Study design: Case report.
Objectives: To demonstrate the efficacy of a transmaxillary and transmandibular approach in achieving a wide view and the aggressive resection of a retropharyngeal chordoma originating from C1.
Summary of background data: Although aggressive surgical resection has been recommended for the treatment of chordomas, wide exposure of the tumors in the upper cervical region is a challenge.
Methods: A 19-year-old man presented with a large ossified retropharyngeal chordoma (6 cm in diameter) originating from the right side of the anterior arch of C1, and extending from the clivus to the C2/3 intervertebral disc level in the sagittal plane. A posterior occipitocervical (O-C3) fusion with an iliac bone graft was first performed. Ten days after the fusion, the tumor was resected using a mandible and tongue midsplitting approach combined with a Le Fort I (transmaxillary) osteotomy, which allowed us to expose the entire tumor. The tumor was hard and immovable because of ossification. The main part of the tumor was resected from the anterior arch of C1, and then the C1 anterior arch was resected en bloc. The retropharyngeal wall was reconstructed using a vascularized radial forearm flap. Radiation therapy (60 Gy) was performed after surgery.
Results: No local recurrence or metastasis was observed 3 years after the operation. The patient had no complaints and has returned to his previous job as a manual laborer.
Conclusions: A transmaxillary and transmandibular approach allowed us to obtain a complete view of a large immovable chordoma located ventral to the upper cervical spine. This enabled us to resect totally the tumor into 2 pieces without major complications or sequelae. This approach is useful for the resection of large tumors located in the median upper cervical spine.