Journal Article•
Inflammatory myofibroblastic tumor of the right ventricle causing tricuspid valve regurgitation.
TL;DR: A case of inflammatory myofibroblastic tumor that involved the right ventricle, thereby causing tricuspid valve regurgitation in an 18-year-old man who presented with a fever of unknown origin and of 1 month's duration is described.
Abstract: Cardiac inflammatory myofibroblastic tumor is a rare lesion consisting of inflammatory cells and myofibroblastic spindle cells. We describe a case of inflammatory myofibroblastic tumor that involved the right ventricle, thereby causing tricuspid valve regurgitation in an 18-year-old man who presented with a fever of unknown origin and of 1 month's duration. With the patient on cardiopulmonary bypass, we excised the lesion and replaced the tricuspid valve without serious intraoperative or postoperative sequelae. The patient had a favorable outcome.
Citations
More filters
TL;DR: Inflammatory pseudotumor is a rare benign process mimicking malignant processes and has been found in almost every organ system and Radiologists should be familiar with this entity as a diagnostic consideration to avoid unnecessary surgery.
Abstract: 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.
267 citations
Cites background from "Inflammatory myofibroblastic tumor ..."
...There have been reports of sudden death as a result of cardiac IPT [46–48]....
[...]
...The sites of involvement vary, but the right atrium and right ventricle are the most common [46–48]....
[...]
TL;DR: 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 and expired 40 min later in the hospital despite resuscitation efforts, report a sudden unexpected death due to occlusion of the coronary arteries by IMT arising from the left coronary cusp of the aorta.
Abstract: 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.
26 citations
Cites background from "Inflammatory myofibroblastic tumor ..."
...The pathogenesis of IMT remains unclear, although the tumors were thought to be an abnormal and exaggerated immunological response by proliferated spindle cells and primary myofibroblasts to injury, inflammation, and infection [5, 6]....
[...]
...Cardiac IMTs mostly occur in children and young adults, and have involved various sites; the right atrium and right ventricle are more predominant [6, 7, 18]....
[...]
...When it does, however, it is potentially fatal and may result in sudden death [6, 7]....
[...]
...Cardiac IMT is generally considered biologically benign with favorable prognosis [6, 7]....
[...]
...Currently, surgical resection of the tumor remains the definitive treatment and has a satisfactory outcome [6]....
[...]
TL;DR: A comprehensive review of cardiac IMT is described, including information on nomenclature, epidemiology, clinical features, pathogenesis, gross/histological features, immunohistochemical profile, diagnosis, treatment, and prognosis.
Abstract: 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.
24 citations
TL;DR: A 35‐year‐old Behçet's disease patient is reported with pseudotumor of the right ventricle and multiple pulmonary emboli and histopathologic analysis identified an inflammatory infiltrate composed of lymphocytes, plasma cells, and other inflammatory cells, without mitosis.
Abstract: Inflammatory pseudotumor is a rare benign neoplasm. It is common in children and has been reported in various locations throughout the body but rarely in the heart. Behcet's disease is a multisystemic, recurrent, inflammatory disorder. We report a 35-year-old Behcet's disease patient with pseudotumor of the right ventricle and multiple pulmonary emboli. Transthoracic echocardiography showed dilation of right atrium, right ventricle, and a mass in the right ventricle. Multislice computed tomography revealed a large, poorly defined mass infiltrating the groove and multiple pulmonary emboli. Surgery was performed with diagnostic and therapeutic intent. Following sternotomy, we identified a mass in the anterior wall of right ventricle, the outflow tract, and the inflow tract of right ventricle. The histopathologic analysis identified an inflammatory infiltrate composed of lymphocytes, plasma cells, and other inflammatory cells, without mitosis. And there were also some thrombus on top of the mass.
11 citations
TL;DR: The case of a 9-year-old girl who had sudden cardiac death as a result of occlusion of the left circumflex coronary artery is reported.
Abstract: Cardiac inflammatory myofibroblastic tumor (IMT) is a rare entity affecting predominantly infants, children, and young adults. Although most tumors have a benign clinical course after complete surgical resection, some have significant clinical effects. We report the case of a 9-year-old girl who had sudden cardiac death as a result of occlusion of the left circumflex coronary artery. A review of 57 cases of cardiac IMTs reported in the literature in terms of epidemiology, clinical presentation, histologic and immunohistologic features, and outcome is presented. Recognition of this rare abnormality is important in order to initiate prompt surgical intervention.
9 citations
References
More filters
TL;DR: In this paper, the authors report their experience with 84 cases occurring in the soft tissues and viscera of 48 female patients and 36 male patients between the ages of 3 months and 46 years (mean, 9.7 years; median, 9 years).
Abstract: Inflammatory myofibroblastic tumor (IMT) or inflammatory pseudotumor is a spindle cell proliferation of disputed nosology, with a distinctive fibroinflammatory and even pseudosarcomatous appearance. Although the lung is the best known and most common site, inflammatory myofibroblastic tumor occurs in diverse extrapulmonary locations. We report our experience with 84 cases occurring in the soft tissues and viscera of 48 female patients and 36 male patients between the ages of 3 months and 46 years (mean, 9.7 years; median, 9 years). A mass, fever, weight loss, pain, and site-specific symptoms were the presenting complaints. Laboratory abnormalities included anemia, thrombocytosis, polyclonal hypergammaglobulinemia, and elevated erythrocyte sedimentation rate. Sites of involvement included abdomen, retroperitoneum, or pelvis (61 cases); head and neck, including upper respiratory tract (12 cases); trunk (8 cases); and extremities (3 cases). The lesions ranged in size from 1 to 17 cm (mean, 6.4; median, 6.0). Excision was performed in 69 cases. Eight had biopsy only. Five patients received chemotherapy or radiation in addition to undergoing biopsy or resection as initial treatment. Sixteen patients had multinodular masses involving one region. Clinical follow-up in 53 cases revealed that 44 patients were alive with no evidence of disease, four were alive with IMT, and five were dead. Thirteen patients had one or more recurrences at intervals of 1-24 months (mean, 6 months; median, 10 months). No distant metastases were documented. The five patients who died had complications either due to the location of the lesion (heart, peritoneum, retroperitoneum, or mesentery) or related to treatment (lymphoproliferative disorder following hepatic transplantation; sepsis following wound infection). The abdominal masses were the largest. All tumors were firm and white with infiltrative borders and focal myxoid change. Three basic histologic patterns were recognized: (a) myxoid, vascular, and inflammatory areas resembling nodular fasciitis; (b) compact spindle cells with intermingled inflammatory cells (lymphocytes, plasma cells, and eosinophils) resembling fibrous histiocytoma; and (c) dense plate-like collagen resembling a desmoid or scar. Immunohistochemistry demonstrated positivity for vimentin, muscle-specific actin, smooth muscle actin, and cytokeratin consistent with myofibroblasts. Based on this series, inflammatory myofibroblastic tumor is a benign, nonmetastasizing proliferation of myofibroblasts with a potential for recurrence and persistent local growth, similar in some respects to the fibromatoses.
1,429 citations
TL;DR: It is suggested that EBV plays a role in a significant number of cases of inflammatory pseudotumor with differences in the incidence of EBV infection and the cell type infected when extranodal and nodal cases are compared, suggesting a difference in pathogenesis.
296 citations
TL;DR: 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.
Abstract: 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.
64 citations
TL;DR: Based on clinical manifestations, laboratory findings, histologic and ultrastructural characteristics of the mass, the speculation is put forth that this lesion may represent a cardiac myxoma with atypical structural features as mentioned in this paper.
Abstract: An intracardiac tumor arising in the right atrium of a young child is reported. Morphologically, the lesion was reminiscent of “plasma cell granulomas,” but unprecedented in this location. Based on clinical manifestations, laboratory findings, histologic and ultrastructural characteristics of the mass, the speculation is put forth that this lesion may represent a cardiac myxoma with atypical structural features. An extension of this proposal is a pathogenetic sequence that would try to reconcile the complex clinical course of some myxomas with the described structural observations. Alternative choices for classification of the lesion are also given consideration.
50 citations
TL;DR: The study shows that IMT must be considered in the differential diagnosis of cardiac fibroma especially in cases of inflammatory syndrome, location outside the ventricular myocardium, or multinodular lesions.
41 citations