Inflammatory myofibroblastic tumor of the liver

Inflammatory myofibroblastic tumor is a rare benign entity formerly known as inflammatory pseudotumor. Involvement of the liver is extremely rare. There are controversies about the optimal treatment of this benign entity. Newer reports suggest an association with autoimmune sclerosing pancreatitis and primary sclerosing cholangitis. We present a case of an 18-year-old patient with biliary obstruction from a perihilar mass of the liver requiring hepatic resection. Division of the hepatic bile duct resulted in drainage of yellow, thick, gelatinous material in the presence of benign margins and absence of cholangitis. Histological examination showed a mass with fibroblastic and myofibroblastic cells set in a loose myxoid matrix containing scattered lymphocytes, consistent with an inflammatory myofibroblastic tumor. One-year recovery was uneventful. This report discusses the presentation, diagnosis, and controversies in management of this disease.