Neurofibromatosis-1 and ampullary gangliocytic paraganglioma causing biliary and pancreatic obstruction

Laura Castoldi, Paolo De Rai, Aldo Marini, Stefano Ferrero, Valeria Midolo De Luca, Giorgio Tiberio

Research output: Contribution to journalArticlepeer-review

Abstract

Gangliocytic paragangliomas (GPs) are exceedingly rare tumors that arise in close proximity of the papilla of Vater. Nevertheless, jaundice is an uncommon presenting symptom, reported in only 3 of 125 cases described in the literature to date, with gastrointestinal bleeding being more common. Association between GPs and neurofibromatosis 1 (NF-1), described in two patients, may be more than casual, being the frequency in normal population 1: 3000. We report an additional case of this association, presenting for the first time with simultaneous obstruction of the common biliary duct, and of the main pancreatic duct. Previous cases of GPs with jaundice and/or NF-1 are reviewed, and updating of the histogenesis presented.

Original languageEnglish
Pages (from-to)93-98
Number of pages6
JournalInternational Journal of Pancreatology
Volume29
Issue number2
Publication statusPublished - 2001

Keywords

  • Duodenal tumor
  • Gangliocytic paraganglioma
  • Immunohistochemistry
  • Neurofibromatosis
  • Von Recklinghausen's disease

ASJC Scopus subject areas

  • Gastroenterology
  • Endocrinology
  • Oncology

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