Intraductal papillary neoplasm of the bile duct:The new frontier of biliary pathology

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.

Neoadjuvant therapy in the treatment of hilar cholangiocarcinoma:Review of the literature

Cholangiocarcinoma(CCA)is a malignant tumor of the biliary system and includes,according to the anatomical classification,intra hepatic CCA(iCCA),hilar CCA(hCCA)and distal CCA(dCCA).Hilar CCA is the most challenging type in terms of diagnosis,treatment and prognosis.Surgery is the only treatment possibly providing long-term survival,but only few patients are considered resectable at the time of diagnosis.In fact,tumor’s extension to segmentary or subsegmentary biliary ducts,along with large lymph node involvement or intrahepatic metastases,precludes the surgical approach.To achieve R0 margins is mandatory for the disease-free survival and overall survival.In case of unresectable locally advanced hCCA,radiochemotherapy(RCT)as neoadjuvant treatment demonstrated to be a therapeutic option before either hepatic resection or liver transplantation.Before liver surgery,RCT is believed to enhance the R0 margins rate.For patients meeting the Mayo Clinic criteria,RCT prior to orthotopic liver transplant(OLT)has proved to produce acceptable 5-years survivals.In this review,we analyze the current role of neoadjuvant RCT before resection as well as before OLT.