Neuroendocrine neoplasm
- Type
- Category
- Group
- Group of disorders
- Inheritance
- Unknown
- Age of onset
- Adult
- Status
- active
A group of rare tumors characterized by predominantly neuroendocrine differentiation, potentially arising in most organs of the body, including the central nervous system, respiratory tract, larynx, gastrointestinal tract, thyroid, skin, breast, and urogenital system. The gastrointestinal tract and lungs are the most common primary tumor sites. Based on clinical behavior, histology, and proliferation rate, the tumors may be categorized as well differentiated (low grade to intermediate grade) neuroendocrine tumors and poorly differentiated (high grade) neuroendocrine carcinomas. They may or may not be associated with clinical hormone hypersecretion syndromes.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Neuroendocrine neoplasm | |
| fr | Néoplasme neuroendocrine | |
| de | Neuroendokrine Neoplasie | |
| es | Tumor neuroendocrino | |
| it | Neoplasia neuroendocrina | |
| nl | Neuro-endocrien neoplasma | |
| pt | Tumor neuro-endócrino | |
| pl | Guz endokrynowy | APUDoma |
| cs | Endokrinní tumor | APUDom · Neuroendokrinní tumor |
| uk | Нейроендокринне новоутворення | |
| zh | 神经内分泌瘤 | |
| tr | Nöroendokrin neoplazm |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 0019496 | E | validated | |
| MeSH | D018358 | E | validated | |
| UMLS | C0206754 | E | validated | |
| GARD | 9316 | E | validated | |
| MedDRA | 10052399 | 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:97978Rare endocrine disease
rare endocrine diseases - ORPHA:182130Tumor of endocrine glands
rare neoplastic diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
5 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 100 000 | 3.2 / 100 000 | Norway | Value and class | Validated | 18853416[PMID] |
| Annual incidence | 1-9 / 100 000 | 5.25 / 100 000 | United States | Value and class | Validated | 18565894[PMID]_National Cancer OTHERitute[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.53 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Point prevalence | 1-5 / 10 000 | 35 / 100 000 | United States | Value and class | Validated | 18565894[PMID] |
| 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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/877
- This record as JSON: /data/d/877.json