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Primary cardiac lipoblastoma
M K Dishop1, W N O'Connor, S Abraham
1Department of Pathology and Laboratory Medicine, University of Kentucky Medical Center, MS 117, Lexington, KY 40536, USA.
Insights
This is the first reported case of cardiac lipoblastoma, a rare benign tumor, in a child. The successful surgical removal of the mediastinal mass in a 17-month-old boy highlights a new potential location for this pediatric adipose tumor.
Area of Science:
- Pediatric Oncology
- Cardiovascular Pathology
- Surgical Oncology
Background:
- Lipoblastoma is a rare, benign pediatric tumor of adipose tissue, typically found in the extremities.
- Primary cardiac tumors are uncommon in children, with lipoblastoma being an unreported entity in this location.
Observation:
- A 17-month-old boy presented with respiratory distress and symptoms of superior vena cava syndrome due to a large mediastinal mass.
- Imaging revealed an intrapericardial mass compressing the left mainstem bronchus, right atrium, and superior vena cava.
Findings:
- Surgical exploration identified an intrapericardial soft tissue mass originating from the posterior interatrial septum.
- Histopathological examination confirmed the diagnosis of lipoblastoma, characterized by immature and mature adipocytes with lipoblasts.
Implications:
- This case expands the known anatomical distribution of lipoblastoma to include the heart in pediatric patients.
- Complete surgical resection appears to be an effective treatment, with no evidence of recurrence at two months post-operation.
Abstract:
Lipoblastoma is a benign adipose tumor in children that has been described in various anatomic locations, most commonly the extremities. We describe the case of a 17-month-old boy diagnosed with cardiac lipoblastoma, a previously unreported primary cardiac tumor in children. Our patient presented with symptoms of coughing, wheezing, and hoarseness and was found to have a large mediastinal mass, which narrowed the left mainstem bronchus and compressed the right atrium and superior vena cava, causing superior vena cava syndrome. Surgical exploration revealed an intrapericardial soft tissue mass arising from the area of the posterior interatrial septum. Grossly, the resected mass was lobulated, pale yellow, and fatty with focal areas of gray myxoid tissue. Microscopically, the tumor consisted of both immature and mature adipocytes, with focal vascular myxoid areas containing lipoblasts, diagnostic of lipoblastoma. Two months after surgery, the patient was in good health without evidence of recurrence.