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Intraductal papillary neoplasms of the bile duct (IPNBs) represent a rare variant of biliary tumors characterized by a papillary growth within the bile duct lumen. Since their first description in 2001, several classifications have been proposed, mainly based on histopathological, radiological and c...

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Published in:World journal of gastroenterology : WJG Vol. 29; no. 38; pp. 5406 - 5427
Main Authors: Demers, Karlijn, Bak, Michiel T J, Bongers, Bart C, de Vries, Annemarie C, Jonkers, Daisy M A E, Pierik, Marieke J, Stassen, Laurents P S
Format: Journal Article
Language:English
Published: Baishideng Publishing Group Inc 14-10-2023
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Summary:Intraductal papillary neoplasms of the bile duct (IPNBs) represent a rare variant of biliary tumors characterized by a papillary growth within the bile duct lumen. Since their first description in 2001, several classifications have been proposed, mainly based on histopathological, radiological and clinical features, although no specific guidelines addressing their management have been developed. Bile duct neoplasms generally develop through a multistep process, involving different precursor pathways, ranging from the initial lesion, detectable only microscopically, i.e. biliary intraepithelial neoplasia, to the distinctive grades of IPNB until the final stage represented by invasive cholangiocarcinoma. Complex and advanced investigations, mainly relying on magnetic resonance imaging (MRI) and cholangioscopy, are required to reach a correct diagnosis and to define an adequate bile duct mapping, which supports proper treatment. The recently introduced subclassifications of types 1 and 2 highlight the histopathological and clinical aspects of IPNB, as well as their natural evolution with a particular focus on prognosis and survival. Aggressive surgical resection, including hepatectomy, pancreaticoduodenectomy or both, represents the treatment of choice, yielding optimal results in terms of survival, although several endoscopic approaches have been described. IPNBs are newly recognized preinvasive neoplasms of the bile duct with high malignant potential. The novel subclassification of types 1 and 2 defines the histological and clinical aspects, prognosis and survival. Diagnosis is mainly based on MRI and cholangioscopy. Surgical resection represents the mainstay of treatment, although endoscopic resection is currently applied to nonsurgically fit patients. New frontiers in genetic research have identified the processes underlying the carcinogenesis of IPNB, to identify targeted therapies.
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Corresponding author: Karlijn Demers, MD, MSc, Department of Surgery, Maastricht University Medical Center+, Maastricht 6229 HX, Netherlands. k.demers@maastrichtuniversity.nl
Author contributions: Demers K, Bak MTJ, Bongers BC, Jonkers DMAE, de Vries AC, Pierik MJ, and Stassen LPS contributed to the conception and design of this review; Demers K performed the literature search; Demers K and Bak MTJ performed study selection and data acquisition; Demers K drafted the manuscript; Bak MTJ, Bongers BC, Jonkers DMAE, de Vries AC, Pierik MJ, and Stassen LPS critically revised the manuscript for important intellectual content; All authors revised the manuscript and approved the final version.
ISSN:1007-9327
2219-2840
DOI:10.3748/wjg.v29.i38.5406