Cardiac Inflammatory Myofibroblastic Tumor
About: Cardiac Inflammatory Myofibroblastic Tumor is a(n) research topic. Over the lifetime, 22 publication(s) have been published within this topic receiving 1544 citation(s).
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.
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.
01 May 2000-Cardiology in The Young
TL;DR: An unusual case of rapid recurrence of a previously excised inflammatory myofibroblastic tumor of the right ventricular outflow tract in a 5-month-old infant is described, and the infant is asymptomatic 18 months after the second surgery.
Abstract: We describe an unusual case of rapid recurrence of a previously excised inflammatory myofibroblastic tumor of the right ventricular outflow tract in a 5-month-old infant The infant is asymptomatic 18 months after the second surgery The very rare cardiac involvement, and the early relapse of the inflammatory pseudotumor, to the best of our knowledge, is a unique combinationThe inflammatory myofibroblastic tumor, as known as a pseudotumor or plasma cell granuloma is an uncommon reactive lesion with unknown aetiology It is found most commonly in the lung and a number of visceral organs, such as the spleen, liver, ileum, salivary glands, urinary bladder, larynx or brain or in the retroperitoneum and lymph nodes To our knowledge only 9 cases have been published of such tumor arising within the heart1–6
TL;DR: The successful treatment of a 2-month-old boy with multiple inflammatory myofibroblastic tumors in the right ventricle protruding into the right atrium through the tricuspid annulus is reported.
Abstract: Reported is the successful treatment of a 2-month-old boy with multiple inflammatory myofibroblastic tumors in the right ventricle protruding into the right atrium through the tricuspid annulus. The patient underwent subtotal excision on cardiopulmonary bypass with no intraoperative or postoperative complications. Cardiac inflammatory myofibroblastic tumors are rare lesions, and surgical resections in patients as young as 2 months have not been previously described. In this report we review the relevant literature and discuss the prevalence, histology, pathogenesis, clinical manifestations, diagnosis, differential diagnosis, and treatment of cardiac inflammatory myofibroblastic tumor.
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.
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