Primary intraosseous haemangiomas are haemangiomas most frequently seen in the vertebrae or skull, and come in four histological varieties 1-2.
Intraosseous haemangiomas are common, with vertebral lesions seen in 10-15% of the adult population. They are more commonly encountered in men (2:1 M:F ratio) and typically seen in the 4th-5th decade of life.
These tumors are slow growing and are generally asymptomatic unless they cause mass effect on sensitive structures. Occasionally they may present as a swelling or a palpable mass, especially in the skull. When large and strategically located they may present as a pathological fracture.
If they are high flow lesions then shunt related symptoms may also be present.
Primary intraosseous haemangiomas are slow growing vascular neoplasms, usually located in the medullary cavity. They are classified as benign, but rarely may be locally aggressive.
Histological sub types
Intraosseous haemangiomas come in four histologic types:
- intraosseous cavernous haemangioma
- intraosseous capillary haemangioma
- intraosseous arteriovenous haemangioma (may represent congenital arteriovenous malformations) 2
- intraosseous venous haemangioma
Histologically demonstrates hamartomatous vascular tissue within endothelium, but may also contain fat, smooth muscle, fibrous tissue, and thrombus.
It is important to note that it is difficult to distinguish between the various types (except for those with a large arterial component).
Location specific sub types
Plain radiographs are usually first line and may be sufficient in vertbera or calvarial lesions. Findings include:
- prominent trabecular pattern
- sclerotic vertebra with vertical trabeculae: Corduroy sign
- lytic calvarial lesions with spoke-wheel appearance
- irregular and lytic in long bones, with a honeycomb appearance
Usually as an incidental finding, especially in the vertebrae.
Better visualization of thickened vertical trabeculation (polka-dot appearance)
Signal intensity is somewhat variable, depending largely on the amount of fat content.
- high is more common (fat rich)
- intermediate to low signal intensity is seen in fat poor haemangiomas
- T2: high
- T1 C+ (Gd): enhancement is often present
MRI is the ideal modality to demonstrates many mass-effect complications, such as neural impingement and extraosseous extension.
Nuclear medicine bone scan
Usually normal but may show increased or decreased uptake.
Treatment and prognosis
Treatment is reserved for symptomatic lesions, and a number of modalities exists:
- radiation therapy
- embolisation to reduce intraoperative blood loss
- surgical resection, especially if complicated by spinal cord compression
- intralesional ethanol injection
The differential diagnosis for bone tumours is dependent on the age of the patient, with a very different set of differentials for the paediatric patient.
- bone-forming tumours
- enchondromatosis (Ollier disease)
- Maffucci syndrome
- chondromyxoid fibroma
- fibrous bone lesions
- bone marrow tumours
- other bone tumours or tumour-like lesions
- simple bone cyst
- aneurysmal bone cyst
- benign fibrous histiocytoma
- haemophilic pseudotumour
- giant cell tumour of bone
- primary intraosseous haemangioma
- intraosseous lipoma
- musculoskeletal angiosarcoma
- Gorham massive osteolysis
- impending fracture risk
- 1. Sweet C, Silbergleit R, Mehta B. Primary intraosseous hemangioma of the orbit: CT and MR appearance. AJNR Am J Neuroradiol. 1997;18 (2): 379-81. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 2. Murphey MD, Fairbairn KJ, Parman LM et-al. From the archives of the AFIP. Musculoskeletal angiomatous lesions: radiologic-pathologic correlation. Radiographics. 1995;15 (4): 893-917. Radiographics (abstract) - Pubmed citation
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Primary osseous haemangioma||✓|
|Primary osseous hemangioma||✗|
|Primary intraosseous hemangioma||✗|
|Primary osseous haemangiomas||✗|
|Primary osseous hemangiomas||✗|