Chondromesenchymal Hamartoma of Chest Wall
Synonyms: Mesenchymal hamartoma of chest wall
Rare rib hamartoma in infants causing respiratory distress
Quick Facts
Behaviour
Benign
Category
Bone
Synonyms
Mesenchymal hamartoma of chest wall
Category
Bone
Behaviour
Benign
Gender
M=F
Tissue of Origin
Cartilage
Epidemiology
- Rare benign hamartomatous rib lesion
- Infants and young children
Clinical Features
- Chest wall mass in infant
- Respiratory compromise if large
- Rapidly growing but benign
Location
- Ribs
- Chest wall
Imaging
Plain radiograph: Expansile rib lesion with cartilaginous and osseous matrix
Pathology
- Hamartomatous mixture of cartilage, bone, and fibrous tissue
- Cystic spaces with blood
- Aneurysmal bone cyst-like areas
Genetics
CTNNB1 mutations in some
Treatment
Surgical excision curative if symptomatic
Prognosis
- Excellent - recurrence after complete excision is rare
- No malignant potential
Key Points
- Rare rib hamartoma in infants causing respiratory distress
- Mixture of cartilage, bone, and cystic components
- Surgical excision curative
Workup - Blood Tests
No blood tests required
Workup - Local Imaging
- Plain radiograph
- CT or MRI
Workup - Biopsy
Excisional biopsy
Workup - Staging
No staging required
Medical disclaimer
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