Hemangioma of bone is a benign vascular tumor most common in vertebral bodies (28%) and skull (20%), typically incidental in adults 40-60 years with 2:1 female predominance. Pathognomonic MRI shows bright T1 AND bright T2 signal (fat content). Radiographs show corduroy pattern in spine, sunburst in skull. 95%+ are asymptomatic requiring observation only. Symptomatic lesions may require radiotherapy, vertebroplasty, or surgery with preoperative embolization. Malignant transformation does NOT occur.

Classic Presentation:

• 50-year-old woman with incidental vertebral lesion on lumbar spine MRI
• Asymptomatic, discovered during evaluation for mechanical back pain
• MRI shows bright T1 and T2 signal in L3 vertebral body
• "Corduroy" appearance on lateral radiograph

Diagnosis:

• Plain X-ray: Vertical striations (corduroy) in vertebrae, sunburst (skull)
• MRI: High T1 and T2 signal (pathognomonic), no enhancement pattern needed
• CT: Thickened vertical or radiating trabeculae, "polka-dot" axial view
• Biopsy: Only if atypical features (soft tissue mass, cortical destruction)

Management Algorithm:

1. Asymptomatic (95%): Observation, no follow-up imaging needed
2. Symptomatic Pain: Radiotherapy (20-40 Gy), vertebroplasty/kyphoplasty
3. Neurological Deficit: Decompression + stabilization, preoperative embolization
4. Pathological Fracture: Surgical fixation + curettage, bone grafting
5. Aggressive Features: Biopsy to exclude angiosarcoma, consider en bloc resection

Demographics

Incidence:

• Most common benign vascular tumor of bone
• Autopsy studies: 10-12% of all spines have hemangiomas
• Clinical significance: Less than 1% become symptomatic
• True prevalence unknown (many never detected)

Age Distribution:

• Peak Detection: 40-60 years (mean 50 years)
• Can occur at any age after skeletal maturity
• Rare in children (less than 5% of cases)
• Likely congenital but clinically apparent in adulthood

Gender:

• Female to male ratio: 2:1 overall
• Vertebral hemangiomas: Female to male 3:1
• Skull hemangiomas: No significant gender predilection

Anatomical Location:

• Vertebral Bodies: 28-30% (thoracic greater than lumbar greater than cervical)
• Skull/Calvarium: 20% (frontal and parietal bones)
• Facial Bones: 10% (maxilla, mandible, zygoma)
• Long Bones: 5% (femur, humerus, tibia)
• Other: Ribs, pelvis, scapula (rare)

Vertebral Distribution:

• Thoracic spine: 55-60% (T4-T9 most common)
• Lumbar spine: 30-35% (L1-L3)
• Cervical spine: 5-10%
• Sacrum: Less than 5%
• Multiple levels: 30-40% of vertebral cases

Bone hemangioma is a benign vascular tumour most commonly affecting vertebral bodies (28%) and skull (20%), typically discovered incidentally in adults aged 40-60 with a 2:1 female predominance. Radiographic hallmarks include "corduroy" appearance (vertical striations) in the spine and "polka-dot" pattern on axial CT. MRI shows bright T1 and T2 signal due to fat content - this is pathognomonic and diagnostic. Approximately 95% are asymptomatic and require only observation. Symptomatic lesions may be treated with radiotherapy, vertebroplasty, or surgical decompression. Malignant transformation does NOT occur; if aggressive features are present, consider biopsy to exclude angiosarcoma.

Pathogenesis

Etiology:

• Hamartomatous: Likely developmental malformation, not true neoplasm
• Congenital: Present from birth, grows slowly over decades
• Estrogen Influence: Female predominance suggests hormonal role
• Pregnancy Association: May enlarge during pregnancy (estrogen effect)

Growth Patterns:

• Capillary Type: Small thin-walled vessels, less aggressive
• Cavernous Type: Large blood-filled spaces, more common, more symptomatic
• Mixed Type: Combination of capillary and cavernous elements

Mechanisms of Symptom Development:

1. Expansion: Slow growth causes vertebral body expansion and pain
2. Fracture: Pathological fracture from vertebral body weakening
3. Compression: Epidural extension causing spinal cord or nerve root compression
4. Hemorrhage: Acute bleeding into lesion (very rare)
5. Pregnancy: Rapid enlargement during pregnancy

Aggressive vs Typical Hemangioma:

Asymptomatic Hemangioma (95%)

Discovery:

• Incidental finding on spine imaging (MRI, CT) for unrelated complaints
• No local symptoms or neurological findings
• No intervention required
• Natural history: Stable throughout life

Imaging Characteristics:

• Confined to vertebral body
• No posterior element involvement
• No soft tissue component
• Bright T1 and T2 signal on MRI

Symptomatic Hemangioma (Less than 1%)

Pain Syndromes:

• Localized Back Pain: Mechanical, worse with activity
• Nocturnal Pain: May be present with active expansion
• Radicular Pain: Nerve root compression from epidural extension
• Acute Pain: Pathological fracture (sudden onset)

Neurological Manifestations:

• Radiculopathy: Dermatomal pain, sensory changes, weakness
• Myelopathy: Spastic gait, hyperreflexia, bowel/bladder dysfunction
• Cauda Equina Syndrome: Rare, lumbar lesions with massive epidural extension
• Acute Paraplegia: Extremely rare, acute hemorrhage or fracture

Pregnancy-Related Symptoms:

• Rapid enlargement during pregnancy (estrogen-driven)
• New-onset back pain or neurological symptoms
• Usually regresses postpartum
• May require urgent intervention if cord compression

Skull/Calvarial Hemangiomas:

• Asymptomatic: Palpable skull mass, cosmetic concern
• Dural Involvement: Headache, seizures (rare)
• Erosion: Through inner or outer table
• Bleeding: Profuse hemorrhage if traumatized or during surgery

Long Bone Hemangiomas:

• Localized pain and swelling
• Pathological fracture (rare)
• Palpable mass in superficial bones

Physical Examination

Inspection:

• Visible deformity (skull lesions)
• Kyphotic deformity (vertebral fracture)
• Normal overlying skin

Palpation:

• Tender to percussion (vertebral)
• Palpable bony prominence (skull)
• Soft tissue mass (aggressive lesions)

Neurological Examination:

• Usually normal (asymptomatic cases)
• Radicular findings (dermatomal sensory loss, weakness)
• Upper motor neuron signs (myelopathy)
• Bowel/bladder dysfunction (cauda equina)

Clinical Pearls:

1. Vast majority (95-99%) are asymptomatic incidental findings requiring NO treatment
2. Symptomatic hemangiomas usually have posterior element involvement or epidural extension
3. Bright T1 AND T2 signal on MRI is pathognomonic (no other lesion has this)
4. Pregnancy can cause rapid enlargement - counsel female patients of childbearing age
5. Profuse bleeding risk during surgery - always consider preoperative embolization

Plain Radiography

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Vertebral Hemangiomas:

• Lateral View: "Corduroy" or "jail-bar" appearance (vertical striations)
  • Thickened vertical trabeculae
  • Loss of horizontal trabeculae
  • Coarse trabecular pattern
• AP View: Vertical striations less apparent
• Vertebral Body: Expansion, increased height (rare)
• Cortex: Usually intact (aggressive lesions may destroy cortex)

Skull Hemangiomas:

• Sunburst: Radiating trabeculae from center (classic)
• Honeycomb: Multiple small lucencies
• Soap-bubble: Expansile lytic lesion with septa
• Well-defined: Sclerotic margins typically

Long Bones:

• Lytic lesion with trabeculated appearance
• "Soap-bubble" or "honeycomb" pattern
• Cortical thinning or expansion

Computed Tomography (CT)

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Vertebral Hemangiomas:

• Axial View: "Polka-dot" or "corduroy" sign
  • Thickened vertical trabeculae seen as dots
  • Low-attenuation marrow between trabeculae
• Sagittal/Coronal: Vertical striations evident
• Cortical Integrity: Assess for destruction
• Epidural Extension: Soft tissue component into spinal canal

Skull:

• Radiating trabeculations
• Diploe expansion (between inner and outer tables)
• Soft tissue mass if dural involvement

Magnetic Resonance Imaging (MRI)

Classic Features (Pathognomonic):

• T1-Weighted: BRIGHT signal (hyperintense)
  • Due to fat content between vascular spaces
  • Same intensity as subcutaneous fat
• T2-Weighted: BRIGHT signal (hyperintense)
  • Due to slow-flowing blood and edema
  • Heterogeneous hyperintensity
• STIR/Fat-Suppression: Signal drops out (confirms fat)

Aggressive Features:

• Extension into posterior elements (pedicles, lamina)
• Epidural soft tissue mass
• Spinal cord compression or displacement
• Vertebral body expansion (greater than 50% canal compromise)
• Heterogeneous signal (less fat, more vascular)

Contrast Enhancement:

• Usually not needed for diagnosis
• Enhances if performed (vascular lesion)
• Helps delineate epidural extent for surgical planning

Angiography

Indications:

• Preoperative planning for surgery
• Embolization procedure (symptomatic lesions)
• Confirm vascular nature if imaging ambiguous

Findings:

• Hypervascular lesion with intense blush
• Arterial feeders (vertebral, intercostal, or lumbar arteries)
• Arteriovenous shunting in some cases

Embolization:

• Reduce vascularity before surgery (decrease bleeding)
• Perform 24-48 hours before operation
• Agents: Polyvinyl alcohol (PVA), coils, Onyx

Histopathology

Indications for Biopsy:

• Atypical radiographic features (cortical destruction, soft tissue)
• Concern for malignancy (angiosarcoma)
• Neurological deficit with unclear diagnosis
• Young patient (hemangiomas rare in children)

Macroscopic:

• Red-purple spongy tissue
• Blood-filled spaces
• Soft consistency

Microscopic Features:

• Cavernous Type: Large thin-walled vascular channels
  • Endothelial-lined spaces
  • Separated by fibrous stroma
  • Fatty marrow between vessels
• Capillary Type: Small capillary-sized vessels
  • Densely packed
  • Less fatty stroma
• No Cellular Atypia: Bland endothelial cells
• No Mitoses: Benign proliferation

Immunohistochemistry:

• CD31: Positive (endothelial marker)
• CD34: Positive (vascular marker)
• ERG: Positive (endothelial transcription factor)
• Ki-67: Low proliferation index (less than 2%)
• Cytokeratin: Negative (excludes carcinoma)

Differential Histology:

• Angiosarcoma: Cellular atypia, mitoses, infiltrative growth
• Hemangioblastoma: CNS lesion, not bone primary
• Vascular Malformation: Similar but developmental anomaly, not tumor

Asymptomatic Hemangiomas

Observation:

• Indications: Incidental finding, no symptoms, no aggressive features
• Protocol: NO follow-up imaging required
• Counseling: Explain benign nature, no risk of malignant transformation
• Activity: No restrictions
• Prognosis: Stable lifelong, intervention rarely needed

Patient Education:

• Benign vascular tumor, not cancer
• Will not cause symptoms in vast majority
• Return if new back pain or neurological symptoms develop
• Safe to ignore for asymptomatic cases

Symptomatic Hemangiomas

Pain Management (Non-Operative):

Radiotherapy:

• Indications: Symptomatic pain without neurological deficit
• Dose: 20-40 Gy fractionated (10-20 fractions)
• Mechanism: Sclerosis of vascular channels, reduces tumor volume
• Success Rate: 80-90% pain relief
• Onset: Gradual over weeks to months
• Side Effects: Radiation myelopathy (rare), skin changes, vertebral compression fracture
• Contraindications: Neurological deficit (surgery preferred), young age

Vertebroplasty/Kyphoplasty:

• Indications: Painful vertebral hemangioma with partial collapse
• Technique: Percutaneous cement injection into vertebral body
• Outcomes: Immediate pain relief in 80-90%
• Complications: Cement extravasation (epidural, vascular), rarely neurological injury
• Advantage: Minimally invasive, quick recovery

Embolization Alone:

• Indications: Reduce tumor vascularity, pain palliation
• Technique: Transarterial embolization of feeding vessels
• Agents: PVA particles, coils, Onyx liquid embolic
• Outcomes: Variable pain relief (50-70%)
• Limitations: Recurrence common (reconstitution of blood supply)
• Best Use: Preoperative adjunct before surgery

Surgical Management:

Decompression + Stabilization:

• Indications:
  1. Neurological deficit (myelopathy, radiculopathy)
  2. Spinal instability or pathological fracture
  3. Epidural extension with cord compression
  4. Failed radiation or vertebroplasty
  5. Rapidly progressive symptoms

Preoperative Planning:

• Embolization: Perform 24-48 hours before surgery (reduce bleeding)
  • Essential for vascular lesions
  • Decreases intraoperative blood loss by 40-60%
• Blood Products: Type and cross 4-6 units PRBC (potential massive bleeding)
• Cell Saver: Available for autotransfusion
• Imaging: Updated MRI for surgical approach planning

Surgical Approaches:

Posterior Decompression:

• Laminectomy to decompress spinal cord
• Remove epidural component of hemangioma
• Instrumented fusion if instability (usually required)
• Avoid aggressive curettage of vertebral body (bleeding risk)

Vertebrectomy + Reconstruction:

• Indications: Extensive vertebral body involvement, instability, failed decompression
• Technique:
  • Anterior or posterior approach (or combined)
  • En bloc or piecemeal vertebrectomy
  • Cage reconstruction (titanium mesh, PEEK, allograft strut)
  • Posterior instrumented fusion
• Outcomes: Definitive treatment, low recurrence
• Morbidity: Significant, requires anterior approach often

Intralesional Curettage + Grafting:

• Long bone hemangiomas
• Thorough curettage, local adjuvants (phenol, cryotherapy)
• Bone grafting (autograft or allograft)
• Prophylactic fixation if structural defect

Surgical Risks:

• Massive Hemorrhage: Most significant risk (up to 2-3 liters blood loss)
• Neurological Injury: Cord or nerve root damage
• Incomplete Resection: Residual tumor if bleeding prevents visualization
• Infection: Standard surgical site infection risk
• Instability: May require fusion if destabilizing procedure

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Treatment Algorithm

Decision-Making Framework:

1. Asymptomatic, Incidental:

   • Observation, no follow-up imaging
   • Patient education, discharge

2. Symptomatic Pain, NO Neurological Deficit:

   • First-line: Radiotherapy (20-40 Gy)
   • Alternative: Vertebroplasty/kyphoplasty
   • Embolization if above contraindicated

3. Neurological Deficit or Cord Compression:

   • Urgent MRI to confirm compression
   • Preoperative embolization (24-48 hours prior)
   • Surgical decompression + fusion
   • Consider adjuvant radiotherapy postoperatively

4. Pathological Fracture:

   • Kyphoplasty if stable fracture, no neurology
   • Surgical stabilization if unstable or neurological deficit

5. Aggressive Features (Cortical Destruction, Soft Tissue):

   • Biopsy to exclude angiosarcoma
   • En bloc resection if feasible
   • Radiotherapy + embolization if unresectable

Key Management Principles:

1. Observation is STANDARD for asymptomatic hemangiomas (95%+)
2. Radiotherapy effective for painful hemangiomas WITHOUT neurological deficit
3. Surgery reserved for neurological compromise or failed conservative measures
4. ALWAYS preoperative embolization before surgery (reduces massive bleeding)
5. Vertebroplasty excellent option for painful hemangioma with vertebral collapse

Complications

Spontaneous Complications (Rare):

• Pathological Fracture: Less than 5% (vertebral compression)
• Spinal Cord Compression: Less than 1% (epidural extension)
• Acute Hemorrhage: Extremely rare (acute neurological deficit)
• Pregnancy-Related Enlargement: Rare, usually regresses postpartum

Treatment-Related Complications:

Radiotherapy:

• Radiation myelopathy (less than 1% with modern techniques)
• Vertebral compression fracture (5-10%)
• Skin changes (erythema, pigmentation)
• Lhermitte sign (transient)

Vertebroplasty/Kyphoplasty:

• Cement extravasation: Epidural (2-5%), vascular (1-2%)
• Neurological injury: Less than 1%
• Infection: Less than 1%
• Adjacent level fracture: 5-10%

Surgery:

• Massive intraoperative hemorrhage (10-20% even with embolization)
• Neurological worsening (2-5%)
• CSF leak (3-5%)
• Infection (2-5%)
• Recurrence (5% if incomplete resection)

Prognosis

Asymptomatic Hemangiomas:

• Excellent: Stable lifelong, no intervention needed
• Risk of becoming symptomatic: Less than 1% over lifetime
• No malignant transformation (benign entity)

Symptomatic Hemangiomas:

• Radiotherapy: 80-90% pain relief, gradual over months
• Vertebroplasty: 80-90% immediate pain relief
• Surgery: 85-95% neurological improvement if preoperative deficit
• Recurrence: 5% after surgical excision

Long-Term Outcomes:

• Most patients remain asymptomatic lifelong
• Symptomatic cases respond well to treatment
• Quality of life: Excellent after successful treatment
• Return to activities: Full return in majority

Vertebral Body Lesions:

Metastatic Disease:

• Age: Usually older (greater than 50 years)
• History: Known primary malignancy
• MRI: Dark T1, bright T2 (opposite of hemangioma)
• Distribution: Multiple lesions common
• Pedicle: Often destroyed (hemangioma spares pedicle usually)

Multiple Myeloma:

• Age: Greater than 60 years typically
• Labs: Monoclonal protein, hypercalcemia, anemia
• MRI: Dark T1, bright T2, diffuse marrow infiltration
• Radiology: "Punched-out" lytic lesions, osteopenia

Paget Disease:

• Age: Elderly (greater than 60 years)
• Labs: Elevated alkaline phosphatase
• Radiology: Mixed lytic and sclerotic, "picture frame" vertebra
• Distribution: Polyostotic, pelvis common

Vertebral Chordoma:

• Location: Sacrum most common, can be mobile spine
• MRI: Destructive, soft tissue mass, bright T2
• Age: Adults 40-60 years
• Pathology: Physaliferous cells, brachyury positive

Giant Cell Tumor (Spine):

• Age: 20-40 years
• Location: Sacrum greater than mobile spine
• MRI: Dark T1, heterogeneous T2
• Radiology: Lytic, destructive, extends into soft tissue

Skull Lesions:

Eosinophilic Granuloma (Langerhans Cell Histiocytosis):

• Age: Children less than 10 years
• Radiology: "Punched-out" lytic lesion, beveled edges
• Pathology: CD1a+ Langerhans cells
• Behavior: May spontaneously resolve

Osteosarcoma (Telangiectatic):

• Age: Adolescents/young adults
• Radiology: Destructive, soft tissue mass, periosteal reaction
• Pathology: Malignant osteoid production, cellular atypia
• Prognosis: Poor if malignant

Intraosseous Meningioma:

• Location: Skull vault, hyperostotic
• Radiology: Sclerotic, "sunburst" can mimic hemangioma
• MRI: Homogeneous enhancement
• Age: Adults 40-60 years

Australian Practice Considerations

RACS Orthopaedic Training Relevance:

• Bone hemangioma appears in FRACS examination as a differential for lytic bone lesions
• Key knowledge: radiographic appearance (corduroy, polka-dot), pathognomonic MRI features (bright T1 AND T2), and recognition that most are incidental findings requiring observation only
• Understanding aggressive vs typical hemangioma features is essential for viva scenarios
• Vertebral hemangioma may present as differential in spine cases

Interventional Radiology Services:

• Preoperative embolization available at major Australian tertiary centres
• Typically performed 24-48 hours before surgical decompression
• Coordinated care between spinal surgeon and interventional radiologist essential

Clinical Referral Pathways:

• Incidental vertebral hemangiomas: GP or physician can manage with observation (no orthopaedic referral needed)
• Symptomatic hemangiomas with neurological deficit: Urgent referral to spinal surgery service
• MDT discussion recommended for complex cases requiring surgery