2009 Southern Regional Meeting Abstracts

Case Report: Clinical History: We describe the case of a 25-year-old African-American male who was admitted to one of our teaching hospitals with a 6-month history of intermittent chest pain and shortness of breath. Transthoracic echocardiography showed a 2x6 cm right ventricular mass. The patient was taken to the operating room, but the mass proved technically impossible to remove. Intraoperative biopsy specimens were taken.
Surgical Pathology: Fibrovascular tissue with florid chronic inflammation and histiocytic reaction. Immunohistochemical stains for actin and CD-68 were positive. Based on these findings a diagnosis of inflammatory myofibroblastic tumor was made.
Discussion: Inflammatory myofibroblastic tumor (IMT) is a rare lesion in adults consisting mainly of spindle-shaped mesenchymal and inflammatory cells. IMT most commonly involves the lung and the orbit, but it has been reported to occur in nearly every site in the body. IMT is probably a neoplasm rather than a post-inflammatory process because of cytogenetic clonality, recurrent involvement of chromosomal region 2p23, occasional aggressive local behavior, and its metastatic nature. Histochemically and immunochemically IMT is characterized by the expression of vimentin, smooth muscle actin, and cytokeratins, corresponding to that of myofibroblasts along with other inflammatory markers. In recent years attention has focused on the putative role of Human Herpes virus 8 and Epstein Barr virus in the activation of proto-oncogenes and anti-apoptotic factors leading to neoplastic transformation to IMT. The definition and characterization of IMT is an evolution in progress. Though rare, this neoplasm often afflicts younger patients and should be kept in the differential diagnosis of undiagnosed masses in various locations as early diagnosis may lead to resection and cure. Delay in diagnosis may lead to unresectability as was unfortunately the case in our index patient.