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Ann Thorac Surg 1996;62:559-564
© 1996 The Society of Thoracic Surgeons
Division of Cardiovascular Surgery, Texas Heart Institute and Texas Children's Hospital, Houston, Texas
Accepted for publication April 13, 1996.
| Abstract |
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Methods. Between January 1961 and January 1996, 40 infants and children (mean age, 3.3 years; range, 2 days to 17 years; 65% female) were diagnosed at our institution with primary cardiac tumors. Of these tumors, 37 (92%) were benign and 3 (8%) were malignant. Tumors were resected in 38 patients (95%). In 2 patients (5%), biopsy confirmed rhabdomyoma; however, presenting symptoms spontaneously resolved, so these patients did not undergo tumor resection. Follow-up echocardiographic studies showed a diminishing tumor mass in each of these patients.
Results. Immediate, symptom-free status was achieved in all patients. There were two early deaths, for an operative mortality of 5%. Three late postoperative deaths (7.5%) occurred as follows: 1 patient with a myocardial hamartoma died at 3 months of congestive heart failure. Another patient with a recurrent rhabdomyosarcoma died at 6 months, and a third patient with a recurrent fibrosarcoma died at 28 months. Long-term follow-up was available for 34 survivors (97% complete) and totaled 240.2 patient-years (mean, 7.1 years/patient). All remaining survivors were without evidence of presenting symptoms and tumor recurrence or progression.
Conclusions. The data suggest that an aggressive operative approach is warranted for benign symptomatic and malignant tumors. This aggressive approach has resulted in extended symptom-free status in patients with benign lesions, and significant palliation and longer survival in patients with malignant lesions, with acceptably low operative risk.
| Introduction |
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Primary cardiac neoplasms occur infrequently both in adult and pediatric age groups. In the general population, cardiac tumors have been noted to have an incidence of between 0.002% and 0.3%, based on autopsy series [4]. In infants and children, cardiac tumors were reported at a frequency of 0.027% in a study comprising more than 11,000 postmortem examinations [5]. Benign lesions usually predominate, making up more than 90% of all pediatric tumors [6].
The literature contains few large or long-term series involving infants and children with primary cardiac tumors. This report summarizes our 35-year experience with such lesions. It examines the clinicopathologic spectrum of such tumors and details operative indications, immediate outcomes, and long-term postoperative results.
| Material and Methods |
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The series included 26 girls and 14 boys, whose average age at operation or biopsy was 3.3 years (range, 2 days to 17 years). In 38 patients (95%), the tumor was symptomatic (Table 1
), causing the family to seek medical help. In the remaining 2 patients (5%), the tumor was asymptomatic and was discovered incidentally, either on a chest roentgenogram (1 patient) or during operative treatment of congenital cardiac abnormalities (1 patient). Except in the patients involving myocardial hamartomas and one of the two lesions found incidentally (see below), the diagnosis was established preoperatively by means of echocardiography or cineangiography, or both.
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Benign Tumors
MYOCARDIAL HAMARTOMAS.
Myocardial hamartomas, which were the most frequently observed neoplasms [7, 8], were identified in 16 patients (40%), including 10 girls and 6 boys, aged 3 to 20 months (mean, 1.1 years) (Table 3
). The increased frequency of these tumors in our series may reflect referral patterns to our center, which is known in our geographic area for treatment of these types of lesions. Upon admission, 15 of the patients with hamartomas had symptoms of cardiovascular collapse (cardiac arrest/hypotension); 1 patient was hemodynamically stable. All 16 patients with hamartomas had ventricular tachycardia unresponsive to medical therapy (Table 1
).
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RHABDOMYOMAS.
Rhabdomyoma, the second most frequently observed tumor, was present in 9 patients (22.5%), including 7 girls and 2 boys, aged 2 days to 19 months (mean, 0.5 year) (Table 3
). These patients had arrhythmias (5 patients) and/or heart failure (6 patients; five involving intracavitary compromise of outflow and one involving valve obstruction) (Table 1
).
Two patients with refractory ventricular tachycardia underwent intraoperative mapping, tumor excision, and circumferential cryoablation of the excision site. Five other patients underwent tumor excision after echocardiographic and angiographic evaluations showed intracavitary filling defects consistent with a tumor.
Two patients presented with a nonsustained arrhythmia or congestive heart failure, which prompted a full diagnostic evaluation. Diagnosis of rhabdomyoma was confirmed by an endomyocardial biopsy specimen. Because symptoms resolved, these patients did not undergo resection, but instead were monitored by serial clinical and echocardiographic examinations.
FIBROMAS.
Fibromas were diagnosed in 8 patients (20%), including 5 girls and 3 boys, aged 7 months to 17 years (mean, 5 years) (Table 3
). These patients had a cardiac murmur (4 patients) and/or congestive heart failure (5 patients; 4 involving compromise of the outflow tract and 1 involving tricuspid valve obstruction) (Table 1
). All patients underwent echocardiography and angiography, followed by tumor excision.
OTHER BENIGN TUMORS.
Other benign tumors were identified in 4 patients (10%) (Table 3
). These lesions included myxomas in 2 girls, aged 13 and 17 years: one had systemic symptoms and the other had symptoms of congestive heart failure. Both myxomas were resected. A 9-year-old girl underwent resection of a hemangioma that compromised the right ventricular outflow tract, causing atrial arrhythmias. A 5-year-old boy presented with a cardiac murmur and intermittent ventricular tachycardia. There was angiographic evidence of a mitral valve filling defect, which caused us to suspect a mitral valve cyst. This child required primary valve repair, as the leaflet was resected to remove the cyst.
Malignant Tumors
Sarcomas were identified in 3 patients (7.5%), 2 boys and 1 girl, aged 3 months to 14.0 years (mean, 8.1 years) (Table 4
). Presenting symptoms included arrhythmia with failure to thrive, a cardiac murmur, and right ventricular outflow tract obstruction (1 patient each) (Table 1
). After the diagnosis was confirmed with echocardiography and angiography, the tumors were excised.
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| Results |
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One patient underwent pacemaker insertion for a complete heart block postoperatively. At hospital discharge, 2 patients required continued antiarrhythmic medication for frequent premature ventricular contractions. The remaining patients were asymptomatic.
Long-term follow-up information was available for 15 patients; the follow-up period ranged from 3.2 to 15.5 years (mean, 9.1 years). All 15 patients underwent echocardiographic and electrocardiographic studies. One patient died of congestive heart failure 3 years postoperatively. The 2 patients discharged on antiarrhythmic medication were successfully weaned from that medication within 6 months of operation. No patient had evidence of recurrent ventricular arrhythmia. At the most recent follow-up visit, 2 patients were being treated for mild congestive heart failure.
RHABDOMYOMAS.
Of the 9 patients with rhabdomyomas, there was one death: a 4-month-old infant, who was admitted with refractory ventricular tachycardia, died 9 hours postoperatively of congestive heart failure. In this patient, a mitral papillary muscle was injured during excision of the tumor. Although the diseased tissue was successfully ablated, the patient's remaining myocardial mass was insufficient to overcome both the myocardial loss and the papillary muscle injury.
In the other 8 patients, excision of multiple tumors (2 patients) or of single tumors (4 patients) led to successful resolution of the symptoms without postoperative complications. Long-term follow-up information was available for all eight survivors. The observation period ranged from 10 months to 13.3 years (mean, 4.6 years). At the most recent follow-up visit, all patients were without recurrent symptoms, late operative sequelae, or cardiac dysfunction. In the 2 patients in whom the diagnosis had been confirmed by an endomyocardial biopsy after temporary symptoms resolved, clinical follow-up examinations showed no recurrence of symptoms, and serial echocardiographic examinations showed a diminishing tumor mass in each patient.
FIBROMAS.
Of the 8 patients with fibromas, one died. This 17-year-old girl, who presented with congestive heart failure, had a massive tumor that had invaded most of the left ventricular cavity, left ventricular free wall and cardiac septum, and that extended circumferentially around the pulmonary artery. The patient underwent tumor resection and coronary artery bypass grafting, using a reversed saphenous vein from the aorta to the left anterior descending coronary artery, but could not be weaned from cardiopulmonary bypass. The remaining 7 patients underwent complete (n = 4) or partial (n = 3) tumor excision and were symptom-free at hospital discharge. At the end of a follow-up period ranging from 7 months to 29.5 years (mean, 11.6 years), all patients were without evidence of cardiac dysfunction and tumor recurrence or progression.
OTHER BENIGN TUMORS.
The 4 patients with other benign tumors (two myxomas, one left ventricular hemangioma, and one mitral valve cyst) underwent uneventful tumor excision or successful primary mitral valve repair after leaflet resection. Postoperatively, these patients were symptom-free, without medication, and without evidence of cardiac dysfunction at 1 month (myxoma), 1.3 years (myxoma), 7.3 years (cyst), and 8.3 years (hemangioma).
MALIGNANT TUMORS.
All 3 patients with malignant tumors underwent operative resection. In 1 patient involving a rhabdomyosarcoma of the left atrium, postoperative histologic evaluation revealed microscopic evidence of tumor at the resection margins. The other two lesions (an undifferentiated sarcoma and a fibrosarcoma) were considered fully resected. Postoperative adjuvant chemotherapy was administered for the rhabdomyosarcoma and the fibrosarcoma.
There were two late deaths. The 14-year-old boy with the rhabdomyosarcoma died 6 months postoperatively. The 10-year-old boy with the fibrosarcoma remained symptom-free until the end of the second postoperative year, when he was found to have a recurrent tumor involving the pericardium and one lung. He underwent successful tumor debulking but died 4 months later.
The 3-month-old girl who underwent successful resection of an undifferentiated right ventricular sarcoma was alive and well 10.3 years later, without evidence of tumor recurrence or cardiac dysfunction.
| Comment |
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Even for "benign" tumors, location or size appears to influence outcome. Presenting symptoms in many patients were indeed life-threatening and warranted aggressive therapy. This aggressive therapy was generally successful, as noted both by immediate and long-term, symptom-free status.
In the subgroup of patients with myocardial hamartomas, one late death occurred secondary to congestive heart failure at 3 years, and 2 other patients are being treated for mild congestive heart failure (one of whom has required hospitalization). Although cardiomyopathy may have accounted for the congestive heart failure, it is also possible that after resection, a critical myocardial mass was approached, one that diminished short- and long-term myocardial reserve and affected both short- and long-term cardiac function.
The origin of myocardial hamartomas is controversial. These nodules have been described alternatively as histiocytoid cardiomyopathy [9], multifocal tumors of Purkinje cells [10, 11], and myocardial hamartomas [12]. That they are tumors is suggested by their morphologic, histologic, and clinical features. These lesions exhibit a nodular or tumorlike growth pattern manifested by clusters of myocytes with primitive morphologic characteristics. The individual cells have large numbers of mitochondria similar to oncocytes found in other organs including the exocrine and endocrine glands and kidneys [13]. In addition, clinical symptoms are eliminated after the mass is excised, which makes the diagnosis of cardiomyopathy less likely [12].
The management of rhabdomyoma tumors is influenced by the presence or absence of symptoms [14, 15]. Diagnosis can be established by analysis of an endomyocardial biopsy specimen. Regression of these tumors has been reported by Farooki and associates [16] and confirmed by the results in 2 patients in our report. Thus, in the absence of symptoms, surgical intervention may be deferred if patients undergo clinical and echocardiographic follow-up studies. Untreated, symptomatic rhabdomyoma, however, has been associated with mortality rates of 60% to 80% in the pediatric population [15, 17, 18].
This report and others [19, 20] have described fibromas that have grown to massive size. In 3 of the 7 surviving patients in our series, the fibromas could only be resected partially. Although the prognosis for a patient with an incompletely resected tumor is uncertain, extended follow-up (at 7 months, 10.0 years, and 14.6 years) in our 3 patients revealed no evidence of recurrent symptoms, cardiac dysfunction, or tumor enlargement. These results confirm results of a previous study [21] and suggest that, although complete excision of tumor remains the goal, significant debulking of the fibroma may provide effective, long-term symptom-free survival. Malignant transformation of fibroma has not been reported.
Although myxomas are the most common cardiac tumors in adults, they are uncommon in the pediatric population. McAllister and Fenoglio [6] report an incidence of 15.5% in children. We described two myxomas in this series, both of which occurred in older children. We favor a biatrial surgical approach to remove myxomas, which permits inspection of all four chambers, minimizes the need to manipulate the tumor, and increases the probability of complete excision. These measures decrease the potential for recurrence [22].
Malignant sarcomas are uncommon but extremely aggressive, and multiple reports document an expected survival of less than 1 year after diagnosis [23, 24]. However, 1 of our patients is alive 10.3 years after resection, and another survived 2.3 years. Although few conclusions can be drawn from so few cases, the outcome of these 2 patients appears to support early, aggressive operative therapy for the best chance of cure or palliation [25]. Doxorubicin-based postoperative adjuvant therapy has been used on the basis of its effectiveness in treating soft tissue sarcomas in other locations [26, 27]. Nevertheless, few investigators [27, 28] have found chemotherapy or radiation to be efficacious in treating primary cardiac sarcomas.
Transplantation has also been used to treat locally aggressive malignant [29] and unresectable benign cardiac tumors [30]. Jamieson and associates [30] performed a successful orthotopic cardiac transplantation in a 17-year-old girl with a ventricular arrhythmia secondary to an unresectable cardiac fibroma. Eighteen months later, the patient was alive and symptom-free. At this institution, Cooley and associates [31] performed cardiac explantation and autotransplantation in an adult with a large pheochromocytoma. We also have performed an orthotopic heart transplant in an adult with recurrent left ventricular histiosarcoma 1 month after resection of the primary tumor. Pulmonary metastatic disease developed 10 months later, which led to the patient's death 2.9 years after transplantation. The ultimate role of cardiac transplantation in treating malignant neoplastic disease in infants and children remains to be established. An obvious constraint is the scarcity of donor organs in the pediatric population.
In conclusion, our data suggest that operative resection results in extended, symptom-free survival for benign cardiac tumors, and significant palliation and longer survival for malignant lesions, with acceptably low operative morbidity and mortality. Thus, we believe that patients who are diagnosed with cardiac tumors should be considered for early surgical treatment.
| Acknowledgments |
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| Footnotes |
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| References |
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