{"paper_id":"2a8602f2-7756-41f5-84a5-010ebef42117","body_text":"Intrahepatic sarcomatoid cholangiocarcinoma with portal vein thrombus misdiagnosed as hepatocellular carcinoma: description of a rare case | 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 Intrahepatic sarcomatoid cholangiocarcinoma with portal vein thrombus misdiagnosed as hepatocellular carcinoma: description of a rare case Ting Zhan, Shan-Shan Xing, Chun-Hua Lu This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-2733045/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 Less than 1% of malignant tumors of the hepatobiliary system are intrahepatic sarcomatoid cholangiocarcinomas, a rare malignant tumor of the liver. Based only on clinical presentation and imaging, this entity is readily mistaken for hepatocellular carcinoma. In this article, we describe the case of a 36-year-old male patient who had a history of \"cirrhosis B\" and was hospitalized for six months due to increased alpha-fetoprotein. Magnetic resonance imaging (MRI) and computed tomography (CT) revealed a 63x 90 mm mass in segments V and VI of the liver, with thrombosis in the right branch of the portal vein. Following the patient's laparoscopic lobectomy and cholecystectomy, sarcomatoid cholangiocarcinoma of the right side of the liver was discovered by histopathological and immunohistochemical examinations. This case report seeks to increase understanding of intrahepatic sarcomatoid cholangiocarcinoma and its diagnosis while reducing the occurrence of clinical misinterpretation. intrahepatic sarcomatoid cholangiocarcinoma hepatobiliary malignancy sarcomatoid change portal vein thrombus CT MRI Figures Figure 1 Figure 2 Figure 3 Introduction Liver malignancy is common. Hepatocellular carcinoma (HCC), cholangiocellular carcinoma (CCC), and metastases of primary extrahepatic neoplasms are the most frequent malignant solid liver lesions[1]. Cholangiocarcinoma, a rare kind of malignant liver tumor that arises in the epithelial cells of the intrahepatic bile ducts, accounts for approximately 3.25% of primary liver malignancies. Sarcomatoid lesions are uncommon in epithelial malignancies and are more prevalent in the upper digestive tract, mammary gland, skin, uterus, urethra, gallbladder, and other organs[2]. HCC is most frequently associated with sarcomatoid alterations in the liver, which are present in approximately 2% of HCC cases treated with resection[3]. Less than 1% of liver cancer cases are sarcomatoid cholangiocarcinomas, and the pathophysiology of this relatively uncommon condition is still unknown[4]. Moreover, prognosis is poor in comparison to other liver cancers. Most commonly, adults over 40 who are middle aged or older develop intrahepatic sarcomatoid cholangiocarcinoma[5]. Imaging results can frequently be confused with hepatocellular carcinoma or other similar malignancies. Here, we present CT and MRI findings of intrahepatic sarcomatoid cholangiocarcinoma with a portal vein thrombus. Case Presentation The patient was a 36-year-old man who had received regular oral entecavir treatment for hepatitis B cirrhosis, which had been present for five years. This patient was recently admitted to the hospital with elevated alpha-fetoprotein. The patient had a family and genetic history of hepatitis B (his brother). A physical examination of the patient revealed no notable anomalies. Laboratory findings were as follows. Liver function biochemical parameters: total bilirubin, 49.3 μmol/L (normal, <23 μmol/L), direct bilirubin, 15.97 μmol/L (normal, <6.8 μmol/L) and indirect bilirubin, 33.33 μmol/L (normal range 1.71-13.68 μmoL/L). Muscle enzyme profile: aspartate aminotransferase, 50.7U/L (normal: 0-40 U/L), alanine aminotransferase, 373.92 U/L (normal: 9-50 U/L), alkaline phosphatase, 230.16 U/L (normal: 45-125 U/L), transglutaminase, 62 U/L (normal range: 11-50 U/L), total bile acids, 12.25 μmol/L (normal range 0.1-10 μmol/L) and glycocholic acid, 12.25 μmol/L (normal range 0-2.7 μmol/L). Tumor marker: alpha-fetoprotein, 461 ng/mL (normal range 0-8.1 ng/mL). Hepatic fibrosis combination analysis: hyaluronidase, 111.6 ng/mL and laminin, 9.84 ng/mL. The liver appeared to have an uneven shape and a rough surface on CT imaging. Segments 5 and 6 of the liver revealed a hypodense mass that was approximately 63 mm × 90 mm in size, had an amorphous shape, and had ill-defined edges. On enhancement scan, the lesion showed mild to moderate heterogeneous enhancement in the arterial phase, and patchy and nodular hypoenhancing regions were detected within the lesion in the delayed phase. In the right branch of the portal vein, a cast-filling defect with little localized enhancement was visible. The splenic vein was thickened, and the spleen's volume was increased(Figure 1). In comparison to the liver, the lesion showed low signal intensity on MRI and T1-weighted imaging. The lesion appeared as a slightly uneven hyper signal on T2-weighted imaging, along with many nodules and bands of T2 hypointense signal. Diffusion-weighted imaging (DWI) also revealed severe diffusion limitation, and the lesions had unevenly elevated signals. Expression diffusion coefficient (ADC) mapping displayed low signals. The lesion displayed heterogeneous and more significant enhancement on magnetic resonance dynamic enhancement, and the delayed phase revealed branching nonenhancing shadows inside the lesion. The intra- and extrahepatic bile ducts were not significantly dilated. The right portal vein tumor thrombus showed a low signal on T1-weighted scans but a significant signal on T2-weighted images. The tumor thrombus showed annular expansion in the arterial stage and concentric filling in the portal vein stage and the delayed stage (Figure 2). According to the patient's history of cirrhosis, clinical symptoms, and imaging signals such as splenomegaly and a portal vein cancer thrombus, the probability of hepatocellular carcinoma was taken into account before surgery. The enhanced pattern, however, led to differential diagnosis of cholangiocarcinoma. The patient underwent lobectomy and cholecystectomy via laparoscopic surgery. A mass measuring approximately 5 x 4 cm in size, firm, and poorly defined, was found in the posterior portion of the right lower lobe of the liver. The liver was reddish-brown with minor cirrhotic alterations. Postoperative issues, including hemorrhage and biliary fistula, did not occur. He recovered well. Histopathological and immunohistochemical analyses of the mass showed that the tumor tissue was arranged in nests and sheets along the bile duct; the tumor cells were round or fusiform with pronounced atypia. Immunohistochemical analysis revealed positive staining for cytokeratin, low molecular cytokeratin, and cancer cell wave proteins. Vimentin, cytokeratin (CK)8, and CK-19) were all positive, but CK7, CK10, and hepatocyte paraffin 1 (Hepa-1)were all negative(Figure 3). The Ki-67 proliferation index was approximately 40%. It was determined that the patient had sarcomatoid cholangiocarcinoma based on the findings of histological and immunohistochemical analyses. Discussion The majority of hepatic sarcomatoid carcinomas are believed to be sarcomatoid HCCs; intrahepatic sarcomatoid cholangiocarcinoma is highly unusual. There are no known radiological signs for intrahepatic sarcomatoid cholangiocarcinomas. Histopathological and immunohistochemical tests are used to determine whether a patient has intrahepatic sarcomatoid cholangiocarcinoma, an uncommon type of disease that most frequently affects middle-aged and older persons over the age of 40 years[6]. Sarcomatoid cholangiocarcinoma is a rare form of intrahepatic cholangiocarcinoma that has been reported to account for 4.5% of cholangiocarcinoma cases [7], and its tumor cells usually have a definite malignant epithelial cholangiocyte and sarcomatoid mixed component [8]. The histological morphology of the tumor in this case was similar to that in earlier publications in this field[2], whereby the sarcomatoid component predominantly displays a spindle cell morphology. According to immunohistochemistry, these tumors are negative for Hepa-1 but positive for the mesenchymal tumor marker vimentin and epithelial cholangiogenic tumor marker (CK8). These immunohistochemistry findings offer hints for hepatocellular carcinoma, intrahepatic cholangiocarcinoma, and metastatic carcinoma differential diagnosis[7-10]. In our case, CT revealed a poorly defined hypodense mass, and dynamic enhanced CT and MRI revealed that the lesion had generally heterogeneous progressive and persistent enhancement. Peripheral contrast enhancement of the lesion was frequently seen in the arterial, portal, and late venous phases. The tumor margin initially exhibits irregular and mild enhancement; however, this enhancement gradually becomes stronger over time and can eventually become more visible in the delayed stage. The lesion in our case displayed a persistent or progressive pattern of intensification in the portal and delayed phases, which was comparable to the intensification pattern of cholangiocarcinoma[11]. This was in contrast to the surrounding liver parenchyma. However, no appreciable bile duct dilation was observed, and portal vein thrombosis is not a typical cholangiocarcinoma symptom. HCC, liver metastases, pancreatitis, pancreatic cancer, hypercoagulable state, liver cirrhosis, and other liver diseases are some of the conditions that might lead to portal vein cancer thrombosis[12]. For patients with a clinical history of cirrhosis who develop a portal vein tumor thrombus in liver disease, HCC is typically a contributing factor[13]. HCC enhancement patterns typically display early enhancement in the arterial phase, with quick declines in imaging in the portal and delayed phases[14]. However, in our case, the enhancement pattern was not consistent with the typical enhancement pattern of HCC. Indeed, the patient had a clinical history of cirrhosis and imaging manifestations such as portal vein carcinoma thrombosis and enlarged spleen volume, which suggested a high likelihood of HCC. Therefore, differential diagnosis between cholangiocarcinoma and HCC was difficult in this case. According to related reports, sarcomatoid cholangiocarcinoma, a very rare tumor with pathological changes in epithelial and mesenchymal components, is highly aggressive and has poor prognosis[15]. Its diagnosis is mostly achieved through a combination of histopathological and immunohistochemical examinations. Early detection and surgical removal of such tumors can provide patients with an increased likelihood of survival. Conclusion We report a case of sarcomatoid cholangiocarcinoma combined with portal vein carcinoma thrombosis. CT and MRI imaging performance for this entity is worthy of study by clinicians to improve the accuracy of diagnosis. Abbreviations CT: computed tomography HCC: hepatocellular carcinoma MRI: magnetic resonance imaging CCC: cholangiocellular carcinoma DWI: diffusion-weighted imaging ADC： apparent Diffusion Coefficient Declarations Ethical approval and consent to participate All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Informed consent was obtained from the participant in this study. Consent for publication This patient consented to the reporting of this case in a scientific publication. Competing interests This research was not supported by any organization, and the authors have no conflict of interest. Authors’ contributions Ting Zhan wrote the article. Ting Zhan and Shan-Shan Xing participated in the histopathological analysis. All authors read and approved the final manuscript. Funding The authors received no funding for the writing. Availability of data and materials All data generated are included in this article. Acknowledgments Not applicable References Schneider, G., et al., MRI of sarcomatoid carcinoma of the liver. European Journal of Radiology Extra, 2005. 54 (2). Malhotra, S., et al., Intrahepatic sarcomatoid cholangiocarcinoma. J Oncol, 2010. 2010 : p. 701476. Yamaguchi, R., et al., Hepatocellular carcinoma with sarcomatous change. Oncol Rep, 1997. 4 (3): p. 525-9. Kim, D.K., et al., Analysis of intrahepatic sarcomatoid cholangiocarcinoma: Experience from 11 cases within 17 years. World J Gastroenterol, 2019. 25 (5): p. 608-621. Sintra, S., et al., Intrahepatic sarcomatoid cholangiocarcinoma. BMJ Case Rep, 2018. 2018 . Kaibori, M., et al., Intrahepatic sarcomatoid cholangiocarcinoma. J Gastroenterol, 2003. 38 (11): p. 1097-101. Sato, K., et al., Intrahepatic sarcomatoid cholangiocarcinoma of round cell variant: a case report and immunohistochemical studies. Virchows Arch, 2006. 449 (5): p. 585-90. Watanabe, G., et al., Prognosis analysis of sarcomatous intrahepatic cholangiocarcinoma from a review of the literature. Int J Clin Oncol, 2014. 19 (3): p. 490-6. Wang, T., et al., Clinical features of sarcomatoid change in patients with intrahepatic cholangiocarcinoma and prognosis after surgical liver resection: A Propensity Score Matching analysis. J Surg Oncol, 2020. 121 (3): p. 524-537. Zhang, N., et al., Sarcomatous intrahepatic cholangiocarcinoma: Case report and literature review. Medicine (Baltimore), 2018. 97 (39): p. e12549. Wengert, G.J., et al., Differentiation of Intrahepatic Cholangiocellular Carcinoma from Hepatocellular Carcinoma in the Cirrhotic Liver Using Contrast-enhanced MR Imaging. Acad Radiol, 2017. 24 (12): p. 1491-1500. Jain, P. and S. Nijhawan, Portal vein thrombosis: etiology and clinical outcome of cirrhosis and malignancy-related non-cirrhotic, non-tumoral extrahepatic portal venous obstruction. World J Gastroenterol, 2007. 13 (39): p. 5288-9. Sun, J., et al., Guidelines for Diagnosis and Treatment of Hepatocellular Carcinoma with Portal Vein Tumor Thrombus in China (2021 Edition). Liver Cancer, 2022. 11 (4): p. 315-328. Rimola, J., et al., Cholangiocarcinoma in cirrhosis: absence of contrast washout in delayed phases by magnetic resonance imaging avoids misdiagnosis of hepatocellular carcinoma. Hepatology, 2009. 50 (3): p. 791-8. Li, X., et al., Sarcomatoid intrahepatic cholangiocarcinoma in a patient with poor prognosis: a case report and literature review. J Int Med Res, 2020. 48 (11): p. 300060520969473. 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-2733045\",\"acceptedTermsAndConditions\":true,\"allowDirectSubmit\":true,\"archivedVersions\":[],\"articleType\":\"Case Report\",\"associatedPublications\":[],\"authors\":[{\"id\":186630861,\"identity\":\"9b57d36f-ba4c-4792-a4cb-5b042384adce\",\"order_by\":0,\"name\":\"Ting Zhan\",\"email\":\"\",\"orcid\":\"\",\"institution\":\"the Second Affiliated Hospital of Nanchang University\",\"correspondingAuthor\":false,\"submittingAuthor\":false,\"prefix\":\"\",\"firstName\":\"Ting\",\"middleName\":\"\",\"lastName\":\"Zhan\",\"suffix\":\"\"},{\"id\":186630862,\"identity\":\"bd3c11b8-d5f2-4fcb-89ca-bcf46a97759c\",\"order_by\":1,\"name\":\"Shan-Shan Xing\",\"email\":\"\",\"orcid\":\"\",\"institution\":\"the Second Affiliated Hospital of Nanchang University\",\"correspondingAuthor\":false,\"submittingAuthor\":false,\"prefix\":\"\",\"firstName\":\"Shan-Shan\",\"middleName\":\"\",\"lastName\":\"Xing\",\"suffix\":\"\"},{\"id\":186630863,\"identity\":\"024c223b-c6cf-428d-b1f6-98fe084e5b8f\",\"order_by\":2,\"name\":\"Chun-Hua Lu\",\"email\":\"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA1ElEQVRIiWNgGAWjYBACPgYGNiB1gIGBmf3AgQ8GNnYEtbDBtbDzJD6cUZCWTIIWfgZjY54PhxgbCGqRSH72mHfHncT+ZoY0aRuDA8wM7IePbsCvJc3cmPfMs8QZhxmPSecY3OFj4ElLu4FfSw6bNG/b4cSGw0BbcgyeMTNI8JgRp2X+YQYzaQuDw4wNRGvZcBjofQaitPA8M5Oc23bYeONhYCD3GKQlsxHyCz978jOJt22HZeedP37gwI8/Nnb87IeP4dWCxV7SlI+CUTAKRsEowAYAPHtHsDpZWc8AAAAASUVORK5CYII=\",\"orcid\":\"\",\"institution\":\"the Second Affiliated Hospital of Nanchang University\",\"correspondingAuthor\":true,\"submittingAuthor\":false,\"prefix\":\"\",\"firstName\":\"Chun-Hua\",\"middleName\":\"\",\"lastName\":\"Lu\",\"suffix\":\"\"}],\"badges\":[],\"createdAt\":\"2023-03-24 16:44:10\",\"currentVersionCode\":1,\"declarations\":\"\",\"doi\":\"10.21203/rs.3.rs-2733045/v1\",\"doiUrl\":\"https://doi.org/10.21203/rs.3.rs-2733045/v1\",\"draftVersion\":[],\"editorialEvents\":[],\"editorialNote\":\"\",\"failedWorkflow\":false,\"files\":[{\"id\":34940018,\"identity\":\"e3ce91db-211e-4738-885a-0beb66f24dd8\",\"added_by\":\"auto\",\"created_at\":\"2023-03-28 20:04:07\",\"extension\":\"png\",\"order_by\":1,\"title\":\"Figure 1\",\"display\":\"\",\"copyAsset\":false,\"role\":\"figure\",\"size\":167625,\"visible\":true,\"origin\":\"\",\"legend\":\"\\u003cp\\u003eA mass in the right hepatic lobe is shown on abdominal dynamic CT (A) arterial, (B) portal, and (C) delayed phase images. The lesion had generally heterogeneous progressive and persistent enhancement. (D). Coronal CT imaging showed tumor thrombi within the right portal vein are noted (arrow).\\u003c/p\\u003e\",\"description\":\"\",\"filename\":\"F1.png\",\"url\":\"https://assets-eu.researchsquare.com/files/rs-2733045/v1/d59a7df4ee59579343b63aa6.png\"},{\"id\":34940019,\"identity\":\"4d2a0184-3cba-429f-b03e-2efea1d55ff2\",\"added_by\":\"auto\",\"created_at\":\"2023-03-28 20:04:07\",\"extension\":\"png\",\"order_by\":2,\"title\":\"Figure 2\",\"display\":\"\",\"copyAsset\":false,\"role\":\"figure\",\"size\":180333,\"visible\":true,\"origin\":\"\",\"legend\":\"\\u003cp\\u003e(A）An axial T1-weighted gradient echo image shows a well-defined hypointense mass .(B) An axial T2-weighted single shot fast spin-echo image reveals the hyperintense mass. (C) DWI revealed severe diffusion limitation, and the lesions had unevenly elevated signals. (D) An axial T1-weighted image obtained during the arterial phase shows the lesion showed uneven and obvious enhancement. (E) An axial T1-weighted image obtained during the delayed phase revealed branching nonenhancing shadows inside the lesion. (F)The tumor thrombus showed annular expansion in the arterial stage and concentric filling in the portal vein stage and the delayed stage.\\u003c/p\\u003e\",\"description\":\"\",\"filename\":\"F2.png\",\"url\":\"https://assets-eu.researchsquare.com/files/rs-2733045/v1/902260b22330d01d406af0fb.png\"},{\"id\":34940020,\"identity\":\"42de53f0-7c3c-479b-b611-00fa7024d160\",\"added_by\":\"auto\",\"created_at\":\"2023-03-28 20:04:07\",\"extension\":\"png\",\"order_by\":3,\"title\":\"Figure 3\",\"display\":\"\",\"copyAsset\":false,\"role\":\"figure\",\"size\":388493,\"visible\":true,\"origin\":\"\",\"legend\":\"\\u003cp\\u003e(A)The histological appearance of the sarcomatoid cholangiocarcinoma is shown. Hematoxylin and eosin staining (×400)shows the cholangiocarcinoma mixed with bundles of atypical spindle cells. (B) The appearance of the sarcomatoid cholangiocarcinoma is shown after immunohistochemical staining. Cytokeratin 19 is focally expressed in sarcomatoid component (Cytokeratin, ×400).\\u003c/p\\u003e\",\"description\":\"\",\"filename\":\"F3.png\",\"url\":\"https://assets-eu.researchsquare.com/files/rs-2733045/v1/d976a2caa4fd4e991274f3ad.png\"},{\"id\":35017481,\"identity\":\"dc3a29b6-92a0-4d5c-8941-b6c1592a89e5\",\"added_by\":\"auto\",\"created_at\":\"2023-03-30 04:29:26\",\"extension\":\"pdf\",\"order_by\":0,\"title\":\"\",\"display\":\"\",\"copyAsset\":false,\"role\":\"manuscript-pdf\",\"size\":866637,\"visible\":true,\"origin\":\"\",\"legend\":\"\",\"description\":\"\",\"filename\":\"manuscript.pdf\",\"url\":\"https://assets-eu.researchsquare.com/files/rs-2733045/v1/44a4c3ed-6a31-4011-afdf-000eb2a4dbc2.pdf\"}],\"financialInterests\":\"No competing interests reported.\",\"formattedTitle\":\"Intrahepatic sarcomatoid cholangiocarcinoma with portal vein thrombus misdiagnosed as hepatocellular carcinoma: description of a rare case\",\"fulltext\":[{\"header\":\"Introduction\",\"content\":\"\\u003cp\\u003eLiver malignancy is common. Hepatocellular carcinoma (HCC), cholangiocellular carcinoma (CCC), and metastases of primary extrahepatic neoplasms are the most frequent malignant solid liver lesions[1]. Cholangiocarcinoma, a rare kind of malignant liver tumor that arises in the epithelial cells of the intrahepatic bile ducts, accounts for approximately 3.25% of primary liver malignancies. Sarcomatoid lesions are uncommon in epithelial malignancies and are more prevalent in the upper digestive tract, mammary gland, skin, uterus, urethra, gallbladder, and other organs[2]. HCC is most frequently associated with sarcomatoid alterations in the liver, which are present in approximately 2% of HCC cases treated with resection[3]. Less than 1% of liver cancer cases are sarcomatoid cholangiocarcinomas, and the pathophysiology of this relatively uncommon condition is still unknown[4]. Moreover, prognosis is poor in comparison to other liver cancers. Most commonly, adults over 40 who are middle aged or older develop intrahepatic sarcomatoid cholangiocarcinoma[5]. Imaging results can frequently be confused with hepatocellular carcinoma or other similar malignancies.\\u003c/p\\u003e\\n\\u003cp\\u003eHere, we present CT and MRI findings of intrahepatic sarcomatoid cholangiocarcinoma with a portal vein thrombus.\\u003c/p\\u003e\"},{\"header\":\"Case Presentation\",\"content\":\"\\u003cp\\u003eThe patient was a 36-year-old man who had received regular oral entecavir treatment for hepatitis B cirrhosis, which had been present for five years. This patient was recently admitted to the hospital with elevated alpha-fetoprotein. The patient had a family and genetic history of hepatitis B (his brother). A physical examination of the patient revealed no notable anomalies. Laboratory findings were as follows. Liver function biochemical parameters: total bilirubin, 49.3 \\u0026mu;mol/L \\u0026nbsp;(normal, \\u0026lt;23 \\u0026mu;mol/L), direct bilirubin, 15.97 \\u0026mu;mol/L (normal, \\u0026lt;6.8 \\u0026mu;mol/L) and indirect bilirubin, 33.33 \\u0026mu;mol/L (normal range 1.71-13.68 \\u0026mu;moL/L). Muscle enzyme profile: aspartate aminotransferase, 50.7U/L (normal: 0-40 U/L), alanine aminotransferase, \\u0026nbsp;373.92 U/L (normal: 9-50 U/L), alkaline phosphatase, 230.16 U/L (normal: 45-125 U/L), transglutaminase, 62 U/L (normal range: 11-50 U/L), total bile acids, 12.25 \\u0026mu;mol/L \\u0026nbsp;(normal range 0.1-10 \\u0026mu;mol/L) and glycocholic acid, 12.25 \\u0026mu;mol/L \\u0026nbsp;(normal range 0-2.7 \\u0026mu;mol/L). Tumor marker: alpha-fetoprotein, 461 ng/mL (normal range 0-8.1 ng/mL). Hepatic fibrosis combination analysis: hyaluronidase, 111.6 ng/mL and laminin, 9.84 ng/mL.\\u003c/p\\u003e\\n\\u003cp\\u003e\\u0026nbsp;The liver appeared to have an uneven shape and a rough surface on CT imaging. Segments 5 and 6 of the liver revealed a hypodense mass that was approximately 63 mm \\u0026times; 90 mm in size, had an amorphous shape, and had ill-defined edges. On enhancement scan, the lesion showed mild to moderate heterogeneous enhancement in the arterial phase, and patchy and nodular hypoenhancing regions were detected within the lesion in the delayed phase. In the right branch of the portal vein, a cast-filling defect with little localized enhancement was visible. The splenic vein was thickened, and the spleen\\u0026apos;s volume was increased(Figure 1). In comparison to the liver, the lesion showed low signal intensity on MRI and T1-weighted imaging. The lesion appeared as a slightly uneven hyper signal on T2-weighted imaging, along with many nodules and bands of T2 hypointense signal. Diffusion-weighted imaging (DWI) also revealed severe diffusion limitation, and the lesions had unevenly elevated signals. Expression diffusion coefficient (ADC) mapping displayed low signals. The lesion displayed heterogeneous and more significant enhancement on magnetic resonance dynamic enhancement, and the delayed phase revealed branching nonenhancing shadows inside the lesion. The intra- and extrahepatic bile ducts were not significantly dilated. The right portal vein tumor thrombus showed a low signal on T1-weighted scans but a significant signal on T2-weighted images. The tumor thrombus showed annular expansion in the arterial stage and concentric filling in the portal vein stage and the delayed stage (Figure 2). According to the patient\\u0026apos;s history of cirrhosis, clinical symptoms, and imaging signals such as splenomegaly and a portal vein cancer thrombus, the probability of hepatocellular carcinoma was taken into account before surgery. The enhanced pattern, however, led to differential diagnosis of cholangiocarcinoma.\\u003c/p\\u003e\\n\\u003cp\\u003eThe patient underwent lobectomy and cholecystectomy via laparoscopic surgery. A mass measuring approximately 5 x 4 cm in size, firm, and poorly defined, was found in the posterior portion of the right lower lobe of the liver. The liver was reddish-brown with minor cirrhotic alterations. Postoperative issues, including hemorrhage and biliary fistula, did not occur. He recovered well.\\u003c/p\\u003e\\n\\u003cp\\u003eHistopathological and immunohistochemical analyses of the mass showed that the tumor tissue was arranged in nests and sheets along the bile duct; the tumor cells were round or fusiform with pronounced atypia. Immunohistochemical analysis revealed positive staining for cytokeratin, low molecular cytokeratin, and cancer cell wave proteins. Vimentin, cytokeratin (CK)8, and CK-19) were all positive, but CK7, CK10, and hepatocyte paraffin 1 (Hepa-1)were all negative(Figure 3). The Ki-67 proliferation index was approximately 40%. It was determined that the patient had sarcomatoid cholangiocarcinoma based on the findings of histological and immunohistochemical analyses. \\u0026nbsp;\\u0026nbsp;\\u003c/p\\u003e\"},{\"header\":\"Discussion\",\"content\":\"\\u003cp\\u003eThe majority of hepatic sarcomatoid carcinomas are believed to be sarcomatoid HCCs; intrahepatic sarcomatoid cholangiocarcinoma is highly unusual. There are no known radiological signs for intrahepatic sarcomatoid cholangiocarcinomas. Histopathological and immunohistochemical tests are used to determine whether a patient has intrahepatic sarcomatoid cholangiocarcinoma, an uncommon type of disease that most frequently affects middle-aged and older persons over the age of 40 years[6]. Sarcomatoid cholangiocarcinoma is a rare form of intrahepatic cholangiocarcinoma that has been reported to account for 4.5% of cholangiocarcinoma cases\\u0026nbsp;[7], and its tumor cells usually have a definite malignant epithelial cholangiocyte and sarcomatoid mixed component\\u0026nbsp;[8]. The histological morphology of the tumor in this case was similar to\\u0026nbsp;that in earlier publications in this field[2], whereby the sarcomatoid component predominantly displays a spindle cell morphology. According to immunohistochemistry, these tumors are negative for Hepa-1 but positive for the mesenchymal tumor marker vimentin and epithelial cholangiogenic tumor marker (CK8). These immunohistochemistry findings offer hints for hepatocellular carcinoma, intrahepatic cholangiocarcinoma, and metastatic carcinoma differential diagnosis[7-10].\\u003c/p\\u003e\\n\\u003cp\\u003eIn our case, CT revealed a poorly defined hypodense mass, and dynamic enhanced CT and MRI revealed that the lesion had generally heterogeneous progressive and persistent enhancement. Peripheral contrast enhancement of the lesion was frequently seen in the arterial, portal, and late venous phases.\\u0026nbsp;The tumor margin initially exhibits irregular and mild enhancement; however, this enhancement gradually becomes stronger over time and can eventually become more visible in the delayed stage.\\u0026nbsp;The lesion in our case displayed a persistent or progressive pattern of intensification in the portal and delayed phases, which was comparable to the intensification pattern of cholangiocarcinoma[11]. This was in contrast to the surrounding liver parenchyma. However, no appreciable bile duct dilation was observed, and portal vein thrombosis is not a typical cholangiocarcinoma symptom.\\u003c/p\\u003e\\n\\u003cp\\u003eHCC, liver metastases, pancreatitis, pancreatic cancer, hypercoagulable state, liver cirrhosis, and other liver diseases are some of the conditions that might lead to portal vein cancer thrombosis[12].\\u0026nbsp;For patients with a clinical history of cirrhosis who develop a portal vein tumor thrombus in liver disease, HCC is typically a contributing factor[13].\\u0026nbsp;HCC enhancement patterns typically display early enhancement in the arterial phase, with quick declines in\\u0026nbsp;imaging in the portal and delayed phases[14]. However, in our case, the enhancement pattern was not consistent with the typical enhancement pattern of HCC. Indeed, the patient had a clinical history of cirrhosis and imaging manifestations such as portal vein carcinoma thrombosis and enlarged spleen volume, which suggested a high likelihood of HCC. Therefore, differential diagnosis between cholangiocarcinoma and HCC was difficult in this case.\\u003c/p\\u003e\\n\\u003cp\\u003eAccording to related reports, sarcomatoid cholangiocarcinoma, a very rare tumor with pathological changes in epithelial and mesenchymal components, is highly aggressive and has poor prognosis[15]. Its diagnosis is mostly achieved through a combination of histopathological and immunohistochemical examinations. Early detection and surgical removal of such tumors can provide patients with an increased likelihood of survival.\\u003c/p\\u003e\"},{\"header\":\"Conclusion\",\"content\":\"\\u003cp\\u003eWe report a case of sarcomatoid cholangiocarcinoma combined with portal vein carcinoma thrombosis. CT and MRI imaging performance for this entity is worthy of study by clinicians to improve the accuracy of diagnosis.\\u003c/p\\u003e\"},{\"header\":\"Abbreviations\",\"content\":\"\\u003cp\\u003eCT: computed tomography\\u003c/p\\u003e\\n\\u003cp\\u003eHCC: hepatocellular carcinoma\\u003c/p\\u003e\\n\\u003cp\\u003eMRI: magnetic resonance imaging\\u0026nbsp;\\u003c/p\\u003e\\n\\u003cp\\u003eCCC: cholangiocellular carcinoma\\u0026nbsp;\\u003c/p\\u003e\\n\\u003cp\\u003eDWI: diffusion-weighted imaging\\u0026nbsp;\\u003c/p\\u003e\\n\\u003cp\\u003eADC： apparent Diffusion Coefficient\\u003c/p\\u003e\"},{\"header\":\"Declarations\",\"content\":\"\\u003cp\\u003e\\u003cstrong\\u003eEthical approval and consent to participate\\u003c/strong\\u003e\\u003c/p\\u003e\\n\\u003cp\\u003eAll procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Informed consent was obtained from the participant in this study.\\u003c/p\\u003e\\n\\u003cp\\u003e\\u003cstrong\\u003eConsent for publication\\u003c/strong\\u003e\\u003c/p\\u003e\\n\\u003cp\\u003eThis patient consented to the reporting of this case in a scientific publication.\\u003c/p\\u003e\\n\\u003cp\\u003e\\u003cstrong\\u003eCompeting interests\\u0026nbsp;\\u003c/strong\\u003e\\u003c/p\\u003e\\n\\u003cp\\u003eThis research was not supported by any organization, and the authors have no conflict of interest.\\u0026nbsp;\\u003c/p\\u003e\\n\\u003cp\\u003e\\u003cstrong\\u003eAuthors\\u0026rsquo; contributions\\u003cbr\\u003e\\u0026nbsp;\\u003c/strong\\u003eTing Zhan wrote the article. Ting Zhan and Shan-Shan Xing participated in the histopathological analysis. All authors read and approved the final manuscript.\\u0026nbsp;\\u003c/p\\u003e\\n\\u003cp\\u003e\\u003cstrong\\u003eFunding\\u003c/strong\\u003e\\u003c/p\\u003e\\n\\u003cp\\u003eThe authors received no funding for the writing.\\u003c/p\\u003e\\n\\u003cp\\u003e\\u003cstrong\\u003eAvailability of data and materials\\u003c/strong\\u003e\\u003c/p\\u003e\\n\\u003cp\\u003eAll data generated are included in this article.\\u003c/p\\u003e\\n\\u003cp\\u003e\\u003cstrong\\u003eAcknowledgments\\u003c/strong\\u003e\\u003c/p\\u003e\\n\\u003cp\\u003eNot applicable\\u003c/p\\u003e\"},{\"header\":\"References\",\"content\":\"\\u003col\\u003e\\n\\u003cli\\u003eSchneider, G., et al., \\u003cem\\u003eMRI of sarcomatoid carcinoma of the liver.\\u003c/em\\u003e European Journal of Radiology Extra, 2005. \\u003cstrong\\u003e54\\u003c/strong\\u003e(2).\\u003c/li\\u003e\\n\\u003cli\\u003eMalhotra, S., et al., \\u003cem\\u003eIntrahepatic sarcomatoid cholangiocarcinoma.\\u003c/em\\u003e J Oncol, 2010. \\u003cstrong\\u003e2010\\u003c/strong\\u003e: p. 701476.\\u003c/li\\u003e\\n\\u003cli\\u003eYamaguchi, R., et al., \\u003cem\\u003eHepatocellular carcinoma with sarcomatous change.\\u003c/em\\u003e Oncol Rep, 1997. \\u003cstrong\\u003e4\\u003c/strong\\u003e(3): p. 525-9.\\u003c/li\\u003e\\n\\u003cli\\u003eKim, D.K., et al., \\u003cem\\u003eAnalysis of intrahepatic sarcomatoid cholangiocarcinoma: Experience from 11 cases within 17 years.\\u003c/em\\u003e World J Gastroenterol, 2019. \\u003cstrong\\u003e25\\u003c/strong\\u003e(5): p. 608-621.\\u003c/li\\u003e\\n\\u003cli\\u003eSintra, S., et al., \\u003cem\\u003eIntrahepatic sarcomatoid cholangiocarcinoma.\\u003c/em\\u003e BMJ Case Rep, 2018. \\u003cstrong\\u003e2018\\u003c/strong\\u003e.\\u003c/li\\u003e\\n\\u003cli\\u003eKaibori, M., et al., \\u003cem\\u003eIntrahepatic sarcomatoid cholangiocarcinoma.\\u003c/em\\u003e J Gastroenterol, 2003. \\u003cstrong\\u003e38\\u003c/strong\\u003e(11): p. 1097-101.\\u003c/li\\u003e\\n\\u003cli\\u003eSato, K., et al., \\u003cem\\u003eIntrahepatic sarcomatoid cholangiocarcinoma of round cell variant: a case report and immunohistochemical studies.\\u003c/em\\u003e Virchows Arch, 2006. \\u003cstrong\\u003e449\\u003c/strong\\u003e(5): p. 585-90.\\u003c/li\\u003e\\n\\u003cli\\u003eWatanabe, G., et al., \\u003cem\\u003ePrognosis analysis of sarcomatous intrahepatic cholangiocarcinoma from a review of the literature.\\u003c/em\\u003e Int J Clin Oncol, 2014. \\u003cstrong\\u003e19\\u003c/strong\\u003e(3): p. 490-6.\\u003c/li\\u003e\\n\\u003cli\\u003eWang, T., et al., \\u003cem\\u003eClinical features of sarcomatoid change in patients with intrahepatic cholangiocarcinoma and prognosis after surgical liver resection: A Propensity Score Matching analysis.\\u003c/em\\u003e J Surg Oncol, 2020. \\u003cstrong\\u003e121\\u003c/strong\\u003e(3): p. 524-537.\\u003c/li\\u003e\\n\\u003cli\\u003eZhang, N., et al., \\u003cem\\u003eSarcomatous intrahepatic cholangiocarcinoma: Case report and literature review.\\u003c/em\\u003e Medicine (Baltimore), 2018. \\u003cstrong\\u003e97\\u003c/strong\\u003e(39): p. e12549.\\u003c/li\\u003e\\n\\u003cli\\u003eWengert, G.J., et al., \\u003cem\\u003eDifferentiation of Intrahepatic Cholangiocellular Carcinoma from Hepatocellular Carcinoma in the Cirrhotic Liver Using Contrast-enhanced MR Imaging.\\u003c/em\\u003e Acad Radiol, 2017. \\u003cstrong\\u003e24\\u003c/strong\\u003e(12): p. 1491-1500.\\u003c/li\\u003e\\n\\u003cli\\u003eJain, P. and S. Nijhawan, \\u003cem\\u003ePortal vein thrombosis: etiology and clinical outcome of cirrhosis and malignancy-related non-cirrhotic, non-tumoral extrahepatic portal venous obstruction.\\u003c/em\\u003e World J Gastroenterol, 2007. \\u003cstrong\\u003e13\\u003c/strong\\u003e(39): p. 5288-9.\\u003c/li\\u003e\\n\\u003cli\\u003eSun, J., et al., \\u003cem\\u003eGuidelines for Diagnosis and Treatment of Hepatocellular Carcinoma with Portal Vein Tumor Thrombus in China (2021 Edition).\\u003c/em\\u003e Liver Cancer, 2022. \\u003cstrong\\u003e11\\u003c/strong\\u003e(4): p. 315-328.\\u003c/li\\u003e\\n\\u003cli\\u003eRimola, J., et al., \\u003cem\\u003eCholangiocarcinoma in cirrhosis: absence of contrast washout in delayed phases by magnetic resonance imaging avoids misdiagnosis of hepatocellular carcinoma.\\u003c/em\\u003e Hepatology, 2009. \\u003cstrong\\u003e50\\u003c/strong\\u003e(3): p. 791-8.\\u003c/li\\u003e\\n\\u003cli\\u003eLi, X., et al., \\u003cem\\u003eSarcomatoid intrahepatic cholangiocarcinoma in a patient with poor prognosis: a case report and literature review.\\u003c/em\\u003e J Int Med Res, 2020. \\u003cstrong\\u003e48\\u003c/strong\\u003e(11): p. 300060520969473.\\u003c/li\\u003e\\n\\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\":\"info@researchsquare.com\",\"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\":\"intrahepatic sarcomatoid cholangiocarcinoma, hepatobiliary malignancy, sarcomatoid change, portal vein thrombus, CT, MRI\",\"lastPublishedDoi\":\"10.21203/rs.3.rs-2733045/v1\",\"lastPublishedDoiUrl\":\"https://doi.org/10.21203/rs.3.rs-2733045/v1\",\"license\":{\"name\":\"CC BY 4.0\",\"url\":\"https://creativecommons.org/licenses/by/4.0/\"},\"manuscriptAbstract\":\"Less than 1% of malignant tumors of the hepatobiliary system are intrahepatic sarcomatoid cholangiocarcinomas, a rare malignant tumor of the liver. Based only on clinical presentation and imaging, this entity is readily mistaken for hepatocellular carcinoma. In this article, we describe the case of a 36-year-old male patient who had a history of \\\"cirrhosis B\\\" and was hospitalized for six months due to increased alpha-fetoprotein. Magnetic resonance imaging (MRI) and computed tomography (CT) revealed a 63x 90 mm mass in segments V and VI of the liver, with thrombosis in the right branch of the portal vein. Following the patient's laparoscopic lobectomy and cholecystectomy, sarcomatoid cholangiocarcinoma of the right side of the liver was discovered by histopathological and immunohistochemical examinations. This case report seeks to increase understanding of intrahepatic sarcomatoid cholangiocarcinoma and its diagnosis while reducing the occurrence of clinical misinterpretation.\",\"manuscriptTitle\":\"Intrahepatic sarcomatoid cholangiocarcinoma with portal vein thrombus misdiagnosed as hepatocellular carcinoma: description of a rare case\",\"msid\":\"\",\"msnumber\":\"\",\"nonDraftVersions\":[{\"code\":1,\"date\":\"2023-03-28 20:04:02\",\"doi\":\"10.21203/rs.3.rs-2733045/v1\",\"editorialEvents\":[{\"type\":\"communityComments\",\"content\":0}],\"status\":\"published\",\"journal\":{\"display\":true,\"email\":\"info@researchsquare.com\",\"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\":\"7a535565-6ec7-4b43-bbc0-61f978b945d8\",\"owner\":[],\"postedDate\":\"March 28th, 2023\",\"published\":true,\"recentEditorialEvents\":[],\"rejectedJournal\":[],\"revision\":\"\",\"amendment\":\"\",\"status\":\"posted\",\"subjectAreas\":[],\"tags\":[],\"updatedAt\":\"2023-03-30T04:29:19+00:00\",\"versionOfRecord\":[],\"versionCreatedAt\":\"2023-03-28 20:04:02\",\"video\":\"\",\"vorDoi\":\"\",\"vorDoiUrl\":\"\",\"workflowStages\":[]},\"version\":\"v1\",\"identity\":\"rs-2733045\",\"journalConfig\":\"researchsquare\"},\"__N_SSP\":true},\"page\":\"/article/[identity]/[[...version]]\",\"query\":{\"redirect\":\"/article/rs-2733045\",\"identity\":\"rs-2733045\",\"version\":[\"v1\"]},\"buildId\":\"WrCJVZZCHTDjtuVLN7oU0\",\"isFallback\":false,\"isExperimentalCompile\":false,\"dynamicIds\":[84888],\"gssp\":true,\"scriptLoader\":[]}","source_license":"CC-BY-4.0","license_restricted":false}