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Hamartomas of the chest wall in infants
R Dounies1, W J Chwals, K P Lally
1Department of Pediatric Surgery, Children's Hospital of Los Angeles, University of Southern California.
Insights
Chest wall hamartomas are rare infantile lesions presenting as hard masses that can cause breathing issues. While typically benign, one case showed malignant transformation, and surgical removal may lead to scoliosis.
Area of Science:
- Pediatric Oncology
- Thoracic Surgery
- Developmental Biology
Background:
- Chest wall hamartomas are uncommon congenital tumors in infants.
- These lesions are characterized by distinct clinical, radiographic, and pathologic features.
Observation:
- Four cases of chest wall hamartomas at Children's Hospital of Los Angeles were reviewed.
- Infantile chest wall hamartomas present as hard, immobile, antenatal masses, potentially causing respiratory insufficiency.
- Radiographically, they appear as extrapleural masses originating from the ribs.
Findings:
- Histologically, these hamartomas show hypercellularity with disorganized mesenchymal tissues native to the chest wall.
- Growth is often rapid but typically self-limiting, with most cases being benign.
- One case demonstrated malignant transformation, highlighting a rare but significant risk.
Implications:
- Complete surgical resection (en bloc) is curative for chest wall hamartomas.
- Large chest wall defects post-resection frequently result in the development of scoliosis.
- Understanding these rare tumors is crucial for appropriate diagnosis and management in pediatric patients.
Abstract:
Chest wall hamartomas in infancy are rare lesions with distinct clinical, radiologic, and pathologic characteristics. Four cases treated at Children's Hospital of Los Angeles are presented and previously reported cases are reviewed. Chest wall hamartomas arise antenatally and present as hard, immobile masses, which may cause respiratory insufficiency. An extrapleural mass arising from the ribs can be seen radiographically. Histologically, these lesions are hypercellular and consist of a disorganized array of mesenchymal tissues endogenous to the chest wall. Rapid growth may occur, but usually is self-limited. Chest wall hamartomas are usually benign. This series includes the malignant transformation of one of these lesions. En bloc resection is curative, but the large residual chest wall defect frequently results in scoliosis.
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