High-grade neuroendocrine carcinoma of the corpus uteri
Synonyms: High-grade neuroendocrine carcinoma of the uterine corpus · Poorly differentiated neuroendocrine carcinoma of the corpus uteri · Poorly differentiated neuroendocrine carcinoma of the endometrium
- Type
- Disease
- Group
- Disorder
- Status
- active
High-grade neuroendocrine carcinoma of the corpus uteri is an extremely rare, aggressive, primary uterine neoplasm, originating from neuroendocrine cells scattered within the endometrium, characterized, macroscopically, by a bulky, frequently polypoid, mass with abundant necrosis located in the uterus and, histologically, by rosette-like and cord-like structures consisting of small, rounded cells with oval nuclei and scarce cytoplasm. Patients often present with dysfunctional uterine bleeding, pelvic or abdominal mass and, especially in later stages of the disease, abdominal pain. Symptomatic metastatic spread or symptoms related to a paraneoplastic syndrome, such as retinopathy, or Cushing syndrome due to ectopic ACTH production, may be associated.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | High-grade neuroendocrine carcinoma of the corpus uteri | High-grade neuroendocrine carcinoma of the uterine corpus · Poorly differentiated neuroendocrine carcinoma of the corpus uteri · Poorly differentiated neuroendocrine carcinoma of the endometrium |
| fr | Carcinome neuroendocrine de haut grade du corps de l'utérus | Carcinome neuroendocrine peu différencié de l'endomètre · Carcinome neuroendocrine peu différencié du corps de l'utérus |
| de | High-grade-NET des Corpus uteri | High-grade neuroendokrines Karzinom des Corpus uteri · Wenig differenziertes endokrines Karzinom des Corpus uteri · Wenig differenziertes endokrines Karzinom des Endometriums |
| es | Carcinoma neuroendocrino del cuerpo uterino de alto grado | Carcinoma neuroendocrino pobremente diferenciado del cuerpo uterino · Carcinoma neuroendocrino pobremente diferenciado del endometrio |
| it | Carcinoma neuroendocrino del corpo dell'utero ad alto grado di malignità | Carcinoma endocrino scarsamente differenziato dell'endometrio |
| nl | Hooggradig neuro-endocrien carcinoom van corpus uteri | Hooggradig neuro-endocrien carcinoom van baarmoederlichaam · Slecht gedifferentieerd neuro-endocrien carcinoom van corpus uteri · Slecht gedifferentieerd neuro-endocrien carcinoom van endometrium |
| pt | Carcinoma endócrino pouco diferenciado do corpo uterino | |
| pl | Neuroendokrynny rak trzonu macicy o wysokim stopniu złośliwości | Słabo zróżnicowany neuroendokrynny rak endometrium · Słabo zróżnicowany neuroendokrynny rak trzonu macicy |
| cs | Špatně diferencovaný endokrinní karcinom těla děložního | Špatně diferencovaný endokrinní karcinom endometria |
| uk | Нейроендокринна карцинома тіла матки високого ступеня злоякісності | Нейроендокринна карцинома тіла матки високого ступеня злоякісності · Погано диференційована нейроендокринна карцинома тіла матки · Погано диференційована нейроендокринна карцинома ендометрію |
| zh | 子宫体高度神经内分泌癌 | 子宫体低分化神经内分泌癌 |
| tr | Korpus uteri'nin yüksek seviyede nöroendokrin karsinomu | Uterus korpusunun yüksek dereceli nöroendokrin karsinomu · Endometriyumun kötü diferansiye nöroendokrin karsinomu · Korpus uteri'nin kötü diferansiye nöroendokrin karsinomu |
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 |
| MONDO | 0016269 | E | validated | |
| UMLS | C4751234 | 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 (2):
- ORPHA:100101Neuroendocrine tumor with other location
rare endocrine diseases; rare neoplastic diseases - ORPHA:213569Rare cancer of 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/213731
- This record as JSON: /data/d/213731.json