Cervical chordoma surgically resected via three‐stage procedure with intraoperative images

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A 78‐year‐old man presented to the head and neck clinic with a 2‐month history of a right‐sided neck mass. Past medical history was notable for hypertension and glaucoma. Examination revealed a large submucosal mass involving the posterior and right lateral oropharyngeal wall. A needle aspiration biopsy was performed, which favoured chordoma.
Magnetic resonance imaging demonstrated a large enhancing expansile soft tissue mass (blue arrow) centred on the right aspect of the C2 vertebra. This had invaded the transverse process and right aspect of the C2 vertebral body. The lobulated mass caused partial effacement of the airway (red arrow) as well as posterior tumour extension compressing the spinal cord (Fig. 1). A cervical computed tomography (CT) further characterizing the mass demonstrated a mixed solid/cystic lesion measuring 36 × 71 × 52 mm. Digital subtraction angiography showed 80–90% occlusion of the right vertebral artery and patent left vertebral.
Preoperative embolization of the right vertebral artery was performed. The patient then underwent a successful three‐stage surgical excision of the mass by the ENT and neurosurgical teams (Fig. 2).
Stage 1 (neurosurgical team, posterior approach): instrumented fusion from the occiput to C4 vertebra was performed and the posterior extradural chordoma was resected. This involved lateral mass screws placed bilaterally on C1, C3 and C4 vertebrae and a left‐sided C2 pedicle screw. C2 and partial C3 laminectomies were performed to access the tumour dorsally. The right C2 and C3 nerve roots were sacrificed due to tumour involvement.
Stage 2 (ENT team, patient placed supine): a surgical tracheostomy was created. The anterior portion of the spinal chordoma was removed via a right lateral neck skin crease incision anterior to the sternocleidomastoid.
Stage 3 (neurosurgical team, anterior approach): C2 vertebrectomy with aggressive tumour debulking within the epidural space was performed along with decompression of the spinal cord and insertion of a C2 vertebral cage. Plate and screws were inserted on C1 and C3 vertebrae.
Post‐operative CT showed successful tumour excision and spinal instrumentation (Fig. 3) and the patient made an uneventful recovery. There were no new neurological deficits and power was preserved bilaterally. Histological analysis of the anatomical specimens confirmed the diagnosis of chordoma.
Post‐operatively, the patient underwent 6 weeks of adjuvant radiotherapy of 25 fractions to the tumour bed.
At 1‐year follow‐up, there was no evidence of recurrence.
Chordomas are extremely rare bone malignancies with an incidence of 0.8 per 100 000 persons.1 Most commonly they arise in the sacrum (45–50%), followed by the skull base (35–40%) and mobile spine (10–15%).2 While current studies suggest a 5‐year survival of 50–68%, only 5% have distant metastasis at presentation.2
Surgery is the mainstay of therapy for these largely chemo‐ and radio‐resistant tumours, with the extent of surgical resection correlating strongly with prognosis.1 Aggressive resection of cervical tumours is complex due to the proximity of these tumours to important anatomical structures.3 A variety of posterior, anterior and lateral approaches are available to a surgeon's armamentarium to allow for the most aggressive and stable resection.
A direct posterior approach is the simplest approach allowing safe and wide access to dorsal tumours and enabling concomitant occipitocervical fusion.3 However, access to ventral elements usually mandates a high anterior cervical approach.
Anterior approaches for high cervical pathologies include transoral and retropharyngeal techniques.4 These enable resection of ventral elements, however, these can be limited by dental malocclusion and palatal dysfunction.3 Furthermore, a surgical tracheostomy is generally indicated in anticipation of post‐operative oedema.
An anterior transoral approach can be limited by inadequate lateral and caudal access as well as the complications associated with interrupting the pharyngeal mucosa, cerebrospinal fluid leak and relatively high morbidity.4 An anterior retropharyngeal approach, however, allows for greater access to tumours with lateral or caudal extension past C3.
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