Key result
Chemotherapy and investigational immunotherapy improved cardiac ejection fraction from 8% to 26%, but the patient ultimately died from progressive neuroblastoma and congestive heart failure.
Case Report (n=1)
This case highlights a rare presentation of refractory dilated cardiomyopathy with endocardial fibroelastosis associated with metastatic neuroblastoma in a pediatric patient.
Transient LVEF gain in this case does not guide practice; leaves open immunotherapy investigation in neuroblastoma cardiomyopathy.
A 2-year-old African American male presented with heart failure and an abdominal mass. Computerized tomography (CT) scan revealed a 7 cm adrenal lesion, confirmed as poorly differentiated neuroblastoma (NB). CT and meta-iodobenzoguanidine (MIBG) scans identified multiple metastases, but cardiac MIBG imaging was absent. Cardiac ejection fraction (EF) was 8% with 7% shortening fraction. The patient underwent six cycles of chemotherapy and investigational immunotherapy. Cardiac function improved to 26% EF. However, the tumor proved unresponsive to treatment. The patient died from stage IV congestive heart failure (CHF) and progressive NB. Autopsy confirmed dilated cardiomyopathy with endocardial fibroelastosis.
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Carlson et al. (2010) conducted a case report in Refractory dilated cardiomyopathy associated with metastatic neuroblastoma (n=1). Chemotherapy and investigational immunotherapy was evaluated. Chemotherapy and investigational immunotherapy improved cardiac ejection fraction from 8% to 26%, but the patient ultimately died from progressive neuroblastoma and congestive heart failure.
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