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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.

ORPHA:391723

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

  A summary on this disease is available in Deutsch (2017) Español (2017) Français (2017) Italiano (2017) Nederlands (2017)

Detailed information

Guidelines

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

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.