Bone Tumors at the Cranio-cervical Junction. Surgical Management and Results from a Series of 41 Cases

Background: Bone tumors located at the cranio-cervical junction (CCJ)are rare. Tumoral involvement of the neighbouring structures including bone, nerves and vertebral artery and the dynamic aspects of the bone structures raise technical difficulties in the surgical approach. The surgical management includes tumoral resection and stabilization of the CCJ.

Methods: Forty-one patients presenting a bone tumor (26 benign and 15 malignant tumors), excluding chordomas, located at the CCJ (including lower third of the clivus, C1 and C2) were observed over 20 years from 1981 to 2001. Imaging work-up included CT scanner with bone windows sequences and reconstruction in the coronal and sagittal plane; since 1984 most of the patients (N=35) underwent a MRI and angioMR scanning. Vertebral angiography was rarely performed (N=9) and mostly when the diagnosis was doubtful. In some cases the diagnosis was clear but in others, imaging studies showed destructive lesions suggesting a malignancy, which sometimes required a biopsy (N=4). The surgical resection was only performed through a lateral approach.

Findings: Complete resection was achieved in 38 cases while in 3 cases a small remnant was left behind. A complementary stabilization procedure was necessary in 18 cases using either bone grafting during the same procedure and through the same approach (N=5) or a craniocervical plating and bone grafting (N=13). No recurrence in the group of benign tumors was seen during an average follow-up of 6 years (from 2 to 11 years). The pre-operative symptoms of pain and neck stiffness, improved or disappeared in most patients. Three patients with lower cranial nerves (N=2) or sphincter disturbances (N=1) remained unchanged. One patient with tetraplegia eventually died.

Conclusions: Various types of bone tumors may be found at the CCJ. Confusion between benign and malignant tumor or pseudo tumors must be avoided, sometimes requiring a biopsy. Surgery using a lateral approach, usually permits the surgeon to achieve a complete resection either preserving the stability of the CCJ whenever intact or associated with a stabilization procedure.