Transnasal and Transoral Approach for a Craniocervical Junction Chordoma: A Case Presentation

Introduction: Craniocervical junction chordomas are some of the most difficult tumors to treat surgically. We describe a case of an approximately 5 cm³ extra- and intradural craniocervical junction chordoma resected via a transnasal/transoral approach.

Case Presentations: A 35-year-old male presented with a progressively enlarging craniocervical tumor causing lower cranial nerve deficits including hoarseness, left-sided tongue atrophy, and shoulder shrug weakness.

Management: Surgical resection and treatment of the tumor involved a multidisciplinary approach with neurosurgery, otolaryngology, and radiation oncology. Via the transnasal, transpterygoid, and transoral approaches, a wide exposure of the nasopharynx was achieved. A pharyngeal wall flap was taken down to allow access to the vertebral column. The lower clivus superior to the tumor, the anterior arch of C1, and the superior aspect of the odontoid process were drilled. The dura was circumferentially opened around the tumor and the tumor was internally debulked with an ultrasonic aspirator. Pathology revealed conventional chordoma (K_i-67 index 3%). The chordoma was sharply dissected away from the medulla, ventral spinal cord and the lower cranial nerves. Closure consisted of DuraGen onlay, tensor fascia lata and fat graft, bilateral nasoseptal flaps sutured to an inferior pharyngeal flap, Surgicel, and Adherus. The patient returned to his neurological baseline; however, 2 weeks postoperatively developed a CSF leak which ultimately required shunting. The patient also required an occipital-cervical fusion.

Discussion: Complete decompression of the medulla and upper cervical cord were achieved ([Fig. 1]). Postoperative imaging showed an approximately 80% tumor resection with residual remaining tumor in the left occipital condyle ([Fig. 2]). As described in the literature, the major limitation of an anterior approach to the lower clivus is the lateral extension of tumor to the condyles. The patient also underwent an occipital to C5 fusion and this was stable at 6 months post-op ([Fig. 3]). The patient is now recovering well at six months from surgery with no tumor growth on follow-up MRI, and further management of the patient with proton beam radiotherapy and additional surgical planning is ongoing.

Fig. 1 Sagittal T1 with contrast MRI images preoperatively and 6-month postoperatively.

Fig. 2 Axial T2 MRI images preoperatively and 6-month postoperatively.

Fig. 3 Lateral X-ray demonstrating occipital–C5 fusion at 6-month postoperatively.

No conflict of interest has been declared by the author(s).

Publication History

Article published online:
07 February 2025