Cervical Tumor in a 14-year-old Female

History

The patient is a 14-year-old female who presents complaining of neck pain and headaches for five months. Recently, the patient experienced upper and lower extremity weakness.

Examination

Upon examination, there was limited cervical range-of-motion and torticollis. Mild diffuse upper extremity weakness, 5-/5, upper/lower extremity hyper reflex.

Images

Figure 1. Lateral x-ray shows pathological fracture at C2

MRI studies represented in Figures 2, 3A, 3B.

Figure 2. Sagittal MRI shows myelopathy, replacement of C2 vertebra with tumor, and extensive prevertebral tumor

Figure 3A. Axial MRI, C2

Figure 3B. Axial MRI, C3

CT studies represented in Figures 4A-B, 5A-B, and 6A-C.

Figure 4A. Anterior CT scan, tumor at C2-C3

Figure 4B. Sagittal CT scan, tumor at C2-C3

Figure 5A. Axial CT scan, C2

Figure 5B. Axial CT scan, C3

Figure 6A. Sagittal CT scan

Figure 6B. Sagittal CT scan

Figure 6C. Sagittal CT scan

Diagnosis

The patient was diagnosed with a chordoma at C2 and C3 and left vertebral artery occlusion. A chordoma is a locally aggressive tumor with a high rate of recurrence with incomplete resection. It requires total resection, if feasible.

Selected Treatment

In this case, a left-sided balloon occlusion of the vertebral artery was performed, and then there were two stages to the surgery:

• Stage 1 - combined transoral and high cervical anterior resection and bone graft (fibular autograft) and plating
• Stage 2 - posterior completion of resection and occiput to C4 instrumentation and fusion

Outcome

The patient did well following surgery except for the need for a percutaneous gastrostomy tube for 6-weeks. There was not local recurrence but she developed pulmonary metastasis 9-months after surgery, which required resection. The patient continues to be closely followed.

Postoperative imaging at 3-months presented in Figures 7A-B, and 8A-C.

Figure 7A. Lateral x-ray at 3-months postop

Figure 7B. Anterior posterior x-ray at 3-months postop

Figure 8A. CT scan at 3-months postop

Figure 8B. CT scan at 3-months postop

Figure 8C. CT scan at 3-months postop

Case Discussion

This case of high cervical chordoma is challenging. Ideally, gross total resection of chordoma is the preferred treatment goal due to the high rate of local recurrence with subtotal resection.

In this case, due to the proximity of the vertebral arteries, a pre-operative angiogram is helpful. If one of the vertebral arteries is encased in tumor, then pre-operative test occlusion with subsequent embolization and sacrifice of the vessel would allow for gross total resection of the tumor.

A combined anterior and posterior approach is needed to remove the tumor and fixate the C2 area.

Typically, for lesions such as this, I prefer a transmandibular/transoral approach with the assistance of an ENT surgeon (along with temporary tracheostomy). I prefer to open the posterior phaynx with a "C" shaped incision which provides more exposure than a midline incision. This approach allows the surgeon to approach and resect the lesion with its soft tissue component with a view from the bottom of the clivus to C3. With such an approach, I prefer to expose the periphery of the tumor without violating it. By circumnavigating the periphery of the tumor, the surgeon can prepare for a gross total resection. The use of neuronavigation can assist with this resection.

Once the lesion is removed, a gap will be created in the upper cervical spine, which can be anteriorly reconstructed with either a structural graft or cage filled with iliac autograft. Anterior buttress fixation can help to secure the graft. The "C-shaped" incision of the posterior pharynx helps in these cases, as the incision line is far away from the underlying graft and thus is less prone to pressure ulceration and breakdown.

Finally, a posterior approach is needed for occipitocervical fixation and fusion to secure the craniocervical junction.