Inflammatory Myofibroblastic Tumor of the Liver: A Diagnostic Challenge.

Abstract

Inflammatory myofibroblastic tumor (IMT) is an uncommon myofibroblastic neoplasm that was formerly included within the broad category of inflammatory pseudotumor (IPT). IMT is rarely encountered in the liver. Similar to IMT of other organs, the interchangeable use of the terms IMT and IPT in liver has made the analysis of these lesions difficult. In this review, clinical and pathological features of IMT of the liver are reviewed and the differential diagnosis of IMT is discussed, with emphasis on IPT and the other entities included in this large category. IMT can mimic malignant tumors. There are no known unique diagnostic clinical, laboratory, or radiological features. The definitive diagnosis of IMT depends on careful pathological examination. The histopathological evaluation of hepatic IMT reveals that, the myxoid/vascular pattern is the most frequently observed, followed by, in decreasing frequency, fibrous histiocytoma-like pattern and hypocellular fibrous pattern. In IMT of the liver, anaplastic lymphoma kinase (ALK) expression reliably predicts the presence of an ALK gene rearrangement. The diagnosis of hepatic IMT depends on the dominant histopathological pattern, and the management of the disease is still controversial. IMT of the liver is a distinctive neoplasm of intermediate biological potential, and should be distinguished from the variety of lesions that are included under the broad category of IPT. Therefore, to avoid confusion regarding the true incidence and behavior of hepatic IMT, the term IPT should not be used interchangeably with IMT. The rarity of IMT in liver should not minimize its consideration in the differential diagnosis of liver tumors, especially in patients with tumor markers in normal range.