Primary pediatric cardiac malignancies: the SEER experience

Abstract

Pediatric cardiac malignancies are exceedingly rare. We sought to examine demographics, presentation, and outcomes for this pathology. The SEER registry from 1973 to 2008 was queried for all patients <20 years of age with cardiac malignancies. A total of 25 pediatric patients were identified with primary cardiac malignancies, with age-adjusted incidence of 0.00686 per 100,000 United States population. Median age at diagnosis was 10 years. The majority of patients were adolescent (n = 13, 52 %), Caucasian (n = 17, 68 %) and males (n = 14, 56 %). The most common histology was soft tissue sarcoma (n = 10, 40%), followed by non-Hodgkin lymphoma and teratoma (both n = 3, 12 %). Six patients presented with distant disease. More than half of patients (n = 16, 64 %) underwent surgical resection, while four patients (16 %) underwent radiation. The mean survival time for the cohort was 47 ± 67 months, with 14 (56 %) patients dying over the study period. Lymphomas had significantly longer survival than other malignancies (108 ± 66 vs. 36 ± 66, p = 0.03), while lack of surgical treatment was associated with worse survival (p = 0.016). Primary malignant cardiac tumors are exceedingly rare in pediatric patients. They are most commonly soft tissue sarcomas and lymphomas demonstrated longer survival.