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Mucinous adenocarcinoma of the appendix

Disease definition

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.


Classification level: Disorder
  • Synonym(s):
    • Appendiceal mucinous adenocarcinoma
  • Prevalence: -
  • Inheritance: -
  • Age of onset: Adult, Elderly
  • ICD-10: C18.1
  • ICD-11: 2B81.00
  • OMIM: -
  • UMLS: C1706832
  • MeSH: -
  • GARD: -
  • MedDRA: 10073361

Detailed information


ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)
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