Intraductal papillary mucinous neoplasm of the biliary tract: a real disease?
BACKGROUND
Despite increasing numbers of reports, biliary tract intraductal papillary mucinous neoplasm (BT-IPMN) is not yet recognized as a unique neoplasm. The aim of the present study was to define the presence of BT-IPMN in a large series of resected biliary neoplasms.


METHODS
From May 1994 to December 2006, BT-IPMN cases were identified by reviewing pathology specimens of all resected cholangiocarcinomas and other biliary neoplasms when cystic, papillary or mucinous features were cited in pathology reports.


RESULTS
BT-IPMN was identified in 23 out of 253 (9%) specimens using the strict histopathological criteria of IPMN. The most common presenting symptom was abdominal discomfort which was present in 15 patients (65%). Only one of the original operative pathology reports used the term IPMN; 16 (70%) used the terms cystic, mucinous and/or papillary. BT-IPMN was isolated to non-hilar extra-hepatic ducts in 12 (52%), intra-hepatic ducts in 6 (26%) and hilar extra-hepatic ducts in 5 patients (22%). Carcinoma was found in association with BT-IPMN in 19 patients (83%); 5-year survival was 38% after resection.


CONCLUSIONS
BT-IPMN occurs throughout the intra- and extra-hepatic biliary system and can be identified readily as a unique neoplasm. Broader acceptance of BT-IPMN as a unique neoplasm may lead to a better understanding of the pathogenesis of biliary malignancies.
Barton
Joshua G
JG
Barrett
David A
DA
Maricevich
Marco A
MA
Schnelldorfer
Thomas
T
Wood
Christina M
CM
Smyrk
Thomas C
TC
Baron
Todd H
TH
Sarr
Michael G
MG
Donohue
John H
JH
Farnell
Michael B
MB
Kendrick
Michael L
ML
Nagorney
David M
DM
Reid Lombardo
Kaye M
KM
Que
Florencia G
FG