Inflammatory pseudotumor of the liver: Demographics, diagnosis, and the case for nonoperative management

BACKGROUND: Inflammatory pseudotumor of the liver (IPL) is an unusual tumor-like condition that is becoming recognized as an important differential diagnosis in the patient presenting with liver masses. This report describes six cases of IPL. STUDY DESIGN: Clinical, diagnostic, pathologic, and followup data were collected prospectively on six patients presenting to a specialist hepatobillary unit. RESULTS: Six patients with IPL presented over a 2-year period. Median age was 35 years (range 2 to 79 years) and five patients were men. Three patients were Polynesian (Tongan and Samoan) and one was New Zealand Maori. Five patients presented with nonspecific symptoms (fever, arthralgia, myalgia) and IPL was an incidental finding in one patient. At presentation, four patients had elevated white cell counts, and five patients had abnormal liver function tests with elevations in alkaline phosphotase and gamma-glutamyl transferase the most commonly seen. Carcinogenic embryonic antigen and alpha fetoprotein were normal in all patients, although one was a known hepatitis B carrier. In all cases of IPL, diagnosis was made on core biopsy of the liver lesions and all patients were managed nonoperatively with complete resolution of the tumors. Two patients had marked reduction in systemic symptoms (fever and pain) from a short course of oral steroids. CONCLUSIONS: IPL appears to be a relatively common problem in Maori and Polynesians. Recognition and differentiation of this condition from malignant liver lesions are important to avoid unnecessary surgery. (J Am Coll Surg 2003; 196:226-235. (C) 2003 by the American College of Surgeons).