TY - JOUR
T1 - Neurofibromatosis-1 and ampullary gangliocytic paraganglioma causing biliary and pancreatic obstruction
AU - Castoldi, Laura
AU - De Rai, Paolo
AU - Marini, Aldo
AU - Ferrero, Stefano
AU - De Luca, Valeria Midolo
AU - Tiberio, Giorgio
PY - 2001
Y1 - 2001
N2 - 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.
AB - 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.
KW - Duodenal tumor
KW - Gangliocytic paraganglioma
KW - Immunohistochemistry
KW - Neurofibromatosis
KW - Von Recklinghausen's disease
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M3 - Article
C2 - 11876254
AN - SCOPUS:0035711460
VL - 29
SP - 93
EP - 98
JO - International Journal of Gastrointestinal Cancer
JF - International Journal of Gastrointestinal Cancer
SN - 1537-3649
IS - 2
ER -