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Cervical chordoma


Introduction

Chordomas are uncommon malignant neoplasms of the bone that can arise anywhere along the central nervous system. These tumors originate from persisting remnants of the notochord, and their behavior is aggressive due to their invasiveness and high recurrence rates. Distant metastases are not unusual, and sacral and vertebral chordomas are more prone to metastasize. Chordomas most often metastasize to the lung, followed by the lymph nodes and bone. This entity represents 1% to 4% of all primary malignant bone tumors. They show predilection for the sacrococcygeal region (49% of the cases), followed by the clival area (36%) and the mobile spine (15%). Interestingly, chordomas in the cervical spine account for only 6% of the cases. Vertebral chordomas occur in the following order of frequency: lumbar, cervical, and thoracic regions. Cervical chordomas affect males and females evenly and appear one decade earlier than other locations. Patients typically present with cervical pain, progressive dysphagia or dysphonia, and the tumor is locally advanced by the time of diagnosis. Posterior growth can lead to radicular symptoms or even myelopathy. Complete surgical resection with or without radiation therapy remains the mainstay of treatment. However, local recurrence affects more than 50% of patients after total resection with or without radiation. In this chapter, we present a case of a patient with right upper extremity weakness due to recurrent C2 chordoma.

Example case

  • Chief complaint: progressive right upper extremity weakness

  • History of present illness: This is a 70-year-old male with a history of C2 chordoma status post resection in 2011 followed by occipital to T2 fusion and radiation therapy, reflux disease, glaucoma, and hypertension, who presents with progressive right upper extremity weakness. In 2011, he presented with swallowing dysfunction and was found to have a large C2 chordoma and underwent transoral C2 corpectomy and partial resection with C2 laminectomy and C1-C4 fusion. He later developed instability and underwent occipital to T2 fusion. He underwent postoperative fractionated radiation therapy and imatinib chemotherapy. He presented with 6 months of right upper extremity intrinsic hand weakness. The patient underwent a magnetic resonance imaging of the cervical spine that demonstrated the presence of a large lobulated recurrent tumor at the level of the upper cervical segment ( Fig. 59.1 ).

    Fig. 59.1, Preoperative cervical spine images. (A) Sagittal T2 and (B) axial T1 with contrast magnetic resonance images demonstrating a large lobulated recurrent tumor involving the C2 corpectomy bed and affects the residual nonresected left lateral mass and odontoid process, the medial portion of the right C1 arch, and the right occipital condyle. There is also a large lobulated extension into the cervical medullary junction on the right, and the left ventral lateral epidural space. Severe compression of the cervicomedullary junction is evident. (C) Sagittal computed tomography scan demonstrating postsurgical changes after craniospinal fusion. Residual chordoma centered at the cranio-cervical junction with surrounding osseous involvement is seen.

  • Medications: atenolol, amlodipine, ranitidine, latanoprost

  • Allergies: no known drug allergies

  • Past medical and surgical history: C2 corpectomy and C1–4 fusion in 2011, occipital to T2 fusion in 2012

  • Family history: noncontributory

  • Social history: retired, no smoking, no alcohol

  • Physical examination: awake, alert, and oriented to person, place, and time; cranial nerves II–XII intact; bilateral deltoids/triceps/biceps 5/5; right interossei 4/5, left interossei 5/5; iliopsoas/knee flexion/knee extension/dorsi, and plantar flexion 5/5

  • Reflexes: 3+ in bilateral biceps/triceps/brachioradialis with positive Hoffman; 3+ in bilateral patella/ankle; three beats of ankle clonus and positive Babinski; sensation intact to light touch

  • Ziya L. Gokaslan, MD

  • Tianyi Niu. MD

  • Neurosurgery

  • Brown University

  • Providence, Rhode Island, United States

  • Tong Meng, MD

  • Orthopaedic Surgery

  • Shanghai General Hospital

  • Shanghai Jiao Tong University

  • Shanghai, China

  • Jorge Navarro Bonnet, MD

  • Neurosurgery

  • Medica Sur, Mexico City, Mexico

  • Meic H. Schmidt, MD, MBA

  • Neurosurgery

  • University of New Mexico

  • Albuquerque, New Mexico, United States

Preoperative
Additional tests requested

  • Cerebral angiogram with right vertebral occlusion evaluation

  • Sacrifice right vertebral artery if able to tolerate

  • C-spine x-ray

  • CT angiography

  • Cerebral angiogram with right vertebral occlusion evaluation

  • C-spine flexion-extension x-rays

  • Electrophysiological evaluation (SSEP, NCS, EMG)

  • Swallow study

  • Vocal cord function

  • Chest/abdomen/pelvis CT

  • Cerebral angiogram with possible left vertebral artery embolization

Surgical approach selected C1-3 laminectomy and tumor debulking with occipital-C3 fusion

  • Stage 1: C1-3 posterior decompression

  • Stage 2: anterolateral retropharyngeal transcervical resection and C1-3 cage

Anterior C1-5 tumor debulking and decompression Left far lateral transcondylar resection of C2
Goal of surgery Palliative neurological function preservation Palliative spinal cord decompression Safe maximal resection, spinal cord decompression Palliative spinal cord decompression
Perioperative
Positioning Prone on Jackson table, with pins

  • Stage 1: prone

  • Stage 2: supine with head rotated to left 30 degrees

Supine, with Mayfield pins Lateral, with Mayfield pins
Surgical equipment

  • IOM (MEP/SSEP)

  • Surgical navigation

  • Surgical microscope

  • Ultrasonic aspirator

  • Dural sealant

  • Ultrasound

Ultrasonic bone scalpel

  • IOM

  • Fluoroscopy

  • Ultrasonic aspirator

  • O-arm

  • IOM

  • Surgical microscope

Medications Steroids, maintain MAP >85 Steroids Steroids Steroids
Anatomical considerations Right vertebral artery, spinal cord Anterior C1 arch, right vertebral artery Right vertebral artery, lower cranial nerves, anterolateral spinal cord C1 tubercle, C2 nerve roots
Complications feared with approach chosen Spinal cord injury, vertebral artery injury, CSF leak Injury to right vertebral artery/carotid artery/laryngeal vessels and nerve/submandibular duct/hypoglossal nerve Injury to right vertebral artery, lower cranial nerve injury, spinal cord injury, bleeding Spinal cord injury, CSF leak
Intraoperative
Anesthesia General General General General
Exposure Occiput-C3 C1-3 C1-5 Skull base, C1, C2
Levels decompressed C1-3 C1-3 C1-5 C1-2
Levels fused Occiput-C3 C1-3 None None
Surgical narrative Intubated, positioned prone, midline incision with hock stick toward right, dissect down to expose posterior bony elements and previous hardware, free suboccipital muscles from bony element to expose suboccipital bone/C1-3 lateral mass on right, perform suboccipital craniectomy and C1-3 laminectomy, remove previous right-sided rod, remove previous right C1 lateral mass and C2 screws, place navigation reference array, obtain intraoperative images, visualize tumor, tumor debulked until sufficient spinal cord decompression with microscopic visualization, resect C1 and C2 lateral masses if necessary, ensure right vertebral artery sacrificed by placing aneurysm clip, intraoperative ultrasound to ensure adequate decompression, assess residual lateral masses and reinstrument, can use occipital condyle screws as salvage if occipital plate not sufficient Intubated, positioned prone, posterior midline longitudinal incision, remove posterior arch of C1 and lamina of C3 with ultrasonic bone scalpel, position supine with head rotated 30 degrees to left and slightly extended, anterolateral retropharyngeal transcervical approach, incision made from midline below the chine and toward the body of the mandible to the mastoid, incision curved downward and medially along posterior border of sternocleidomastoid, open platysma, develop fascial plane between pharyngeal and prevertebral musculature, separate carotid sheath laterally from esophagus and trachea medially, identify hypoglossal nerve by locating posterior digastric muscle and stylohyoid, expose external carotid branches and superior laryngeal nerve, palpate anterior arch of C1, open posterior pharyngeal tissue, expose C1-3, remove as much tumor as possible, reconstruct with titanium mesh filled with allograft, confirm hardware location with x-ray, closure in layers Position supine with mild elevation of head and slight neck extension, fluoroscopy/O-arm to plan incision aiming for C5, displace carotid-jugular vessels laterally and trachea/esophagus medially, internally debulk tumor with coagulation and ultrasonic aspiration, avoid going too lateral to right vertebral artery as well as too deep for lower cranial nerves and spinal cord, support with hemostatic agents if needed, duraplasty with dural graft and sealant if CSF leak encountered, review surgical bed for esophageal injury with water and infuse air through nasogastric tube, layered closure Intubated with reinforced ET tube, lateral decubitus position with left side up, lazy S-incision, muscles dissected down to C1/C2/base of skull, resect embolized vertebral artery, ligate both C1 and C2 nerve roots, resect tumor as much as possible, no reconstruction
Complication avoidance Sacrifice right vertebral artery if possible, surgical navigation, decompress spinal cord, remove hardware for improved access, intraoperative ultrasound to assess decompression, occipital condyle screws as salvage if necessary, new rods placed and secured, closure in layers Two-staged approach, decompress posteriorly, preserve hypoglossal nerve, palliative debulking Anterior approach, avoid going too far right to avoid injury to vertebral artery and too deep for lower cranial nerves, duraplasty if CSF leak encountered, evaluate for esophageal injury Vertebral artery embolization, ligation of C1-2 nerve roots
Postoperative
Admission ICU ICU ICU ICU
Postoperative complications feared CSF leak, spinal cord injury, hardware failure Neurological deficit, spinal instability Hematoma, lower cranial neuropathy Spinal cord injury, CSF leak
Anticipated length of stay 4–5 days 10 days 4–5 days 4–5 days
Follow-up testing

  • MRI C-spine prior to radiation therapy

  • Standing AP/lateral cervical x-rays prior to discharge

  • C-spine x-ray 1 day after surgery

  • CT and MRI 3 days after surgery, 3 months, 6 months, 6 month intervals for 2 years, then annually

MRI C-spine within 48 hours of surgery MRI and CT while inpatient
Bracing None Cervicothoracic brace for 3–6 months None None
Follow-up visits 2 weeks and 3 months after surgery 3 months, 6 months, 6 month intervals for 2 years, then annually 2 weeks after surgery 2 weeks after surgery
Radiation therapy for STR Proton beam Proton beam Radiosurgery Proton beam
Radiation therapy for GTR Proton beam Proton beam Observation Proton beam
AP , Anteroposterior; CSF , cerebrospinal fluid; CT , computed tomography; EMG , electromyography; GTR , gross total resection; ICU , intensive care unit; IOM , intraoperative monitoring; MAP , mean arterial pressure; MEP , motor evoked potential; MIS , minimally invasive surgery; MRI , magnetic resonance imaging; NCS , nerve conduction study; SSEP , somatosensory evoked potential; STR , subtotal resection.

Differential diagnosis

  • ▪

    Chordoma

  • ▪

    Chondrosarcoma

  • ▪

    Meningioma

  • ▪

    Myoepithelioma/myoepithelial carcinoma

  • ▪

    Glioma

  • ▪

    Metastatic tumor (mucinous adenocarcinoma and clear cell renal cell carcinoma)

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