Inflammatory pseudotumor of the liver with primary sclerosing cholangitis

Abstract

Inflammatory pseudotumor (IPT) of the liver is a rare benign variant of hepatic masses, and its exact etiology has not been elucidated. We report a case of IPT associated with primary sclerosing cholangitis (PSC). The patient was a 50-year-old man admitted to our hospital because of jaundice. Abdominal ultrasonography (US) and computed tomography showed multiple dilations of the intrahepatic bile ducts and multiple masses in the liver. On magnetic resonance imaging, the masses were slightly hypointense on T1-weighted images and slightly hyperintense on T2-weighted images. On T1-weighted images after the bolus infusion of Gd chelate, the masses had no contrast enhancement, and they were hypointense in the arterial phase and portal venous phase. However, they were slightly enhanced and became almost isointense relative to the surrounding normal liver parenchyma in the delayed phase. Endoscopic retrograde cholangiography demonstrated multiple irregular strictures and dilations of the intrahepatic bile ducts. Angiography demonstrated no abnormal findings, but, interestingly, subsequent dynamic CO2-enhanced US showed a strongly hyperechoic string, indicating that an artery had penetrated through the hypoechoic mass. A US-guided percutaneous needle biopsy revealed that the lesions were morphologically comparable to IPT. After cholangiography and microscopic analysis of the tumor, the final diagnosis was determined to be IPT of the liver with PSC. A number of previous reports have suggested a possible relationship between IPT and PSC, based on pathological findings. This report confirmed, based on clinical findings, that PSC is one of the causes of hepatic IPT.

Inflammatory pseudotumor of the liver associated with malignant disease: report of two cases and a review of the literature

“…The inflammatory pseudotumor (IPT) of the liver is a rare, benign tumor-like lesion [6,15], and about 60 cases have been reported in the English-language literature [17]. Some cases of IPT of the liver have been associated with malignant tumors [1, 7-9, 11, 13, 16].…”

Inflammatory Pseudotumor of the Liver in Patient with Type 2 Diabetes

“…Many mechanisms and hypothesized etiologic factors like infections, trauma, autoimmunity reactions, radiotherapy, and local reaction to changes in the walls of bile ducts are suggested [1][2][3][4][5][6][7]10,13,[18][19][20][21][22][23][24][25][26]. Some microorganisms that access the portal vein including Escherichia coli, Staphylococcus aureus, Klebsiella pneumoniae, Gram-positive cocci and Actinomyces were regarded as causative agents.…”

“…Viral infections (Epstein-Barr virus [20], hepatitis B and C virus) [10,27] and parasitic infestations (Schistosoma mansoni) may play role in the pathogenesis [18]. Some authors suggested that disturbance of biliary drainage, e.g., due to biliary stones [1,7], recurrent pyogenic cholangitis [4,26] or sclerosing cholangitis is also important [25]. The tumor was also noted in HIV infected persons [24].…”

Inflammatory Pseudotumor of the Liver Complicated with Recurrent Gouty Arthritis.

“…Nonomuraet al reported a case of IPT with marked destructive and sclerosing cholangitis mimicking PSC (12). More recently, Toda et al reported a case of IPT with definite PSC (13). Autoimmunemechanism was suggested in the pathogenesis of PSC because of the presence of autoantibodies.…”