Inflammatory angiomyolipoma of the liver: an unusual case suggesting relationship to IgG4-related pseudotumor.

Inflammatory angiomyolipoma of the liver: an unusual case suggesting relationship to IgG4-related pseudotumor. Int J Clin Exp Pathol. 2013;6(4):771-9 Authors: Agaimy A, Märkl B Abstract Hepatic angiomyolipoma (AML) is rare. Based on its wide histomorphological range, several distinctive histological variants have been delineated. However, hepatic AML displaying predominantly or exclusively inflammatory pattern closely mimicking inflammatory pseudotumor (IPT) is exceptionally rare with only 7 cases reported so far. We herein describe a new case of hepatic inflammatory AML in a 51-year-old woman who presented with unexplained constitutional symptoms suggesting an infectious disease. A liver mass was detected during imaging examination and resected (4.3 cm in maximum diameter). The patient's symptoms resolved completely after surgery. Currently, she is alive and well 7 years after surgery. She has no evidence of other organ manifestations of IgG4-related systemic disease. The tumor displayed a pure IPT-like histological pattern with dense infiltrates of plasma cells, lymphocytes and histiocytes admixed with scattered few adipocytes, irregularly distributed thick-walled vessels (some of them showed obliterative phlebitis) as well as aggregates and fascicles of histiocytoid and spindle-shaped myoid cells that were immunoreactive for HMB45 and Melan A with focal expression of alpha smooth muscle actin. Lesional cells were negative for desmin, prote...
Source: International Journal of Clinical and Experimental Pathology - Category: Pathology Authors: Tags: Int J Clin Exp Pathol Source Type: research