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

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Summary

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.

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Authors and Affiliations

  1. Institute of Emergency Surgery, University of Milan, Ospedale Maggiore IRCCS, Italy

    Laura Castoldi M.D., Paolo De Rai, Aldo Marini & Giorgio Tiberio

  2. Department of Medicine, Surgery and Dentistry, University of Milan, Ospedale San Paolo

    Stefano Ferrero & Valeria Midolo De Luca

Authors
  1. Laura Castoldi M.D.
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  2. Paolo De Rai
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  3. Aldo Marini
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  4. Stefano Ferrero
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  5. Valeria Midolo De Luca
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  6. Giorgio Tiberio
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Correspondence to Laura Castoldi M.D..

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Castoldi, L., De Rai, P., Marini, A. et al. Neurofibromatosis-1 and ampullary gangliocytic paraganglioma causing biliary and pancreatic obstruction. International Journal of Pancreatology 29, 93–97 (2001). https://doi.org/10.1385/IJGC:29:2:093

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  • DOI: https://doi.org/10.1385/IJGC:29:2:093

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