Mucinous adenocarcinoma of the appendix
Synonyms: Appendiceal mucinous adenocarcinoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
Mucinous adenocarcinoma of the appendix is a very rare, slow growing, well-differentiated epithelial neoplasm of the appendix characterized by abundant mucin production. Clinically, it presents as acute appendicitis (with abdominal pain, fever, leukocytosis) or as pseudomyxoma peritonei (wide-spread presence of mucin within the peritoneal cavity), however some patients may be completely asymptomatic at the time of diagnosis. In many cases, a second gastrointestinal malignancy is present.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Mucinous adenocarcinoma of the appendix | Appendiceal mucinous adenocarcinoma |
| fr | Adénocarcinome mucineux de l'appendice | Adénocarcinome mucineux appendiculaire |
| de | Muzinöses Adenokarzinom des Appendix | |
| es | Adenocarcinoma mucinoso del apéndice | Adenocarcinoma mucinoso apendicular |
| it | Adenocarcinoma mucinoso dell'appendice | Adenocarcinoma mucinoso appendicolare |
| nl | Mucineus adenocarcinoom van appendix | Appendiculair muceus adenocarcinoom |
| pt | Adenocarcinoma mucinoso do apêndice | |
| pl | Rakowiak gruczołowy śluzowy wyrostka robaczkowego | Rakowiak gruczołowy śluzowy wyrostka robaczkowego |
| cs | Mucinózní adenokarcinom apendixu | Apendikální mucinózní adenokarcinom |
| uk | Муцинозна аденокарцинома апендикса | Муцинозна аденокарцинома апендикса |
| zh | 阑尾粘液腺癌 | 阑尾粘液性腺癌 |
| tr | Apendiksin müsinöz adenokarsinomu | Apandise ait müsinöz adenokarsinom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C18.1 | NTBT | attributed | validated |
| ICD-11 | 2B81.00 | E | specific | validated |
| MONDO | 0018330 | E | validated | |
| UMLS | C1706832 | E | validated | |
| MedDRA | 10073361 | 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:423982Epithelial tumor of the appendix
rare gastroenterological diseases; rare neoplastic diseases; rare transplant-related disorders
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/391723
- This record as JSON: /data/d/391723.json