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J Thorac Cardiovasc Surg 1994;108:862-870
© 1994 Mosby, Inc.
SURGERY FOR ACQUIRED HEART DISEASE |
Washington, D.C., and Baltimore, Md.
Received for publication Jan. 6, 1994. Accepted for publication April 19, 1994. Address for reprints: Allen P. Burke, MD, Department of Cardiovascular Pathology, Armed Forces Institute of Pathology, Washington, D.C. 20306-6000.
Abstract
The clinicopathologic findings of 23 patients with cardiac fibroma are presented. The mean patient age was 13 years, with a range of 1 day to 56 years. The presenting symptoms included heart failure, arrhythmias, sudden death, cyanosis, and chest pain. Some patients had no symptoms, and one patient had Gorlin's syndrome. Echocardiography and magnetic resonance imaging were very sensitive in diagnosis. Nineteen patients underwent tumor resection or biopsy, and four tumors were diagnosed at cardiac transplantation or autopsy on explanted hearts. In five surgical cases, complex operations were necessary, including pericardial or synthetic patches, valve replacement, or coronary artery grafting. Two operative deaths occurred in patients with surgical resections and four operative deaths in patients who at the time of the operation were deemed to have inoperable disease and only biopsy was performed. All deaths but one were in patients younger than 2 years of age. Four patients with subtotal resections did well after the operation. Dense collagen was more frequent in tumors in older patients. We conclude that fibromas are congenital tumors that are frequently discovered in adolescents and adults. Surgical treatment is generally successful, and imaging techniques are helpful in preoperative assessment. (J THORACCARDIOVASCSURG1994;108:862-70)
Cardiac fibroma is a rare benign tumor of the heart that occurs primarily in infants and children. Despite a number of recent reports of single patients or small numbers of patients,
1-8 no large series of these lesions have been published that allow a better characterization of the pathology and the clinical, radiologic, and surgical outcome of these lesions. The purpose of this study is to describe 23 previously unreported cardiac fibromas that have been pathologically documented, with emphasis on clinical findings, histologic features, and radiologic diagnosis.
MATERIALS AND METHODS
We retrospectively reviewed the files of the Armed Forces Institute of Pathology for cases of fibrous tumors of the heart between 1967 and 1993. Twenty-three cases of cardiac fibroma with pathologic material were identified. The diagnosis depended on the finding of a discrete, bulging mass in one or more ventricles or atria, which histologically was composed primarily of fibroblasts or collagen. Excluded from the diagnosis were tumors that contained myofibroblasts and histiocytic cells (inflammatory pseudotumors) or tumors of endocardial location with luminal growth (papillary fibroelastoma, myxoma with fibrosis).
The clinical information was based on material submitted at the time of consultation supplemented by follow-up information requested from the treating institution at the time of this study. Fourteen cases were submitted as part of a radiologic-pathologic correlation course; for these, detailed radiologic evaluation was included with the pathologic materials.
Sections of tumors were studied with hematoxylin-eosin stains and Movat's pentachrome stain for collagen, elastic tissue, and acid mucopolysaccharides. The degree of collagen and the cellularity were estimated blindly on an arbitrary scale of 1 to 3+.
RESULTS
Patient data
The mean age at the time of diagnosis was 13 years; the age range was 1 day to 56 years. More than one third of the patients were younger than 1 year of age at the time of diagnosis. No sex or race predominance was apparent.
Clinical findings
Symptoms related to cardiac fibroma were related primarily to heart failure, arrhythmias, sudden death, and chest pain. One patient had a persistent pericardial effusion that was at first ascribed to collagen vascular disease; one patient whose tumor obstructed the pulmonary outflow had syncope; two tumors were incidental findings. Of the latter patients, one was being evaluated for multiple mandibular cysts and was noted to have multiple digits of the hands and an enlarged cardiac silhouette. A diagnosis of the Gorlin syndrome was subsequently made. The other patient with an incidental tumor was first investigated after a murmur was auscultated during a routine physical examination.
Little correlation exists between the type of initial symptom and the location of the tumor in the heart. Arrhythmias were common in patients with tumors in the interventricular septum, right ventricle, and left ventricle. Patients with heart failure had large, bulky tumors in either ventricle that were difficult to excise. Although tumors in the ventricular septum were usually symptomatic, a calcified mass in the ventricular septum was first noted during coronary artery bypass grafting in a 56-year-old man.
Gross findings
The site of cardiac fibroma was the ventricle or interventricular septum in 21 cases and the right atrium in two cases. The left ventricle was more frequently involved than the right (12:8); in three cases the tumor was predominantly in the interventricular septum. The gross appearance of the tumors was typically described as fibrous, white, and whorled (Fig. 1). An exact size was available for resected or autopsy specimens. The mean size was 5 cm, with a range of 2.5 to 8 cm. The tumors were described at operation or autopsy as circumscribed in 10 cases and infiltrative in 13.
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Table I, mean age 12 years), simple surgical excision was done; in four of these patients (mean age 7 years), the resection was considered subtotal by the surgeon. In five patients (Table II), complex operations were performed, including patching of the myocardial defect, valve replacement, or coronary artery bypass grafting. The mean age of these patients was 22 years. Only two patients died as a result of surgical complications; one was a 1-day-old infant with a large tumor, and the other was a 28-year-old patient in whom valve replacement and coronary artery bypass grafting were necessary. The other patients were well at the time of most recent follow-up study (Table II)
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(Table III). Five of these patients were younger than 2 years old (mean age 9 months); in these cases, resection was considered impossible because of the extent of tumor at the time of the operation. Five of these six patients died as a result of surgical complications; one remained well 1 month after the operation. The sixth patient in this group was a 19-year-old man with a circumscribed tumor; in this patient, it was elected to obtain a biopsy specimen of the tumor for diagnostic purposes only and observe the patient without further surgical intervention.
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(Table IV).
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(Table V).
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In 11 of 12 echocardiograms, the mass was identified. Two masses were described as echogenic and three were of complex echogenicity, one with the suggestion of central cystic areas.
Two unenhanced chest computed tomograms (CTs) demonstrated the cardiac mass and both identified focal central calcifications in the lesions (Fig. 2). Three intravenous contrast-enhanced chest CT scans were available for review. Homogeneous enhancement of the tumor was seen in one case and heterogeneous enhancement in one case; in the third case the mass was not visualized prospectively. The reports of three additional CT studies (imaging technique not specified) described homogeneous masses in two and a nondiagnostic report in one. Two chest CT scans demonstrated pericardial effusions.
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In four patients, right and left heart catheterization was performed. Displacement of the left anterior descending coronary artery was noted in two, and ventriculograms demonstrated a mass in the ventricle in each case.
Thallium scans, performed on two patients, demonstrated photopenic defects at the tumor site.
Histologic findings
The histologic appearance was essentially as previously described. 9 However, the amount of collagen increased with age, whereas the degree of cellularity decreased with age (Table VI). Occasional mitoses were present in three cellular tumors of patients less than 5 months of age, and occasional perivascular aggregates of lymphocytes and histiocytes were observed in two cellular tumors. Sparse chronic inflammation was present primarily at the junction of the tumor and uninvolved myocardium in seven cases. Prominent elastic fibers were present in only four cases. Calcification was present in tumors of patients of all ages but was somewhat more common in older individuals. A focal myxoid background was present in two patients.
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Cardiac fibroma is considered a tumor of infancy and childhood. 9 Although a majority of patients in this study were younger than 10 years of age, a substantial proportion (10/23) were 10 years of age or older. The diagnosis of cardiac fibroma should be considered, therefore, even in adolescents and adults with mass lesions in the heart.
The propensity for cardiac fibroma to cause arrhythmias is becoming increasingly evident. 8 Nine patients in this report had documented arrhythmias, had symptoms most likely related to arrhythmias, or died suddenly.
This report includes a patient with cardiac fibroma and Gorlin syndrome (multiple nevoid basal cell carcinomas, jaw cysts, and bifid ribs), corroborating the association that has been established between these two entities. 1 The prevalence of Gorlin syndrome in patients with cardiac fibromas may be higher than 1 in 23, because many patients were infants in whom manifestations of this syndrome may not yet have appeared. The presence of multiple neural midline defects has been described in a patient with cardiac fibroma 2; no such abnormalities were present in patients in our series.
The optimal treatment of children and adults with cardiac fibroma has not yet been determined, although reports of successful resection have been published. 3,6,7 In this series, surgery was successful in all but one patient who had severe symptoms. This patient died after a complex operation that included valve replacement and coronary revascularization. Therefore, surgery appears to be the optimal treatment in patients with symptomatic resectable tumors. The role of surgery in patients with asymptomatic tumors is less clear. 5 Cardiac fibromas can remain dormant for many years and even regress. 10 The only patient in our series who died suddenly and who was older than 3 months had concomitant severe coronary artery disease; therefore, the cause for the fatal arrhythmia may have been unrelated to the cardiac tumor. It may thus be reasonable to observe some symptom-free patients without risking cardiac surgery. Most symptom-free patients in our series, however, had a successful operation, one going on to competitive athletic pursuits in high school. The good surgical outcome of patients in our series who were older than the age of 2 years indicates that even in symptom-free patients, tumors that are amenable to resection should probably be removed, especially if optimal cardiac function is to be preserved. Because two infants in our series died suddenly without any previous symptoms, surgical treatment for cardiac fibroma is most likely indicated in this age group to avoid the development of lethal arrhythmias.
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3,11 We have demonstrated that CT has a high sensitivity for the detection of intralesional calcification. MRI is an excellent modality for demonstrating the anatomic location of these lesions and the extent of the mass, which is usually isointense to hyperintense when compared with skeletal muscle on T1-weighted spin echo images and are of homogeneous or heterogeneous signal. Because one case in this study imaged after intravenous gadolinium administration exhibited enhancement of the mass, gadolinium may be helpful in evaluating the extent of these lesions before the operation. T2-weighted MR images in cases of cardiac fibroma may further characterize the fibrous nature of these tumors, because fibrous tissue characteristically has low signal intensity in T2-weighted MRI studies.
Cardiac fibroma is part of the spectrum of soft tissue lesions that embraces soft tissue fibromas and fibromatoses. Many of the latter tumors are considered aggressive lesions with the capacity for local invasion and growth. 12 In contrast to fibromatosis, cardiac fibromas do not appear to continue to grow and proliferate after 1 or 2 years of age. The degree of fibrosis increases with age, and the degree of cellularity decreases with age. Surgical approach must be tailored to the individual case; however, the surgeon can, with some degree of certainty, advise the patient after the operation that the tumor is not likely to recur and that short-term and mid-term prognosis is excellent.
Footnotes
From the Departments of Cardiovascular Pathologya and Radiologic Pathology,b Armed Forces Institute of Pathology, Washington, D.C., and the Department of Radiology,c University of Maryland Medical System, Baltimore, Md. 
The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or reflecting the views of the Department of the Army, the Department of the Air Force, or the Department of Defense.
References
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