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Primary cardiac tumors in infants and children: immediate and long-term operative results
T J Takach1, G J Reul, D A Ott
1Division of Cardiovascular Surgery, Texas Heart Institute, Houston 77225, USA.
Insights
Aggressive surgery for primary cardiac tumors in children offers excellent outcomes. This 35-year study shows extended symptom-free survival for benign tumors and improved survival for malignant ones.
Area of Science:
- Pediatric Cardiology
- Pediatric Oncology
- Cardiac Surgery
Background:
- Primary cardiac tumors in infants and children are rare.
- Few large or long-term studies exist for pediatric cardiac tumors.
- This study reviews 35 years of experience with these rare lesions.
Purpose of the Study:
- To summarize a 35-year institutional experience with primary cardiac tumors in pediatric patients.
- To evaluate the outcomes of surgical intervention for these tumors.
- To assess the long-term prognosis for children diagnosed with cardiac tumors.
Main Methods:
- A retrospective review of 40 infants and children diagnosed with primary cardiac tumors between 1961 and 1996.
- Tumor resection was performed in 38 patients (95%); 2 patients with rhabdomyoma were managed conservatively.
- Follow-up included echocardiography and clinical assessment.
Main Results:
- Of 40 tumors, 37 (92%) were benign and 3 (8%) were malignant.
- Operative mortality was 5% (2 early deaths).
- Late mortality occurred in 3 patients (7.5%) due to tumor recurrence or complications; long-term follow-up in survivors showed no evidence of recurrence or progression.
Conclusions:
- An aggressive surgical approach is recommended for symptomatic benign and all malignant primary cardiac tumors in children.
- This strategy leads to extended symptom-free status for benign tumors.
- It offers significant palliation and improved survival for malignant tumors with acceptable operative risk.
Background:
The literature contains few large or long-term series involving infants and children with primary cardiac tumors. This article summarizes our 35-year experience with such lesions.
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.
