuroectodermal tumor of the cervix uteri · Peripheral neuroectodermal cancer of cervix uteri |
| fr | Tumeur neuroectodermique primitive du col de l'utérus | Cancer neuroectodermique périphérique cervical · Cancer neuroectodermique périphérique du col de l'utérus · Tumeur neuroectodermique périphérique maligne cervicale · Tumeur neuroectodermique périphérique maligne du col de l'utérus |
| de | Primitiver neuroektodermaler Tumor der Cervix uteri | Maligner peripherer neuroektodermaler Tumor der Cervix uteri · Zervixkarzinom, neuroektodermales peripheres · Zervixtumor, maligner neuroektodermaler peripherer |
| es | Tumor neuroectodérmico primitivo del cuello uterino | Cáncer neuroectodérmico periférico cervical · Cáncer neuroectodérmico periférico de cuello de útero · Tumor neuroectodérmico periférico maligno cervical |
| it | Tumore neuroectodermico primitivo della cervice uterina | Cancro cervicale neuroectodermico periferico · Cancro neuroectodermico periferico della cervice uterina · Tumore cervicale neuroectodermico periferico maligno |
| nl | Primitieve neuro-ectodermale tumor van cervix uteri | Cervicale maligne perifere neuro-ectodermale tumor · Cervicale perifere neuro-ectodermale kanker · Maligne perifere neuro-ectodermale tumor van cervix uteri · Perifere neuro-ectodermale kanker van cervix uteri · Primitieve neuro-ectodermale tumor van baarmoederhals |
| pt | Tumor maligno neuroectodérmico periférico do colo do útero | |
| pl | Niedojrzały guz neuroektodermalny szyjki macicy | Obwodowy rak neuroektodermalny szyjki · Obwodowy rak neuroektodermalny szyjki · Obwodowy rak neuroektodermalny szyjki macicy |
| cs | Zhoubný periferní neuroektodermální nádor hrdla děložního | Cervikální maligní periferní neuroektodermální nádor · Cervikální periferní neuroektodermální rakovina · Periferní neuroektodermální rakovina hrdla děložního |
| uk | Примітивна нейроектодермальна пухлина шийки матки | Злоякісна периферична нейроектодермальна пухлина шийки матки · Периферичний нейроектодермальний рак шийки матки · Злоякісна периферична нейроектодермальна пухлина шийки матки · Периферичний нейроектодермальний рак шийки матки |
| zh | 子宫颈原始神经外胚层肿瘤 | 子宫颈恶性外周神经外胚层癌 |
| tr | Serviks uterinin ilkel nöroektodermal tümörü | Servikal periferik nöroektodermal kanser · Periferik nöroektodermal rahim ağzı kanseri · Servikal habis periferik nöroektodermal tümör · Serviks uteri'nin habis periferik nöroektodermal tümörü |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C53.0 | BTNT | attributed | validated |
| ICD-10 | C53.1 | BTNT | attributed | validated |
| ICD-10 | C53.8 | BTNT | attributed | validated |
| ICD-11 | 2B52.3 | NTBT | index | validated |
| MONDO | 0016284 | E | validated | |
| UMLS | C4707725 | 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:213797Sarcoma of cervix uteri
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 |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_21962148[PMID] |
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/213812
- This record as JSON: /data/d/213812.json