Pseudo-Tumoral Presentation of Portal Cavernous Cholangiopathy: A Diagnostic Pitfall

Portal cavernous cholangiopathy (PCC), also known as portal biliopathy, represents biliary abnormalities resulting from cavernous transformation of the portal vein secondary to chronic portal vein thrombosis. 1 2 The formation of extensive peribiliary venous collaterals may lead to extrinsic compression, ischemia, and fibrosis of the biliary tree, producing imaging findings that can closely mimic primary biliary malignancies. 3 4 5 Among its various forms, the pseudo-tumoral variant is exceedingly rare and poses a significant diagnostic challenge. 3 6 We report three cases of pseudo-tumoral PCC encountered at our tertiary care center. All patients presented with non-specific abdominal pain and mild derangement of liver enzymes without significant hyperbilirubinemia. Imaging initially suggested malignant etiologies, such as cholangiocarcinoma or lymphoma, due to the presence of soft tissue thickening encasing the common bile duct and hepatic ducts. However, further evaluation revealed hallmark features of chronic portal vein thrombosis, including non-visualization of the portal vein, cavernoma formation, and extensive periportal collaterals 4 7 ( Fig. 1 ). Fig. 1 Arterial phase CT images showing an irregular soft tissue mass at the porta encasing the hepatic arteries ( A ). Venous phase images showing enhancing soft tissue mass at the porta (marked by white arrow) encasing the central biliary ducts ( B ) (red arrow) with no definite ductal dilatation. PET images showing no FDG uptake within the soft tissue thickening ( C ). Axial section of arterial phase of CEMRI showing non-enhancing thickening around CHD and CBD ( D ). Axial section of venous phase image showing homogenous enhancement of the thickening ( E ). DWI image showing no restriction of diffusion of the thickening ( F ).Coronal section of the arterial phase showing non-enhancing thickening around CHD and CBD ( G ). Coronal section of the venous phase image showing homogenously enhancing soft tissue thickening around CHD and CBD ( H ). T1 fs venous contrast MRI images showing homogenous enhancement on venous phase ( I ). CBD, common bile duct; MRI, magnetic resonance imaging; PET, positron emission tomography; CEMRI, contrast enhanced magnetic resonance imaging; CHD, common hepatic duct, T1 fs, T1 weighted fat sat. Contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) were instrumental in establishing the diagnosis by demonstrating homogeneous enhancement of the soft tissue thickening in the portal venous phase, absence of diffusion restriction, and lack of significant lymphadenopathy. 6 7 In one case, positron emission tomography computed tomography (PET-CT) showed no FDG uptake, further supporting a benign etiology. 8 Pseudo-tumoral PCC corresponds to the fibrotic variant of portal biliopathy and is characterized by circumferential fibrous thickening around the biliary ducts. 3 6 Differentiating this benign entity from malignancies such as cholangiocarcinoma or IgG4-related sclerosing cholangiopathy is critical, as misdiagnosis may lead to unnecessary invasive procedures. 5 9 Notably, biopsy in such cases carries a high risk of hemorrhage due to the presence of collateral vessels and should be avoided when imaging findings are characteristic. 10 A high index of suspicion and careful interpretation of imaging findings are essential for accurate diagnosis. Recognition of associated features such as portal cavernoma, collateral channels, and typical enhancement patterns can help distinguish PCC from malignant mimics and prevent unwarranted interventions. In conclusion, pseudo-tumoral PCC, though rare, should be considered in the differential diagnosis of biliary mass lesions, particularly in the presence of portal vein thrombosis and cavernous transformation. Increased awareness among clinicians and radiologists is crucial to avoid misdiagnosis and ensure appropriate management. Publication History Article published online: 18 June 2026 © 2026. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/) Thieme Medical and Scientific Publishers Private Limited A-13A, Graphix Tower 1, 6th floor, Sector 62, Noida 201309, Uttar Pradesh, India