Misdiagnosis of primary dumbbell chordoma of the cervical spine

Aim: This study aimed to analyze the clinical characteristics and outcomes of patients with primary dumbbell chordoma of the cervical spine and to summarize the causes of misdiagnosis.

Methods: The clinical data of patients were retrospectively collected. The diagnostic process, surgical procedures, and outcomes were analyzed, then the difference was compared between dumbbell and non-dumbbell chordomas of the cervical spine.

Results: This study included six patients with primary dumbbell chordoma (one male and five females) with a mean age of 32.2 ± 24.5 years (range: 5-61 years). Five cases with no computed tomography (CT) examination before the first operation were misdiagnosed, and on magnetic resonance imaging (MRI), primary dumbbell chordoma showed the following specific features: extensive invasion of the surrounding soft tissues with an obscure boundary (≥5 cm), intervertebral disc sparing, and hemorrhagic necrosis, furthermore, the CT features included atypical destructive vertebral lesions, minimal intralesional calcification, and neural foraminal enlargement. After comparison with non-dumbbell chordomas, it show statistical difference (p < 0.05) in terms of calcification, foramen enlargement, FNA, misdiagnosis rate but with different recurrent rate.

Conclusion: Primary dumbbell chordomas of the cervical spine can easily be misdiagnosed as neurogenic tumors. Preoperative CT-guided fine-needle aspiration puncture biopsy helps make an accurate diagnosis. Gross total excision with postoperative radiotherapy has been proven effective in reducing the recurrence rate.