Biliary papillomatosis: analysis of 18 cases

2610Chin Med J 2008;121(24):2610-2612Clinical experienceBiliary papillomatosis: analysis of 18 casesJIANG Li, YAN Li-nan, JIANG Li-sheng, LI Fu-yu, YE Hui, LI Ning, CHENG Nan-sheng and ZHOU YongKeywords: bile duct neoplasms; papilloma :R iliary, paillomatosis (BP)、 is an extremely rareTable 1. Laboratory findings in 18 patients with biliary) pathological condition, which is characterized bypapillomatosispapillary proliferation of the bile duct epithelia.l4Laboratory lestsNumber' Percentage* (%) Normal rangeTotal bilinubin66.742.5- -240.4Although initially thought to be a benign entity, this(TB, μmol/L)tumor has been shown to have a tendency for malignantDirect bliubin1372.225.4-141.5transformation. 6 As reported in the literature, malignant(DB, umolL)degeneration occurs in 20% to 35% of cases. BecauseAlanine aminotransferase95(50-382 .patients typically present with。 abdominalpain,(ALT, IU/L)obstructive jaundice and fever,r 1.5they are usuallyAspartate aminoransferase5068-232(AST, IU/L)mistaken as having more serious pathology such asHepatitis B surface antigen5.cholangiocarcinoma, cholangitis and so forth. In general,(HBsAg, positive)the rate of preoperative diagnosis is very low. At present,27.85.96-31.5the number of published articles about this ilness is(CEA. ng/m)CA19-9 (U/ml)33.358.8-501.9limited, mainly as single case reports. In addition, the22.252.8- 95.1diagnosis, treatment and prognosis of BP have not beenGamma-glutamylransferase1688.975-862well known, We, therefore, discuss age, gender, clinical- CGT.IU/L) .manifestations, diagnostic approaches, histopathological'The number and percentage of patients with abnormal result.findings, treatments and prognosis by reviewing theclinical data of eighteen patients with BP treated in our(66.7%) and intrahepatic retecious darkground (16.7%).hospital, so as to better understand this rare ilness.Features seen by CT included nodule (60%) and bile ductdilatation (40%). MRCP showed bile duct dilatationCLINICAL DATA(53.8%)，nodule (61.5%) and filing defect (69.2%).Unfortunately, no suggestive preoperative diagnosis wasPatientsmade by these diagnostic approaches. Additionally, it isBetween January 2003 and March 2007, eighteen patients,noteworthy that intraoperative frozen section waswho were diagnosed with BP based on histopathologicalperformed in all patients and produced the definitiveexaminations and treated in the West China Hospital ofdiagnosis.Sichuan University, were enrolled in our study. Elevenpatients were men (61.1%) and seven were womenTreatment and anatomical findings(38.9%), the male-to-female ratio was 1.6:1. Their meanTherapeutic approaches, according to the tumor site, aresummarized in Table 2. A solitary lesion was observed in 8age was 49 years (range, 16 -70 years).patients (44.4%), by contrast, multiple lesions occurred inClinical manifestationsthe remaining 10 patients (55.6%). The median tumor sizeThe principal clinical manifestations at admission werewas 2.4 cm (range, 0.2- 4.5 cm). Seven cases were locatedabdominal pain (83.3%), obstructive jaundice (72.2%),in the left intrahepatic bile duct, 2 in the right intrahepaticchills and fever (38.8%)， pruritus (22.2%) andbile duct, 1 in the bilateral intrahepatic bile duct, 6 in thecommon bile duct, 1 in the common hepatic duct and 1 inclay-colored stool (11.1%).the left intrahepatic bile duct and common bile duct.Laboratory testsLiver function tests, hepatitis B surface antigen (HBsAg),Histopathological findingscarcinoembryonic antigen (CEA), CA19-9, CA12-5 wereMacroscopic findingsEleven patients (61.1%) had whitish, lobular, polypoidexamined in all eighteen patients (Table 1).lesiqns grisinq frm the lataral bile duct, and the中国煤化工Radiological findingsFirst_ang L Jiang LS, LiFY, YeAll patients had an abdominal ultrasonography (US)H, LC N M H Gnd Department of Generalperformed, 10 had a computed tomography (CT) scan andSurgery (Yan LN), wesi Lnina Hospital of Sichuan University,13 had a magnetic resonance cholangiopancreatographyChengdu, Sichuan 610041, China(MRCP) performed. The main appearances on USCorrespondence to: Prof. JIANG Lisheng, First Department ofGeneral Surgery, West China Hospital of Sichuan University,included bile duct dilatation (44.4%), a hypoechoic areaChengdu, Sichuan 610041, China (Email: jls339@ 126.com)Chinese Medical Joumal 2008; 121(24):2610-26122611Table 2. Therapeutic approaches of 18 patients with biliaryand fever. Of note, jaundice may become apparentpapillomatosisgradually, with the progression of ilness. Moreover,Tumor siteTherapeutic approachesNumber Totalpruritus and clay-colored stool may be observed in theseLeft itrahepatic bile ductLeft hepatic lobecomypatients. However, when the bile duct is not completelyLeft hemihepatctomyRight itahepatic bilePartial hepatic resection2obstructed by the tumor, the above significant symptomsductmay not be found. Thus, these typical clinicalLeft and right intnahepaicmanifestations could serve as a reminder that BP can alsobile ductexist in the patients without significant symptoms. InCommon bile ductaddition, the demographic data from our series revealedResection of bile duct including thethat BP is more common in men at their fourth to fifthlesion and cholangiojcjunostormyCommon hepatic ductDrainage'decade of life.Left itrhepatic bile ductLeft hepatic lobectomy andand comon bile ducttumorectomy andPreoperative diagnosis of BP is usually difficult. Nocholangiojejunostomynarientpatient in our series had obtained the definitive diagnosisSurgia rsection can not be perfoformed in this patient because extensive areahad been involved.preoperatively.Althoughnospecific clinicalmanifestations have been described, the clinical feature isremaining 7 patients (38.9%) had yellow, polypoidstill important. As a result of the rarity of BP, it is usuallymasses. In addition, mucoid material was observed in 10mistaken for more seriouspathology such aspatients (55.6%).cholangiocarcinoma, acute cholangitis and so forth.Similarly, laboratory findings, owing to the absence ofMicroscopic. findingsspecificity, have not contributed to the preoperativeIn our series, ten tissue specimens were obtained bydiagnosis.cholangioscopic biopsy, 8 by surgical resection. Alsamples underwent histopathological examination andCompared with laboratory findings, radiologic findingswere demonstrated to have papillary proliferation of thecould provide more direct information. By US, BIbiliary epithelia. Cytologically, carcinomatous changeappears as a nonshadowing intrabiliary mass which iswas observed in one patient, mild atypia in 3, andfairly well defined and demarcated from the surroundingmoderate atypia in 1.bile duct wall and associated with proximal biliarydilatation.0 CT also reveals a solid intraluminal massRESULTSwith proximal biliary dilatation. Theprincipalappearances on MRCP for BP are of an iregular fllingAll patients did well postoperatively except 1 patient withdefect, complete or incomplete obstruction of the bilepancreaticoduodenectomy, who had an anastomotic leakduct, and proximal biliary dilatation.five days after surgery. Fortunately, this patient recoveredeventually through nonoperative management.Furthermore, some uncommon morphologic investigations,including endoscopic ultrasonography(EUS) andAll eighteen patients were followed up by telephoneendoscopic retrograde cholangiopancreatography (ERCP),contact, no patient was lost during follow-up. The meanmay contribute to obtaining a suggestive diagnosis.duration of follow-up was 28 months (range, 4 -52Advanced EUS has been more accurate in determiningmonths). During follow-up, three patients died as a resultthe etiology of biliary obstruction and provides a detailedof malignant transformation, one patient had a recurrenceassessment of the extent of the tumor." The appearance20 months after surgery, and the remaining patients wereon ERCP is similar to those of MRCP. In addition, ERCPwithout any sign of recurrence.may also show excessive mucus within the common bileduct and duodenum. However, the availability of ERCP isDISCUSSIONrestricted by some complications such as hemorrhage,cholangitis and pancreatitis.3P is an exceedingly rare condition, which ischaracterized by papillary proliferation of the bile ductBecause the definitive diagnosis mainly depends onepithelia. As reported in the related literature, BPintraoperative frozen sections, the histopathologicalusually includes single papilloma, multiple papillomasfindings with regard to BP should be addressed. Grossly;and papillary adenoma. In addition, BP may be classifiedBP could arise from any site in the biliary tree."s either mucin-hypersecreting type (MBP) orWhitish or_ yellow lobular polypoid lesions with ornonmucin-producing type (NMBP)， according to thewitho中国煤化工d to be arising frompresence or absence of mucoid material. Unfortunately,the lamucus within the bilethe etiology of BP remains unclear until now.duct il:fYHC N M H Gome patients lack thisobvious feature. In our study, mucus was found in 10The clinical data from our study demonstrated that thepatients (55.6%). Microscopically, the dilated ductmost common clinical manifestations of patients with BPcontains papillary adenoma, which is composed ofare recurrent abdominal pain, obstructive jaundice, chillsfibrovascular cores lined by columnar, biliary-type cells.'.2612Chin Med J 2008:12124):2610-2612Cytologically, the proliferated biliary epithelia can showREFERENCESvarious degree of atypia. As repoted in the literature,malignant degeneration occurs in about 20% to 35% of1. Kim JD, Lee KM, Chung WC, Chang UI, Lee BI, Lee JS, et al.cases. Thus， BP should be considered to be aAcute pancreatitis and cholangitis caused by hemobilia frompremalignant disease with high malignant potential. .biliary papillomatosis. Gastrointest Endosc 2007; 65: 177-180.2. Chung DI, Lee SK, Ha HK, Kim PN, Lee MG MultipleTherapeutic approaches of BP should be adopted flexibly,biliary papillomatosis: comparison of MR cholangiographyaccording to tumor site, neoplastic extent and patient'swith endoscopic retrograde cholangiography. J Comput Assistcondition. Because of its malignant transformation andTomogr 2002; 26: 968-974.tendency to recur, treatment should be aimed at radical3. Amaya S, Sasaki M, Watanabe Y, Tsui WM, Tsuneyama K,excision, if possible. When the lesions are confined to aHarada K, et al. Expression of MUCI and MUC2 andsingle lobe, partial hepatectomy is thus advocated. 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Liver Transpl 2007; 13: 1045- 1048.even with radical resection, tumor could recur in the13. Beavers KL, Fried MW, Johnson MW, Zacks SL, Gerber DA,remaining bile ducts.' ”" As far as our patients wereWeeks SM, et al. Orthotopic liver transplantation for biliayconcerned, two patients died as a result of malignantpapillomatosis. Liver Transpl 2001; 7: 264 -266.transformation and one patient had a recurrence, althoughthese patients performed radical resection. Thus, it isnecessary to increase the rate of preoperative diagnosis(Received March 26, 2008)and recheck regularly after surgery.Edited by HAO Xiu-yuan中国煤化工MYHCNMHG