Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
Sign up to receive an email alert when a new Case of the Week is posted.
January 11, 2016
Aggressive Spinal Hemangioma
- Background:
- Hemangiomas can be classified as typical, atypical and aggressive (compressive). These are benign tumors composed of capillary-sized to cavernous blood vessels. The term aggressive refers to the presence of radiologic features such as extension beyond the vertebral body, destruction of the cortex, and invasion of the epidural and paravertebral spaces.
- Aggressive hemangioma can occur at any age, with peak prevalence in young adults, and is localized preferentially in the thoracic spine.
- Clinical Presentation:
- Neurologic symptoms due to compression of the spinal cord, nerve roots, or both, leading to myelopathy and/or radiculopathy
- Clinical worsening and growth during pregnancy is a well known phenomenon. The main explanation is vena cava compression and re-routing of blood to the paravertebral, epidural, and acygous venous system.
- Key Diagnostic Features:
- CT scan reveals hypodense expansile vertebral body mass, with soft tissue extension and spinal cord/nerve root compression. Typical "polka-dot" and "corduroy" signs can guide the correct diagnosis.
- MRI shows a T1 hypointense, high-T2 lesion with variable contrast enhancement. Aggressive hemangiomas are difficult to diagnose by MRI because findings overlap with those of metastatic disease and focal bone marrow infiltration.
- Differential Diagnosis:
- Metastases: Findings that favor an aggressive hemangioma over a metastasis are maintained vertebral body height, a sharp margin with normal marrow, intact cortex adjacent to a paraspinal mass, enlarged paraespinal vessels, and the "polka dot" sign on axial images.
- Solitary bone plasmocytoma: Classically, plasmocytomas can have a "mini brain" appearance on axial CT and MR imaging (thickened cortical struts resemble the sulci seen in the brain) or, less typically, multicystic "soap bubble" appearance.
- Lymphoma: The epidural component of a vertebral lymphoma appears less hyperintense on T2W compared with the hypervascular component of aggressive hemangioma.
- Epitheloid hemangioendothelioma: Lytic pattern of bone destruction with mixed signal intensity on T1- and T2-weighted MRI due to the absence of fat and the presence of inflammatory infiltrate
- Treatment:
- Surgery in cases of rapid or progressive neurologic symptoms: compressive myelopathy or radiculopathy
- Radiotherapy for patients with slow progressive neurologic deficits
- Recently, there has been increasing interest in the management of symptomatic lesions using vertebroplasty, transarterial embolization, and direct intralesional ethanol injection (such us in the presented case).
Current Issue
Case Collections
