Craniovertebral Junction Tumor
Definition: CVJ tumors are neoplastic lesions involving the occipital bone, atlas (C1), axis (C2), and adjacent structures. These tumors may be extradural, intradural-extramedullary, or intramedullary.
Common Tumor Types
- Meningioma
- Chordoma
- Schwannoma (CN XI, XII)
- Paraganglioma
- Metastasis
- Epidermoid cyst
- Neurofibroma
Treatment
Goals of Treatment
- Maximal safe resection or control
- Preservation of neurological function
- Stabilization of the craniovertebral junction (if destabilized)
- Symptom relief (e.g., brainstem compression, myelopathy, cranial nerve dysfunction)
Multidisciplinary Planning
- Imaging: MRI with contrast ± CT with bone windows
- Neuro-navigation / intraoperative neuromonitoring
- Preoperative angiography ± embolization (esp. in vascular tumors like meningiomas or paragangliomas)
- Histology: Biopsy when necessary (for chordomas, metastases)
Surgical Indications
- Neurological deterioration
- Brainstem compression
- Spinal cord compression or instability
- Progressive symptoms
- Tumor growth
Surgical Approaches
Non-Surgical Management
- Stereotactic Radiosurgery (SRS): e.g., for chordomas, schwannomas, or residuals
- Fractionated Radiotherapy: if high risk for injury with surgery
- Chemotherapy: rarely indicated, unless lymphoma or metastasis
- Observation: for small, asymptomatic, benign tumors in high-risk locations
Complications
- Lower cranial nerve palsies
- CSF leak
- Vertebral artery injury
- Instability requiring fusion
- Dysphagia / aspiration
- Infection / wound healing issues
Outcome Predictors
- Tumor location and extension (midline vs lateral)
- Histology (benign vs aggressive)
- Preoperative neurological status
- Surgeon’s experience and approach selection
- Use of neuromonitoring and neuronavigation