Aggressive Hemangioma of the Spine: Symptoms and Treatment

Aggressive hemangiomas of the spine are rare but can cause significant pain, neurological deficits, and structural instability. Unlike non-aggressive hemangiomas, these lesions may expand rapidly, leading to spinal cord compression or vertebral collapse. Early recognition is crucial to prevent severe complications.

A combination of imaging, clinical assessment, and appropriate treatment strategies helps manage symptoms and maintain spinal function.

Pathologic Features

Aggressive spinal hemangiomas exhibit distinct histological and radiological characteristics. These vascular tumors originate from proliferating endothelial cells within the vertebral body, forming a dense network of thin-walled blood vessels interspersed with trabecular bone. Unlike typical hemangiomas, which remain confined and asymptomatic, aggressive variants erode cortical bone and extend into the epidural space, increasing the risk of spinal cord compression and neurological impairment.

Microscopically, these lesions contain a high proportion of endothelial-lined vascular channels, often accompanied by hemosiderin deposits and reactive bone formation. Their large, dilated vessels with minimal stromal support contribute to fragility, making them prone to hemorrhage and microfractures. Additionally, aggressive hemangiomas exhibit higher cellular density and mitotic activity, suggesting greater proliferative potential. On imaging, they often present with a “honeycomb” or “polka-dot” pattern on MRI and CT scans due to the interspersion of vascular spaces and residual trabecular bone.

Increased vascular endothelial growth factor (VEGF) expression has been linked to their progression, promoting angiogenesis and allowing invasion of adjacent structures. Mechanical stress on the vertebral column may further drive expansion, particularly in weight-bearing regions such as the thoracic and lumbar spine. This interplay between vascular proliferation and mechanical forces underscores the potential for rapid progression, necessitating close monitoring.

Spine Stability Considerations

Structural integrity is a key concern, as aggressive hemangiomas weaken vertebral architecture and increase the risk of pathological fractures. Unlike non-aggressive hemangiomas, which typically do not compromise load-bearing capacity, aggressive variants erode cortical bone and infiltrate adjacent structures. This destruction can lead to vertebral collapse, kyphotic deformities, and instability, particularly in the thoracic and lumbar regions, where mechanical stress is greatest.

Biomechanical studies show that affected vertebrae exhibit reduced bone mineral density and compromised trabecular integrity. Microfractures within the lesion further weaken the spine’s ability to support axial loads, potentially causing dynamic instability, where normal movement induces vertebral shifting. This instability places strain on adjacent spinal segments, accelerating degenerative changes and increasing susceptibility to complications such as facet joint arthropathy or disc herniation.

Radiographic assessment plays a pivotal role in evaluating spinal stability. MRI and CT imaging often reveal cortical thinning, vertebral height loss, and epidural extension, all indicators of structural failure. The Spinal Instability Neoplastic Score (SINS) helps quantify instability risk, incorporating lesion location, spinal alignment, and vertebral body involvement. A higher SINS score suggests a greater likelihood of mechanical compromise, guiding decisions regarding surgical stabilization.

Clinical Presentation

Patients with aggressive spinal hemangiomas often experience persistent, localized pain that worsens with movement or weight-bearing activities. Unlike incidental vertebral hemangiomas, which remain asymptomatic, aggressive variants provoke significant discomfort due to cortical bone erosion and soft tissue involvement. The pain is typically deep and aching, with nocturnal exacerbation that does not respond well to conventional analgesics. Some patients report radicular pain radiating along dermatomal distributions, reflecting nerve root irritation or compression.

As the lesion progresses, neurological symptoms may emerge due to spinal cord or nerve root encroachment. Motor deficits, sensory disturbances, or autonomic dysfunction can develop depending on the affected spinal level. Thoracic involvement may lead to lower limb weakness, gait instability, or hyperreflexia, while lumbar lesions can cause radiculopathy, foot drop, or bowel and bladder dysfunction. The severity of neurological impairment often correlates with the extent of epidural extension and compression, making early recognition essential to prevent irreversible damage.

Vertebral collapse can further complicate the condition, resulting in spinal deformities such as kyphosis or scoliosis. These changes exacerbate pain and functional limitations, particularly in younger individuals or those with preexisting spinal conditions. Patients may adopt compensatory postures to alleviate discomfort, leading to secondary musculoskeletal strain and reduced mobility. In severe cases, compression fractures can precipitate acute neurological deterioration, necessitating urgent intervention.

Diagnostic Approaches

Diagnosing aggressive spinal hemangiomas requires a combination of imaging and clinical correlation. Magnetic resonance imaging (MRI) is the primary tool due to its sensitivity in detecting soft tissue involvement and vascular characteristics. These lesions typically appear hyperintense on T1- and T2-weighted sequences, reflecting their high-fat and vascular content. Contrast-enhanced MRI reveals intense gadolinium uptake, signifying active vascular proliferation and helping differentiate these hemangiomas from metastases or primary bone tumors.

Computed tomography (CT) complements MRI by providing detailed visualization of bone integrity. A hallmark radiologic feature of aggressive hemangiomas is the “polka-dot” sign, caused by thickened vertical trabeculae within the affected vertebra. Cortical thinning, expansion, and erosion further indicate aggressive behavior. In cases where spinal instability is suspected, CT reconstruction aids in surgical planning by assessing vertebral height loss and fracture risk.

In some instances, a biopsy may be warranted when imaging findings are inconclusive or malignancy remains a concern. Histopathological examination confirms the diagnosis by identifying proliferative endothelial-lined vascular channels interspersed with trabecular bone. However, due to the vascular nature of these lesions, biopsy carries a risk of significant bleeding and is generally reserved for cases where imaging alone does not establish a definitive diagnosis.

Surgical And Non-Surgical Interventions

Management depends on lesion size, location, and structural compromise. Some patients respond well to conservative measures, while others require surgery to prevent worsening deficits and instability.

Surgical Approaches

Surgery is necessary when significant spinal cord compression, vertebral collapse, or progressive neurological impairment occurs. Decompression procedures, such as laminectomy, relieve pressure on the spinal cord and nerve roots. However, if the vertebral body is extensively weakened, stabilization with spinal instrumentation and fusion may be required to restore structural integrity.

In severe cases, vertebrectomy with reconstruction may be indicated, particularly when multiple levels are affected or kyphotic deformity is present. Reconstruction typically involves titanium cages, bone grafts, or polymethylmethacrylate (PMMA) cement to restore vertebral height and maintain alignment. Due to the vascular nature of these tumors, surgery carries a risk of excessive bleeding, necessitating careful intraoperative hemostasis and, when feasible, preoperative embolization.

Non-Surgical Approaches

For patients with mild to moderate symptoms, non-surgical interventions can help manage pain and slow lesion progression. Percutaneous vertebroplasty and kyphoplasty reinforce weakened vertebrae by injecting PMMA cement, providing immediate pain relief and reducing fracture risk. These minimally invasive procedures are particularly beneficial for patients with vertebral body involvement but without significant neurological compromise.

Radiotherapy may be considered for inoperable lesions or those that continue to progress despite other treatments. Targeted radiation reduces tumor vascularity and slows expansion. Embolization techniques, which selectively occlude feeding blood vessels, can be used preoperatively to reduce intraoperative bleeding or as a standalone treatment to limit lesion growth. While these approaches do not remove the hemangioma entirely, they effectively alleviate symptoms and improve quality of life.

Distinctions From Non-Aggressive Hemangiomas

Aggressive hemangiomas differ significantly from non-aggressive counterparts in behavior and clinical impact. While typical vertebral hemangiomas are often incidental findings, aggressive forms exhibit rapid expansion, cortical erosion, and epidural extension, increasing the risk of neurological complications.

Radiological features help differentiate them. Non-aggressive hemangiomas display a “corduroy” pattern on sagittal MRI due to their well-organized trabecular structure, whereas aggressive variants often present with a “honeycomb” or “polka-dot” pattern indicative of cortical thinning and vascular proliferation. Additionally, aggressive hemangiomas show higher contrast enhancement on MRI, reflecting increased vascularity and potential for expansion beyond the vertebral body. These imaging characteristics guide clinicians in distinguishing between benign and aggressive lesions, ensuring appropriate follow-up and intervention.