Neuroendocrine neoplasm of appendix
Synonyms: Appendiceal NEN · Appendiceal neuroendocrine neoplasm · NEN of appendix
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
A rare sporadic neoplasm of the appendix and the second most common type of digestive endocrine tumor, often with no specific clinical presentation. They are divided into either classic endocrine tumor of the appendix or the more aggressive goblet cell carcinoma (GCC).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Neuroendocrine neoplasm of appendix | Appendiceal NEN · Appendiceal neuroendocrine neoplasm · NEN of appendix |
| fr | Néoplasme neuroendocrine de l'appendice | NEN appendiculaire · NEN de l'appendice · Néoplasme neuroendocrine appendiculaire |
| de | Tumor, neuroendokriner, der Appendix | Appendikale NEN · Appendikale neuroendokrine Neoplasie · NEN der Appendix |
| es | Neoplasia neuroendocrina de apéndice | Neoplasia neuroendocrina apendicular · TNE apendicular · TNE de apéndice |
| it | Tumore endocrino dell'appendice | Tumore endocrino appendicolare |
| nl | Neuro-endocrien neoplasma van appendix | Appendiculair NEN · Appendiculair neuro-endocrien neoplasma · NEN van appendix |
| pt | Tumor endócrino do apêndice | |
| pl | Guz neuroendokrynny wyrostka robaczkowego | = en |
| cs | Endokrinní nádor appendixu | = en |
| uk | Нейроендокринна пухлина апендикса | НЕП аппендіксу · Нейроендокринна пухлина аппендіксу · НЕП аппендіксу |
| zh | 阑尾神经内分泌肿瘤 | 阑尾神经内分泌瘤 |
| tr | Apendiks nöroendokrin neoplazmı | Apansise ait NEN · Apendiks NEN · Apendiks nöroendokrin neoplazmı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C18.1 | NTBT | attributed | validated |
| ICD-10 | D37.3 | NTBT | attributed | validated |
| ICD-11 | 2B81.2 | E | specific | validated |
| MONDO | 0015066 | E | validated | |
| MONDO | 15066 | E | validated | |
| UMLS | C1879718 | 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:423982Epithelial tumor of the appendix
rare gastroenterological diseases; rare neoplastic diseases; rare transplant-related disorders - ORPHA:481508Gastroenteric neuroendocrine neoplasm
rare endocrine diseases; rare neoplastic diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
30 annotations · validated 2017-11-08| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0006723 | Intestinal carcinoid | |
| Frequent (79-30%) | ||
| HP:0011848 | Abdominal colic | |
| HP:0030142 | Abnormal bowel sounds | |
| HP:0012701 | Bowel urgency | |
| HP:0002574 | Episodic abdominal pain | |
| HP:0005249 | Functional intestinal obstruction | |
| HP:0030144 | Hypoactive bowel sounds | |
| HP:0010676 | Mechanical ileus | |
| HP:0002017 | Nausea and vomiting | |
| HP:0004396 | Poor appetite | |
| Occasional (29-5%) | ||
| HP:0040276 | Adenocarcinoma of the colon | |
| HP:0002039 | Anorexia | |
| HP:0002730 | Chronic noninfectious lymphadenopathy | |
| HP:0002019 | Constipation | |
| HP:0002910 | Elevated circulating hepatic transaminase concentration | |
| HP:0003148 | Elevated serum acid phosphatase | |
| HP:0002240 | Hepatomegaly | |
| HP:0030412 | Ileal adenocarcinoma | |
| HP:0004385 | Protracted diarrhea | |
| Very rare (<4-1%) | ||
| HP:0011749 | Adrenocorticotropic hormone excess | |
| HP:0031499 | Appendiceal mucinous neoplasm | |
| HP:0002099 | Asthma | |
| HP:0030148 | Heart murmur | |
| HP:0002615 | Hypotension | |
| HP:0003144 | Increased serum serotonin | |
| HP:0005211 | Midgut malrotation | |
| HP:0100615 | Ovarian neoplasm | |
| HP:0001962 | Palpitations | |
| HP:0001579 | Primary hypercortisolism | |
| HP:0010446 | Tricuspid stenosis | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 12115365[PMID]_21741512[PMID]_25840530[PMID]_8466309[PMID]_25840530[PMID]_22052063[PMID]_28326748[PMID]_27311320[PMID]_27306228[PMID]_26774818[PMID]_23049637[PMID]_20631606[PMID]_19363829[PMID]_17082811[PMID]_10832290[PMID]_10352529[PMID]_10323177[PMID]_14047627[PMID]_18887660[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/100079
- This record as JSON: /data/d/100079.json