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PPoma

Disease definition

PPoma is a type of pancreatic endocrine tumor (see this term) that hypersecretes pancreatic polypeptide (PP) but that does not cause a hypersecretion syndrome (is non-functioning) and instead presents with only non-specific symptoms such as weight loss, abdominal pain, jaundice, diarrhea and/or an abdominal mass, hence leading to a late diagnosis. PPoma can be associated with multiple endocrine neoplasia 1 (MEN-1; see this term).

ORPHA:97278

Classification level: Disorder
  • Synonym(s):
    • Pancreatic polypeptidoma
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: E16.8
  • ICD-11: 2C10.1
  • OMIM: -
  • UMLS: C0346407
  • MeSH: -
  • GARD: -
  • MedDRA: -

  A summary on this disease is available in Español (2014) Français (2014) Italiano (2014) Nederlands (2014) Polski (2014, pdf)

Detailed information

Guidelines

Disease review articles

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.