An unusual association of inflammatory pseudotumor of the liver and dorsal pancreatic agenesis presenting as reversible portal hypertension: a case report.

Inflammatory pseudotumor of liver although a rare entity is an important differential diagnosis of hepatic space occupying lesions as well as an important cause of portal hypertension, commonly mimicking malignant tumors on imaging and histology. We report a case of a 25-year-old postpartum female who presented to our emergency with seizures and altered sensorium and was found to be having uncontrolled hyperglycemia and metabolic encephalopathy. She had a 2 month history of low grade fever and pain in right hypochondrium. Examination revealed an enlarged, tender, left lobe of the liver, splenomegaly and ascites. CT scan of the abdomen revealed an ill-defined mass lesion in left lobe of the liver with dilated intrahepatic biliary radicles along with dorsal pancreatic agenesis and evidence of portal hypertension in the form of ascites and splenomegaly. Histopathology confirmed the presence of inflammatory pseudotumor in left lobe of the liver. Conservative management of the patient resulted in reduction of the tumor size and regression of splenomegaly and ascites in 1 month. Present case highlights inflammatory pseudotumor of liver as a rare entity, it's resemblance to malignant conditions, pancreatic agenesis as a previously unreported association as well as a potentially reversible cause of portal hypertension by conservative treatment only.