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Disease definition

A rare neoplastic gastroenterologic disease most often found in children, which usually presents with the non-specific symptoms of a palpable mass, vomiting, abdominal pain, jaundice, and weight loss/failure to thrive. Histologically, this malignant epithelial pancreatic neoplasm of the exocrine cells is characterized by multiple lines of differentiation (acinar, ductal, mesenchymal, neuroendocrine) and the presence of squamoid nests.


Classification level: Disorder
  • Synonym(s): -
  • Prevalence: <1 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Childhood, Adult
  • ICD-10: C25.1
  • ICD-11: 2C10.0  XH27L5
  • OMIM: -
  • UMLS: C0334489
  • MeSH: C537162
  • GARD: 4210
  • MedDRA: 10073367

Detailed information


  • Clinical practice guidelines
  • English (2012) - J Pediatr Hematol Oncol
ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)
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.