OBJECTIVE. The purpose of this review is to describe the pathophysiologic findings, differential diagnosis, imaging features, and management of inflammatory pseudotumor in various locations throughout the body. CONCLUSION. Inflammatory pseudotumor is a rare benign process mimicking malignant processes and has been found in almost every organ system. Radiologists should be familiar with this entity as a diagnostic consideration to avoid unnecessary surgery.

https://www.ajronline.org/doi/pdf/10.2214/AJR.11.7288

Inflammatory Pseudotumor: The Great Mimicker

Cardiac tumors other than myxomas are rare. We report a series of 10 intracavitary polypoid myofibroblastic proliferations in children and young adults emphasizing gross, histologic, and clinical features. There were 6 females and 4 males, with a mean age of 10 years (range 5 wk to 21 y). All lesions were endocardial-based, located in the right atrium (1), right ventricular inflow/tricuspid valve (1), right ventricular outflow (3), mitral valve (3), aortic valve/left coronary sinus (1), and left ventricular free wall (1). Symptoms included shortness of breath or dyspnea (3), syncope (2), chest pain (1), transient ischemic attacks (1), and fever with myalgias (1). All tumors were surgical resections, except 1 tumor that resulted in sudden coronary death and that was diagnosed at autopsy, and 1 tumor that embolized into the coronary artery and was treated by cardiac transplant. Two tumors, present in the aortic and mitral valves, respectively, caused cardiac ischemia. The tumors were polypoid or filiform and histologically resembled inflammatory myofibroblastic tumors of extracardiac sites, with loose spindle cell growth with sparse inflammation. Although there were frequent collagen bundles interspersed among the tumor cells, there were no large areas of dense fibrosis. Surface fibrin was present on the polypoid projections in 7 cases. Symptoms resulted from prolapse into coronary ostia or embolization, but no patient developed metastasis. Long-term follow-up in 2 patients demonstrated no evidence of disease or recurrence. Although metastatic potential was not identified, these tumors may result in serious symptoms, including myocardial infarct, syncope, and sudden death. These cardiac myofibroblastic tumors are readily distinguished from other endocardial-based cardiac tumors, including papillary fibroelastoma and myxoma, which may present clinically in the same manner.

Cardiac inflammatory myofibroblastic tumor: a "benign" neoplasm that may result in syncope, myocardial infarction, and sudden death.

https://www.annalsthoracicsurgery.org/article/S0003-4975(12)02683-5/pdf

Immunoglobulin G4-related disease of the heart causing aortic regurgitation and heart block.

Inflammatory myofibroblastic tumor (IMT) or inflammatory pseudotumor is a rare primary cardiac tumor that may result in sudden death. We report a sudden unexpected death due to occlusion of the coronary arteries by IMT arising from the left coronary cusp of the aortic valve. An 8-year-old child suddenly woke up from his sleep with complaint of severe chest pain to his parents, and shortly he became unresponsive. He expired 40 min later in the hospital despite resuscitation efforts. The postmortem examination revealed a 2.5 × 2 × 1-cm mass composed of multiple entangled slender cylindrical fronts, filling the coronary sinus and obstructing the coronary ostia. The patient had complained of recurrent chest pains about 2 weeks prior to his death. Echocardiogram was conducted on the patient but did not recognize the mass. Histological examination of the mass established the diagnosis of primary cardiac IMT. The detailed pathological findings are described. In addition, the literature is reviewed, and pathogenesis, clinical presentation, and the importance of forensic autopsy examination are discussed.

Sudden unexpected death due to inflammatory myofibroblastic tumor of the heart: a case report and review of the literature

Although inflammatory myofibroblastic tumors (IMTs) can be found in virtually every major organ, cardiac origin is rare. After recently providing care to a child who presented with a significant myocardial infarction, interest in this rare tumor was piqued. We describe a comprehensive review of cardiac IMT, including information on nomenclature, epidemiology, clinical features, pathogenesis, gross/histological features, immunohistochemical profile, diagnosis, treatment, and prognosis. Fifty-seven cases were identified in the literature. Interestingly, our case represents the seventh case of coronary artery involvement reported. Moreover, it was found that an initial presentation of sudden death most commonly involves the coronary arteries.

Lingaraj Krishna

Papers

Inflammatory pseudotumor of the heart causing aortic regurgitation.