Intraductal papillary neoplasm of the intrahepatic bile duct with gastric extension and left lobe atrophy: a case report

Intraductal papillary neoplasm of the intrahepatic bile duct with gastric extension and left lobe atrophy: a case report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Intraductal papillary neoplasm of the intrahepatic bile duct with gastric extension and left lobe atrophy: a case report Bekkozha Eskendirov, Tair Koishibayev, Aibar Agynbay, Aslan Karimov, and 7 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-8558085/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background Intraductal papillary neoplasm of the bile duct (IPNB) is a rare condition that might cause bile duct cancer. The prevalence of Intraductal papillary neoplasm of the bile duct in ​​the world is not clearly defined, although it is believed that in East Asian countries (Korea, Japan and China), it is higher and accounts for 9.9–30% of bile duct tumors, while in Western countries it is 7–11%. It is detected more often in men (the ratio of men to women is 2:1–3:2) and at the age of 60–66 years. While IPNB predominantly affects the intrahepatic or extrahepatic bile ducts, extension into adjacent organs is uncommon. We present a rare case of intraductal papillary-mucinous neoplasm of the intrahepatic bile ducts with cyst formation and invasion into the serous layer of the stomach. Case Presentation: A 66-year-old Asian man was admitted to the Department of Hepatopancreatobiliary Surgery and Liver Transplantation with complaints of paroxysmal pain in the epigastrium and right upper quadrant of the abdomen (RUQ) for the past few months, without signs of jaundice and fever. In the anamnesis - a year ago a liver mass was accidentally detected by ultrasound, at which point the examination was completed. The patient underwent left hepatectomy with a good outcome after radical surgery. Histopathology examination revealed Intraductal papillary neoplasm of the bile duct with invasive carcinoma. Conclusions This case highlights a rare presentation of intrahepatic IPNB with gastric extension and left lobe atrophy. Early recognition and surgical resection can lead to favorable outcomes. Awareness of atypical growth patterns and associated hepatic atrophy is important for accurate diagnosis and optimal surgical planning. cholangiocarcinoma intraductal papillary neoplasm of the bile duct hepatectomy Bile duct neoplasms case report Figures Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Introduction Intraductal papillary neoplasm of the bile duct (IPNB) is a rare condition that might cause bile duct cancer. IPNB is a rare variant of bile duct tumors characterized by papillary or villous growth within the bile duct [ 1 ]. IPNB is characterized by slow and multiple growth, various cell types, varying degrees of invasion and the amount of mucin produced [ 2 ]. IPNB encompass a disease spectrum from benign intraepithelial neoplasm to malignant cholangiocarcinoma [ 3 ]. Аccording to the morphology of the papillae and the immunohistochemical features of mucin glycoproteins (MUCs), IPNB is divided into subtypes: gastric, intestinal, pancreaticobiliary, and oncocytic [ 4 ]. The prevalence of IPNB in ​​the world is not clearly defined, although it is believed that in East Asian countries (Korea, Japan and China), it is higher and accounts for 9.9–30% of bile duct tumors, while in Western countries it is 7–11%. [ 5 ]. Unfortunately, we cannot provide statistics for Kazakhstan, as prevalence and awareness are still low and there are no reported cases. It is detected more often in men (the ratio of men to women is 2:1–3:2) and at the age of 60–66 years [ 5 ]. Clinically, it may present with periodic and intermittent abdominal pain, cholangitis and jaundice [ 5 ], but 12% are asymptomatic [ 6 ]. The tumor can arise anywhere in the bile ducts, including the intrahepatic bile duct, perihilar, distal common bile duct, and even the cystic duct, but is most commonly located intrahepatically in the left lobe or on the hepatic hilum [ 5 ]. We present a rare case of intraductal papillary-mucinous neoplasm of the intrahepatic bile ducts with cyst formation and invasion into the serous layer of the stomach. The patient underwent left hepatectomy with a good outcome after radical surgery. Case presentation A 66-year-old Asian man was admitted to the Department of Hepatopancreatobiliary Surgery and Liver Transplantation with complaints of paroxysmal pain in the epigastrium and right upper quadrant of the abdomen (RUQ) for the past few months, without signs of jaundice and fever. In the anamnesis - a year ago a liver mass was accidentally detected by ultrasound, at which point the examination was completed. Suffers from type 2 diabetes and arterial hypertension grade 2 high risk. During physical examination, no visible pathology was detected; there were no masses or pain in the abdomen upon palpation. According to laboratory data, everything is within normal limits except for an increase in CA19-9 351.0 U/ml. Ultrasonography of the upper abdominal cavity (ultrasound) revealed a heterogeneous lesion in the left lobe of the liver, measuring 6.7*7.7 cm, the bile ducts were not dilated, the gallbladder was enlarged without signs of inflammation. On CT with contrast, the liver mass has low density relative to the liver, with minimal contrast accumulation. The formation does not contain calcifications or fat. Large liver vessels are intact. [Fig. 1 ]. An MRI revealed a solid, irregularly shaped soft tissue mass in the left lobe of the liver. The mass has a tortuous course, originating from the dilated left hepatic duct and then continuing onward, expanding in diameter. Adjacent to the left of the mass is a cystic mass with a mural soft tissue component. The parenchyma of the left lobe of the liver is almost completely atrophied [Fig. 2 ]. In DWI mode, the formation has a high MR signal, which on ADC maps means that the formation limits the diffusion of water, most likely due to hypercellularity (i.e., a high content of tumor cells) [Fig. 3 ]. Thus, a lesion originating from the left hepatic duct, the shape and course of the duct, with the presence of an abundant intraductal soft tissue component, is most characteristic of the invasive form of IPNB. The surgical approach was discussed collectively, and the patient underwent surgery. During abdominal exploration, a 6-cm tumor was discovered in the left lobe of the liver, radiating from a 7-cm cystic mass with invasion of the serosa of the stomach [Figure 4 ]. A resection of the left lateral liver sector was performed, including careful removal of the serosa of the stomach adjacent to the tumor, a cholecystectomy, and a standard lymphadenectomy. Histological examination: Intraductal papillary liver tumor associated with invasive carcinoma G2 (in the invasive component), biliary type. Invasive component within the liver tissue [Fig. 5 ]. Retention cyst of the liver. Resection margin is negative. R0. Lymph nodes without metastatic lesions. The postoperative period was uneventful and the patient was discharged on the 9 day. Discussion IPNB has unique clinicopathological features and is defined as an intraductal growing tumor developing in the intrahepatic and extrahepatic bile ducts. [ 6 ]. In the first systematic review of IPNB, invasive carcinoma occurred in 43% with a higher frequency in pancreaticobiliary type IPNB [ 7 ]. And according to the assessment of patient survival by histologic subtype, patients with pancreaticobiliary type demonstrated significantly worse survival compared to those with gastric and intestinal types [ 8 ]. Compared to Pancreatic IPMN, IPNB less frequently forms fistulas, usually the stomach and pancreas [ 6 ], in our case there was no fistula, but there was invasion into the serous membrane of the stomach, which could subsequently transform into a gastric fistula, as recently described by colleagues [ 3 ]. 5-year survival rate after surgical treatment is 65% [ 7 ]. Curative resection with negative margin is the treatment of choice, so all patients without distant metastasis are surgical candidates [ 1 ]. IPNB should be resected in a manner similar to cholangiocarcinoma: bile duct resection with or without major hepatectomy [ 1 ]. In cases where, for whatever reason, resection was not performed, the survival rate of patients with the cystic variant (i.e., a cystic tumor with a papillary tumor inside and the presence of bile duct communication) decreases to 19.7 months with 100% malignancy [ 10 ]. Summary Intraductal papillary biliary neoplasms carry a risk of biliary obstruction and increase the risk of progression to invasive carcinoma. All patients should undergo early complete surgical resection to improve prognosis. Accurate preoperative imaging evaluation and thorough intraoperative cholangioscopy evaluation are key to curative resection. Abbreviations IPNB Intraductal papillary neoplasm of the bile duct MUCs mucin glycoproteins RUQ right upper quadrant of the abdomen CT computer tomography MRI magnetic resonance imaging DWI diffusion-weighted imaging ADC apparent diffusion coefficient Declarations Ethics approval and consent to participate: Written informed consent was obtained from the patient for publication of this case report and any accompanying images. This case report was conducted in compliance with the principles of the Declaration of Helsinki. The Ethical Committee of the National Research Oncology Center (permit number №36, 15.01.2026) approved this study. Consent to participate Informed consent to participate was obtained from the patient Consent for publication: Written informed consent was obtained from the patient for publication of this case report and accompanying images. Competing interests The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. Funding No financial support was used for this case report Author Contribution All authors contributed to the clinical management of the patient, data collection, manuscript drafting, and critical revision. All authors read and approved the final manuscript. Acknowledgements: Not applicable. Data Availability The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding author. All data generated or analysed during this study are included in this article. Further enquiries can be directed to the corresponding author on request. References Ohtsuka M, Shimizu H, Kato A, et al. Intraductal papillary neoplasms of the bile duct. Int J Hepatol. 2014;2014:459091. 10.1155/2014/459091 . Luvira V. Progression of intraductal papillary neoplasm of the bile duct (IPNB): A proposed model through the observation of patients with non-resected tumors. Ann Hepatol. 2021;23:100299. 10.1016/j.aohep.2020.100299 . Chan WH, Chen CM, Wang SY et al. Intraductal papillary neoplasm of the bile duct presenting with hepatogastric fistula: a case report and literature review. Front Oncol . 2023;13:1193918. Published 2023 May 19. 10.3389/fonc.2023.1193918 Furukawa T, Klöppel G, Volkan Adsay N, et al. Classification of types of intraductal papillary-mucinous neoplasm of the pancreas: a consensus study. Virchows Arch. 2005;447(5):794–9. 10.1007/s00428-005-0039-7 . Park HJ, Kim SY, Kim HJ, et al. Intraductal Papillary Neoplasm of the Bile Duct: Clinical, Imaging, and Pathologic Features. AJR Am J Roentgenol. 2018;211(1):67–75. 10.2214/AJR.17.19261 . Nakanuma Y, Uesaka K, Kakuda Y, et al. Intraductal Papillary Neoplasm of Bile Duct: Updated Clinicopathological Characteristics and Molecular and Genetic Alterations. J Clin Med. 2020;9(12):3991. 10.3390/jcm9123991 . Published 2020 Dec 9. Gordon-Weeks AN, Jones K, Harriss E, Smith A, Silva M. Systematic Review and Meta-analysis of Current Experience in Treating IPNB: Clinical and Pathological Correlates. Ann Surg. 2016;263(4):656–63. 10.1097/SLA.0000000000001426 . Kim KM, Lee JK, Shin JU, et al. Clinicopathologic features of intraductal papillary neoplasm of the bile duct according to histologic subtype. Am J Gastroenterol. 2012;107(1):118–25. 10.1038/ajg.2011.316 . Rocha FG, Lee H, Katabi N, et al. Intraductal papillary neoplasm of the bile duct: a biliary equivalent to intraductal papillary mucinous neoplasm of the pancreas? Hepatology. 2012;56(4):1352–60. 10.1002/hep.25786 . Luvira V. Progression of intraductal papillary neoplasm of the bile duct (IPNB): A proposed model through the observation of patients with non-resected tumors. Ann Hepatol. 2021;23:100299. 10.1016/j.aohep.2020.100299 . Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-8558085","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":588660503,"identity":"1c5105d7-4379-42b0-a6cd-f1a5135536a2","order_by":0,"name":"Bekkozha Eskendirov","email":"","orcid":"","institution":"National Scientific Oncology Centre","correspondingAuthor":false,"prefix":"","firstName":"Bekkozha","middleName":"","lastName":"Eskendirov","suffix":""},{"id":588660504,"identity":"bc39344f-72c5-40c3-a206-548c3097289a","order_by":1,"name":"Tair Koishibayev","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA70lEQVRIiWNgGAWjYBACCQbGBmYGgwNQbgWIYCNFy4EzRGlhYGAGqoXwDrYRoUWyvbn5c0HBHXmD4z0GzB/n2UUbHGBL/oBPizTPwTbpGQbPDDecOWPAcHBbcu6GA2wHDPBpkZNIbGPmMTjMuOFGWgJQCzNQC3tDAgEtzZ+BWuw33H8G1DKnHqzlAF6HSSQ2SAO1JG64wXyA4WDDYZDDDjbg0yLZA/QLj8Gz5Jlnkg8cOHPseO7Mw2zJ+HQwSBxvf/yZ588d277jBxsfVNRU5/YdbzPGG2JwoHAAFjvMRKkHAvkGYlWOglEwCkbBiAMAHOhVlo4RiRgAAAAASUVORK5CYII=","orcid":"","institution":"National Scientific Oncology Centre","correspondingAuthor":true,"prefix":"","firstName":"Tair","middleName":"","lastName":"Koishibayev","suffix":""},{"id":588660505,"identity":"8bfbdbea-5ffd-471e-a25c-2be66e800e6b","order_by":2,"name":"Aibar Agynbay","email":"","orcid":"","institution":"National Scientific Oncology Centre","correspondingAuthor":false,"prefix":"","firstName":"Aibar","middleName":"","lastName":"Agynbay","suffix":""},{"id":588660506,"identity":"b90f70b7-d4bc-4aa4-a8aa-3ab7b5d3feca","order_by":3,"name":"Aslan Karimov","email":"","orcid":"","institution":"National Scientific Oncology Centre","correspondingAuthor":false,"prefix":"","firstName":"Aslan","middleName":"","lastName":"Karimov","suffix":""},{"id":588660507,"identity":"2e114932-4c25-4e05-9c70-56e29ff43761","order_by":4,"name":"Yesseniya Ganiyeva","email":"","orcid":"","institution":"National Scientific Oncology Centre","correspondingAuthor":false,"prefix":"","firstName":"Yesseniya","middleName":"","lastName":"Ganiyeva","suffix":""},{"id":588660508,"identity":"ff17a8c8-7e38-4546-bbc7-5d3da7760f3f","order_by":5,"name":"Zhanat Spatayev","email":"","orcid":"","institution":"National Scientific Oncology Centre","correspondingAuthor":false,"prefix":"","firstName":"Zhanat","middleName":"","lastName":"Spatayev","suffix":""},{"id":588660509,"identity":"49d43b9e-721e-4716-b1d9-df0e2eaff3d8","order_by":6,"name":"Chokhan Aytbayev","email":"","orcid":"","institution":"National Scientific Oncology Centre","correspondingAuthor":false,"prefix":"","firstName":"Chokhan","middleName":"","lastName":"Aytbayev","suffix":""},{"id":588660510,"identity":"57f27c1d-17ea-4c82-806e-33bdbfcca683","order_by":7,"name":"Assylmurat Zhumukov","email":"","orcid":"","institution":"National Scientific Oncology Centre","correspondingAuthor":false,"prefix":"","firstName":"Assylmurat","middleName":"","lastName":"Zhumukov","suffix":""},{"id":588660511,"identity":"c84f4084-b48a-4801-aba0-c41ab5d4b425","order_by":8,"name":"Jamilya Saparbay","email":"","orcid":"","institution":"National Scientific Oncology Centre","correspondingAuthor":false,"prefix":"","firstName":"Jamilya","middleName":"","lastName":"Saparbay","suffix":""},{"id":588660512,"identity":"7b8429e4-5ae5-4ec0-ac57-47186239fe18","order_by":9,"name":"Zhandos Burkitbayev","email":"","orcid":"","institution":"National Scientific Oncology Centre","correspondingAuthor":false,"prefix":"","firstName":"Zhandos","middleName":"","lastName":"Burkitbayev","suffix":""},{"id":588660513,"identity":"46667572-2499-4209-b4a2-7c72c119ae04","order_by":10,"name":"Adina Kulanbayeva","email":"","orcid":"","institution":"National Scientific Oncology Centre","correspondingAuthor":false,"prefix":"","firstName":"Adina","middleName":"","lastName":"Kulanbayeva","suffix":""}],"badges":[],"createdAt":"2026-01-09 07:39:01","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-8558085/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-8558085/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":102438771,"identity":"cac379e3-e382-4b04-aad2-556d4c4b99d0","added_by":"auto","created_at":"2026-02-11 16:36:28","extension":"jpeg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":180019,"visible":true,"origin":"","legend":"\u003cp\u003eAbdominal computer tomography (CT) of the patient. On contrast-enhanced CT, the mass in the liver is low-density in relation to the liver, with minimal contrast accumulation. The mass does not contain calcifications or fat. The large vessels of the liver are intact.\u003c/p\u003e","description":"","filename":"Fig1.jpeg","url":"https://assets-eu.researchsquare.com/files/rs-8558085/v1/39f86bf91168d95843ee1722.jpeg"},{"id":102438774,"identity":"ca2d8151-3919-419b-81ef-91e2265d9cdd","added_by":"auto","created_at":"2026-02-11 16:36:28","extension":"jpeg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":177543,"visible":true,"origin":"","legend":"\u003cp\u003eAbdominal magnetic resonance imaging (MRI) of the patient. The mass (arrow) has a tortuous course originating from the dilated left hepatic duct (arrowhead), then continuing the course and expanding in diameter.\u003c/p\u003e","description":"","filename":"fig2.jpeg","url":"https://assets-eu.researchsquare.com/files/rs-8558085/v1/9c109573c4a446fe5c1f2770.jpeg"},{"id":102745809,"identity":"82e351cc-4bbe-438d-bd3a-c8ae13e13b0d","added_by":"auto","created_at":"2026-02-16 08:54:07","extension":"jpeg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":145897,"visible":true,"origin":"","legend":"\u003cp\u003eMRI On DWI (left), the mass has a high MR signal, which on ADC maps (right), means that the mass limits the diffusion of water, most likely due to hypercellularity (i.e., a high content of tumor cells)\u003c/p\u003e","description":"","filename":"Fig3.jpeg","url":"https://assets-eu.researchsquare.com/files/rs-8558085/v1/841e35c6655124b94e648a7f.jpeg"},{"id":102438773,"identity":"f58d5f89-44ad-42a7-ae3c-e369d3517263","added_by":"auto","created_at":"2026-02-11 16:36:28","extension":"jpeg","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":142964,"visible":true,"origin":"","legend":"\u003cp\u003ePicture of liver formation (opened cystic formation)\u003c/p\u003e","description":"","filename":"Fig4.jpeg","url":"https://assets-eu.researchsquare.com/files/rs-8558085/v1/8ef1c9120b58473b493e092a.jpeg"},{"id":102438772,"identity":"c95b3659-61a6-43bb-a347-e265dc1dc495","added_by":"auto","created_at":"2026-02-11 16:36:28","extension":"jpeg","order_by":5,"title":"Figure 5","display":"","copyAsset":false,"role":"figure","size":366911,"visible":true,"origin":"","legend":"\u003cp\u003eLow-power photomicrograph showing papillary growths within the lumen of a dilated intrahepatic duct (blue arrow), foci of invasive carcinoma in the wall of the bile duct (red arrow). Hematoxylin and eosin staining.\u003c/p\u003e","description":"","filename":"Fig5.jpeg","url":"https://assets-eu.researchsquare.com/files/rs-8558085/v1/72f03bdc78f9f85a6618972d.jpeg"},{"id":107524585,"identity":"c52026ab-cc7c-4676-96b9-9981c535825c","added_by":"auto","created_at":"2026-04-22 09:29:09","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1155584,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-8558085/v1/42a54952-4eeb-4bea-9138-25afd2bf24ca.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Intraductal papillary neoplasm of the intrahepatic bile duct with gastric extension and left lobe atrophy: a case report","fulltext":[{"header":"Introduction","content":"\u003cp\u003eIntraductal papillary neoplasm of the bile duct (IPNB) is a rare condition that might cause bile duct cancer. IPNB is a rare variant of bile duct tumors characterized by papillary or villous growth within the bile duct [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. IPNB is characterized by slow and multiple growth, various cell types, varying degrees of invasion and the amount of mucin produced [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. IPNB encompass a disease spectrum from benign intraepithelial neoplasm to malignant cholangiocarcinoma [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]. Аccording to the morphology of the papillae and the immunohistochemical features of mucin glycoproteins (MUCs), IPNB is divided into subtypes: gastric, intestinal, pancreaticobiliary, and oncocytic [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe prevalence of IPNB in ​​the world is not clearly defined, although it is believed that in East Asian countries (Korea, Japan and China), it is higher and accounts for 9.9\u0026ndash;30% of bile duct tumors, while in Western countries it is 7\u0026ndash;11%. [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. Unfortunately, we cannot provide statistics for Kazakhstan, as prevalence and awareness are still low and there are no reported cases.\u003c/p\u003e \u003cp\u003eIt is detected more often in men (the ratio of men to women is 2:1\u0026ndash;3:2) and at the age of 60\u0026ndash;66 years [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. Clinically, it may present with periodic and intermittent abdominal pain, cholangitis and jaundice [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e], but 12% are asymptomatic [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. The tumor can arise anywhere in the bile ducts, including the intrahepatic bile duct, perihilar, distal common bile duct, and even the cystic duct, but is most commonly located intrahepatically in the left lobe or on the hepatic hilum [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eWe present a rare case of intraductal papillary-mucinous neoplasm of the intrahepatic bile ducts with cyst formation and invasion into the serous layer of the stomach. The patient underwent left hepatectomy with a good outcome after radical surgery.\u003c/p\u003e"},{"header":"Case presentation","content":"\u003cp\u003eA 66-year-old Asian man was admitted to the Department of Hepatopancreatobiliary Surgery and Liver Transplantation with complaints of paroxysmal pain in the epigastrium and right upper quadrant of the abdomen (RUQ) for the past few months, without signs of jaundice and fever. In the anamnesis - a year ago a liver mass was accidentally detected by ultrasound, at which point the examination was completed. Suffers from type 2 diabetes and arterial hypertension grade 2 high risk. During physical examination, no visible pathology was detected; there were no masses or pain in the abdomen upon palpation. According to laboratory data, everything is within normal limits except for an increase in CA19-9 351.0 U/ml.\u003c/p\u003e \u003cp\u003eUltrasonography of the upper abdominal cavity (ultrasound) revealed a heterogeneous lesion in the left lobe of the liver, measuring 6.7*7.7 cm, the bile ducts were not dilated, the gallbladder was enlarged without signs of inflammation. On CT with contrast, the liver mass has low density relative to the liver, with minimal contrast accumulation. The formation does not contain calcifications or fat. Large liver vessels are intact. [Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eAn MRI revealed a solid, irregularly shaped soft tissue mass in the left lobe of the liver. The mass has a tortuous course, originating from the dilated left hepatic duct and then continuing onward, expanding in diameter. Adjacent to the left of the mass is a cystic mass with a mural soft tissue component. The parenchyma of the left lobe of the liver is almost completely atrophied [Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e]. In DWI mode, the formation has a high MR signal, which on ADC maps means that the formation limits the diffusion of water, most likely due to hypercellularity (i.e., a high content of tumor cells) [Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e]. Thus, a lesion originating from the left hepatic duct, the shape and course of the duct, with the presence of an abundant intraductal soft tissue component, is most characteristic of the invasive form of IPNB.\u003c/p\u003e \u003cp\u003eThe surgical approach was discussed collectively, and the patient underwent surgery. During abdominal exploration, a 6-cm tumor was discovered in the left lobe of the liver, radiating from a 7-cm cystic mass with invasion of the serosa of the stomach [Figure \u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003e]. A resection of the left lateral liver sector was performed, including careful removal of the serosa of the stomach adjacent to the tumor, a cholecystectomy, and a standard lymphadenectomy.\u003c/p\u003e \u003cp\u003eHistological examination: Intraductal papillary liver tumor associated with invasive carcinoma G2 (in the invasive component), biliary type. Invasive component within the liver tissue [Fig.\u0026nbsp;\u003cspan refid=\"Fig5\" class=\"InternalRef\"\u003e5\u003c/span\u003e]. Retention cyst of the liver. Resection margin is negative. R0. Lymph nodes without metastatic lesions.\u003c/p\u003e \u003cp\u003eThe postoperative period was uneventful and the patient was discharged on the 9 day.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eIPNB has unique clinicopathological features and is defined as an intraductal growing tumor developing in the intrahepatic and extrahepatic bile ducts. [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. In the first systematic review of IPNB, invasive carcinoma occurred in 43% with a higher frequency in pancreaticobiliary type IPNB [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. And according to the assessment of patient survival by histologic subtype, patients with pancreaticobiliary type demonstrated significantly worse survival compared to those with gastric and intestinal types [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eCompared to Pancreatic IPMN, IPNB less frequently forms fistulas, usually the stomach and pancreas [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e], in our case there was no fistula, but there was invasion into the serous membrane of the stomach, which could subsequently transform into a gastric fistula, as recently described by colleagues [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e].\u003c/p\u003e \u003cp\u003e5-year survival rate after surgical treatment is 65% [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. Curative resection with negative margin is the treatment of choice, so all patients without distant metastasis are surgical candidates [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. IPNB should be resected in a manner similar to cholangiocarcinoma: bile duct resection with or without major hepatectomy [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eIn cases where, for whatever reason, resection was not performed, the survival rate of patients with the cystic variant (i.e., a cystic tumor with a papillary tumor inside and the presence of bile duct communication) decreases to 19.7 months with 100% malignancy [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e].\u003c/p\u003e"},{"header":"Summary","content":"\u003cp\u003eIntraductal papillary biliary neoplasms carry a risk of biliary obstruction and increase the risk of progression to invasive carcinoma. All patients should undergo early complete surgical resection to improve prognosis. Accurate preoperative imaging evaluation and thorough intraoperative cholangioscopy evaluation are key to curative resection.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cdiv class=\"DefinitionList\"\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eIPNB\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eIntraductal papillary neoplasm of the bile duct\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eMUCs\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003emucin glycoproteins\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eRUQ\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eright upper quadrant of the abdomen\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eCT\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003ecomputer tomography\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eMRI\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003emagnetic resonance imaging\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eDWI\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003ediffusion-weighted imaging\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eADC\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eapparent diffusion coefficient\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003c/div\u003e"},{"header":"Declarations","content":"\u003cp\u003e \u003ch2\u003eEthics approval and consent to participate:\u003c/h2\u003e \u003cp\u003e Written informed consent was obtained from the patient for publication of this case report and any accompanying images. This case report was conducted in compliance with the principles of the Declaration of Helsinki. The Ethical Committee of the National Research Oncology Center (permit number №36, 15.01.2026) approved this study.\u003c/p\u003e \u003c/p\u003e \u003cp\u003e \u003cstrong\u003eConsent to participate\u003c/strong\u003e \u003cp\u003eInformed consent to participate was obtained from the patient\u003c/p\u003e \u003c/p\u003e \u003cp\u003e \u003cstrong\u003eConsent for publication:\u003c/strong\u003e \u003cp\u003e Written informed consent was obtained from the patient for publication of this case report and accompanying images.\u003c/p\u003e \u003c/p\u003e\u003cp\u003e \u003ch2\u003eCompeting interests\u003c/h2\u003e \u003cp\u003eThe authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.\u003c/p\u003e \u003c/p\u003e\u003ch2\u003eFunding\u003c/h2\u003e \u003cp\u003eNo financial support was used for this case report\u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eAll authors contributed to the clinical management of the patient, data collection, manuscript drafting, and critical revision. All authors read and approved the final manuscript.\u003c/p\u003e\u003ch2\u003eAcknowledgements:\u003c/h2\u003e \u003cp\u003eNot applicable.\u003c/p\u003e\u003ch2\u003eData Availability\u003c/h2\u003e\u003cp\u003eThe original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding author. All data generated or analysed during this study are included in this article. Further enquiries can be directed to the corresponding author on request.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eOhtsuka M, Shimizu H, Kato A, et al. Intraductal papillary neoplasms of the bile duct. Int J Hepatol. 2014;2014:459091. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1155/2014/459091\u003c/span\u003e\u003cspan address=\"10.1155/2014/459091\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eLuvira V. Progression of intraductal papillary neoplasm of the bile duct (IPNB): A proposed model through the observation of patients with non-resected tumors. Ann Hepatol. 2021;23:100299. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1016/j.aohep.2020.100299\u003c/span\u003e\u003cspan address=\"10.1016/j.aohep.2020.100299\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eChan WH, Chen CM, Wang SY et al. Intraductal papillary neoplasm of the bile duct presenting with hepatogastric fistula: a case report and literature review. \u003cem\u003eFront Oncol\u003c/em\u003e. 2023;13:1193918. Published 2023 May 19. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.3389/fonc.2023.1193918\u003c/span\u003e\u003cspan address=\"10.3389/fonc.2023.1193918\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eFurukawa T, Kl\u0026ouml;ppel G, Volkan Adsay N, et al. Classification of types of intraductal papillary-mucinous neoplasm of the pancreas: a consensus study. Virchows Arch. 2005;447(5):794\u0026ndash;9. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1007/s00428-005-0039-7\u003c/span\u003e\u003cspan address=\"10.1007/s00428-005-0039-7\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003ePark HJ, Kim SY, Kim HJ, et al. Intraductal Papillary Neoplasm of the Bile Duct: Clinical, Imaging, and Pathologic Features. AJR Am J Roentgenol. 2018;211(1):67\u0026ndash;75. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.2214/AJR.17.19261\u003c/span\u003e\u003cspan address=\"10.2214/AJR.17.19261\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eNakanuma Y, Uesaka K, Kakuda Y, et al. Intraductal Papillary Neoplasm of Bile Duct: Updated Clinicopathological Characteristics and Molecular and Genetic Alterations. J Clin Med. 2020;9(12):3991. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.3390/jcm9123991\u003c/span\u003e\u003cspan address=\"10.3390/jcm9123991\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e. Published 2020 Dec 9.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eGordon-Weeks AN, Jones K, Harriss E, Smith A, Silva M. Systematic Review and Meta-analysis of Current Experience in Treating IPNB: Clinical and Pathological Correlates. Ann Surg. 2016;263(4):656\u0026ndash;63. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1097/SLA.0000000000001426\u003c/span\u003e\u003cspan address=\"10.1097/SLA.0000000000001426\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eKim KM, Lee JK, Shin JU, et al. Clinicopathologic features of intraductal papillary neoplasm of the bile duct according to histologic subtype. Am J Gastroenterol. 2012;107(1):118\u0026ndash;25. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1038/ajg.2011.316\u003c/span\u003e\u003cspan address=\"10.1038/ajg.2011.316\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eRocha FG, Lee H, Katabi N, et al. Intraductal papillary neoplasm of the bile duct: a biliary equivalent to intraductal papillary mucinous neoplasm of the pancreas? Hepatology. 2012;56(4):1352\u0026ndash;60. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1002/hep.25786\u003c/span\u003e\u003cspan address=\"10.1002/hep.25786\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eLuvira V. Progression of intraductal papillary neoplasm of the bile duct (IPNB): A proposed model through the observation of patients with non-resected tumors. Ann Hepatol. 2021;23:100299. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1016/j.aohep.2020.100299\u003c/span\u003e\u003cspan address=\"10.1016/j.aohep.2020.100299\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"cholangiocarcinoma, intraductal papillary neoplasm of the bile duct, hepatectomy, Bile duct neoplasms, case report","lastPublishedDoi":"10.21203/rs.3.rs-8558085/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-8558085/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e \u003cp\u003eIntraductal papillary neoplasm of the bile duct (IPNB) is a rare condition that might cause bile duct cancer. The prevalence of Intraductal papillary neoplasm of the bile duct in ​​the world is not clearly defined, although it is believed that in East Asian countries (Korea, Japan and China), it is higher and accounts for 9.9\u0026ndash;30% of bile duct tumors, while in Western countries it is 7\u0026ndash;11%. It is detected more often in men (the ratio of men to women is 2:1\u0026ndash;3:2) and at the age of 60\u0026ndash;66 years. While IPNB predominantly affects the intrahepatic or extrahepatic bile ducts, extension into adjacent organs is uncommon. We present a rare case of intraductal papillary-mucinous neoplasm of the intrahepatic bile ducts with cyst formation and invasion into the serous layer of the stomach.\u003c/p\u003e\u003ch2\u003eCase Presentation:\u003c/h2\u003e \u003cp\u003eA 66-year-old Asian man was admitted to the Department of Hepatopancreatobiliary Surgery and Liver Transplantation with complaints of paroxysmal pain in the epigastrium and right upper quadrant of the abdomen (RUQ) for the past few months, without signs of jaundice and fever. In the anamnesis - a year ago a liver mass was accidentally detected by ultrasound, at which point the examination was completed. The patient underwent left hepatectomy with a good outcome after radical surgery. Histopathology examination revealed Intraductal papillary neoplasm of the bile duct with invasive carcinoma.\u003c/p\u003e\u003ch2\u003eConclusions\u003c/h2\u003e \u003cp\u003eThis case highlights a rare presentation of intrahepatic IPNB with gastric extension and left lobe atrophy. Early recognition and surgical resection can lead to favorable outcomes. Awareness of atypical growth patterns and associated hepatic atrophy is important for accurate diagnosis and optimal surgical planning.\u003c/p\u003e","manuscriptTitle":"Intraductal papillary neoplasm of the intrahepatic bile duct with gastric extension and left lobe atrophy: a case report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2026-02-11 16:36:09","doi":"10.21203/rs.3.rs-8558085/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"40a8aa56-8156-47fc-967b-b1dac57df87b","owner":[],"postedDate":"February 11th, 2026","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2026-04-22T09:27:56+00:00","versionOfRecord":[],"versionCreatedAt":"2026-02-11 16:36:09","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-8558085","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-8558085","identity":"rs-8558085","version":["v1"]},"buildId":"XKTyCvWXoU3ODBz1xrDgd","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}