Case Diagnosed As Oncocyctic Biliary Cystadenocarcinoma, but Tanaka Et Al. 18 Suggested

Supplementary Table 2. Cases of intraductal oncocytic papillary neoplasms of the bile ducts described in the literature (including 1 previously not reported case).#

No Year of Age Gender Signs and Preoperative diagnostics Tumor Surgery Location Size Grade of Invasion Type of Margins Metastases Survival publicati symptoms markers dysplasia / invasive involved at the time on presence of carcinoma of surgery and invasion or authors presentatio n 1# 1992 56 M Fever In CT well demarcated, NG Left-lateral Left lobe 12 cm Single small Capsule Tubular NG No Peritoneal * Wolf et predominantly hypodense segmentecto glands (focal) carcinomatosis al. lesion, in biopsy rare benign my suggestive of and recurrence [39] appearing columnar cells invasion after 20 and mucin within months, death capsule of cerebrovascula r accident after 25 months 2# 2001 71 M Abdominal In CT and MRI multiple CEA: Autopsy Left lobe 4 cm Invasive Invasion of Mixed - Abdominal Death without Sudo et fullness, chest cystic lesions and adjacent 4.4 ng/ml, (largest carcinoma hepatic disseminati surgical al. pain, ascites solid lesion CA19-9: cystic hilum, on treatment [13] 37 U/ml lesion) hepatoduod enal ligament, abdominal disseminati on 3# 2002 46 M Right upper In MRI septated cystic mass NG Left Left lobe 21 cm Low-grade to - - NG - Alive, at 30 Martin et abdominal pain with marked bile duct lobectomy moderate months with al. and rigors dilatation with caudate dysplasia recurrence (at [40] lobe biliary resection, anstomosis) hilar lymphadene ctomy 4# 2002 50 M Vague In CT large cystic mass with NG Left Left lobe 7 cm Low-grade to - - NG - Alive, without Martin et abdominal pain, biliary duct dilatation hepatecomy moderate disease at 18 al. palpable mass dysplasia months [40]

5# 2002 39 M Painless In MRI lobulated enhancing NG Right Right lobe 21 cm Low-grade to - - NG - Alive, without Martin et jaundice mass with biliary duct hepatectomy moderate disease at 24 al. dilatation and atrophy of dysplasia months [40] segments 5 and 8

6# 2004 63 M Abdominal In US, CT, MRI unilocular CEA: Left Left lobe, 14 cm High-grade - - No No Alive, without Terada pain, jaundice, cystic mass, in percutaneous 5.5 U/l lobectomy medial dysplasia disease at 30 and palpable mass cholangiography and ERCP (normal <5), segment months Taniguch communication with CA19-9: i intrahepatic ducts, ductal 87.1 U/l [41] system filled with mucin (normal <37)

7# 2004 52 M Multiple In US common bile duct NG Resection of Distally to 1.7 cm Moderate - - Negative - Alive, without 1 Spector episodes of measuring 11 mm, in ERCP extrahepatic the grade disease at 36 et al. relapsing biliary 1-cm polypoid lesion at the biliary bifurcation dysplasia months [42] obstruction bifurcation of the hepatic ducts, of the ducts, in MRI a filling cholecystect CBD, mass defect within common bile omy, extended duct reconstructi into a on by lumen of separate left left hepatic and right duct hepaticojeju nostomies 8# 2004 43 F Right upper In CT large hypervascular NG Right Right lobe 18 x 13.5 Invasive Hepatic Tubular NG No No follow-up ** Bardin et quadrant pain, heterogenous mass, tumor hepatic x 8 cm carcinoma parenchym after discharge al. palpable mass thrombus extending from trisegmentec a from hospital [43] the hepatic vein into the tomy, vena cava and right atrium, atriotomy in biopsy “low-grade on papillary neoplasm” cardiopulmo nary bypass with extraction of tumor 9-10 2004 NG 2 cases NG NG NG NG NG NG At least high- NG NG NG NG NG Shibahar grade a et al. dysplasia, [56] mixed pancreaticobil iary and oncocytic 11# 2006 83 M Right In CT and MRI cystic lesion CEA and Left Left medial 3 cm High-grade - - NG - Alive, without Zen et al. hypochondrial CA19-9 lobectomy segment dysplasia disease at 15 [11] pain within month normal limits 12# 2006 57 F Incidental NG NG No Right lobe 0.3 cm Low-grade - - NG - NG Arena et finding during dysplasia al. autopsy [44] 13 2006 NG NG NG NG NG NG NG NG Invasive NG Tubular NG NG NG Zen et al. carcinoma [14]

14-15 2006 NG 2 cases NG 1. High-grade Zen et al. dysplasia [57] 2. Invasive carcinoma 15# 2007 71 M Abdominal In CT and MR multiple NG Autopsy Left lobe NG Invasive Hepatic Tubular - - NG Rouzbah fullness cystic lesion and adjacent carcinoma hilum and man et al. solid lesion hepatoduod [45] enal ligament

16# 2007 59 M Abdominal in US, ERCP and NG Pancreatico Common 5 cm High-grade - - NG - Alive, without 2 Rouzbah pain, transient intraoperative duodenecto bile duct dysplasia disease at 12 man et al. jaundice, dark cholangiography thickening my months [45] urine of intrapancreatic portion of common bile duct 17 2007 52 M Intermittent NG NG Resection of Common 1.7 cm Low-grade - - NG - Alive, without Rouzbah biliary gallbladder bile duct, dysplasia disease at 36 man et al. obstruction and left hepatic months [45] common duct hepatic duct at the (the same junction of case in right and [42]) left hepatic ducts 18# 2007 81 F NG NG NG Cholecystec Cystic 2.5 cm Invasive NG Tubular NG Lymph NG Rouzbah tomy mass carcinoma nodes (3) man et al. adjacent to [45] the liver, gallbladder and cystic duct 19# 2007 65 F NG In CT mass lesion with NG Left Left lobe 4 cm Invasive Hepatic Tubular NG No NG Itatsu et embedded dilated lobectomy carcinoma parenchym al. intrahepatic bile ducts filled a [46] with intraluminal neoplastic lesion 20# 2007 64 F Upper In MRI marked dilatation CEA and Left Left lobe 2 x 1.7 x High-grade - - Negative - Alive, without Gulluogl abdominal pain, and ‘crowding’ of bile ducts CA19-9 hepatectomy 1.2 cm dysplasia disease at 8 u et al. nausea, of the segment 4, no mass within months [47] vomiting lesion); in percutaneous normal cholangiography multiple limits filling defects 21# 2008 50 F Weakness, In CT and MRI cavitary CEA and Orthotopic Both lobes 10 cm Microinvasiv Subepitheli Tubular - No Alive, without Tabibian anorexia, mass spanning the left and CA19-9 liver e carcinoma al disease at 6 et al. cough, chest right lobes within transplantati hyalinized months [20] pain, lower normal on, partial stroma extremity limits segmental edema, palpable resection of abdominal non- the second tender mass portion of duodenum 22# 2008 67 M Abdominal pain In US, CT and MRI 3-cm NG Segmentect V segment 3 cm Moderate - - NG - NG Carrafiell solid mass omy grade o et al. dysplasia [48] (extensive GF cosmponent) 23 2008 NG NG NG NG NG NG NG NG Non-invasive NG NG NG NG NG Nakanish lesion i et al. [58]

24 2008 NG NG NG NG NG Resection NG NG High-grade - - NG NG NG Ji et al. dysplasia 3 [59]

25# 2009 54 M Jaundice, In US left lobe hyperplasia NG Segmentect Left lobe 2 cm Moderate - - NG No Alive, without Yaman et abdominal pain and 2-cm lesion in 4A omy grade to high- disease at 12 al. segment, cystic at the grade months [16] periphery and hyperechoic dysplasia in the center 26# 2009 63 M Jaundice, In ERCP defect in the left CEA: Resection of Left lobe NG High-grade - - No No Alive, without Nakanish abdominal pain hepatic duct, 1.5 ng/ml left and dysplasia, disease at 6 i et al. in CT nodular lesion in CA19-9: caudate lobe tumor arose years and 3 [15] dilated bile duct, papillary 9.2 U/ml from the months appearance of tumor in peribiliary percutaneous transhepatic gland cholangioscopy 27# 2009 59 F No US, CT, ERCP NG Hepatic Intrahepatit 16 cm Microinvasiv Subepitheli Tubular No No Alive, without Tanaka et resection ic duct e carcinoma al disease at 10 al. (medial to connective months [18] anterior tissue segment) 28# 2009 58 F Abdominal pain US, CT, ERCP NG Hepatic Intrahepatic 5 cm Microinvasiv Subepitheli Tubular No No Alive, without Tanaka et resection duct e carcinoma al disease at 28 al. (left lobe) connective months [18] tissue

29# 2009 62 F Abdominal US, CT, ERCP NG Hepatic Intrahepatit 3.5 cm Microinvasiv Subepitheli Tubular No No Alive, without Tanaka et pressure resection c duct e carcinoma al disease at 112 al. (left lobe) connective months [18] tissue

30# 2009 51 F No US, CT, ERCP CA19-9 Hepatic Intrahepatic 8 cm Microinvasiv Subepitheli Tubular No No Recurrence in Tanaka et 105 U/ml resection duct e carcinoma al lymph node at al. (right lobe) connective 6 months, [18] tissue dead of the disease (multiple metastases and ascites) at 26 months 31# 2009 8 F Non-specific US, CT, ERCP NG Hepatic Hilar duct 1.5 cm Microinvasiv Subepitheli Tubular No No Alive, without Tanaka et abdominal resection e carcinoma al disease at 60 al. symptoms connective months [18] tissue

32# 2009 64 M No US, CT, ERCP NG Hepatic Hilar duct 2 cm Microinvasiv Subepitheli Tubular No No Alive, without Tanaka et resection e carcinoma al disease at 19 al. connective months [18] tissue

33# 2009 57 F Abdominal in US and CT 4-cm well CEA and En block Extrahepati 4 cm High-grade - - NG - Alive, without Lee at al. pain, chronic demarcated with 2 mural CA19-9 resection of c bile duct dysplasia disease at 12 [49] fatigue nodules within tumor with months normal common 4 limits bile duct 34# 2009 51 M No in CT well-circumscribed CEA and Left Left lobe 17 x 12 High-grade - - NG - Alive, without Lee at al. cystic mass with ill-defined CA19-9 hemihepatec cm dysplasia disease at 48 [49] mural nodule within tomy months normal limits 35# 2009 71 M No NA NA Pan Common 3 cm Invasive Pancreatic Tubular No No NA present creaticoduo bile duct carcinoma parenchym study denectomy a, duodenum # cases submitted for detailed study, in which minimum available data of individual patients (patient’s age and gender, tumor localization and grade of dysplasia/presence of invasion) were described * case diagnosed as oncocyctic biliary cystadenocarcinoma, but Adsay et al. [17], Sudo et al [13], Martin et al. [40] and Tanaka et al. [18] suggested that it represents IOPN-B ** case diagnosed as oncocyctic biliary cystadenocarcinoma, but Tanaka et al. [18] suggested that it represents IOPN-B US – ultrasonography, CT – computed tomography, MRI – magnetic resonance imaging, ERCP – endoscopic retrograde cholangiopancreatography, NG – not given, NA – not available