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|LETTER TO EDITOR
|Year : 2019 | Volume
| Issue : 6 | Page : 1545-1548
Intradural Invasion of Cervical Chordoma in a 34 Year Old Patient
Huibing Ruan1, Ratnesh N Mehra2, Daehyun Park3, Dean Chou2
1 Department of Orthopedics, The Fourth Affiliated Hospital of Nanchang University, Nanchang, China; Department of Neurosurgery, University of California San Francisco, San Francisco, California, United States of America
2 Department of Neurosurgery, University of California San Francisco, San Francisco, California, United States of America
3 Department of Neurosurgery, University of California San Francisco, San Francisco, California, United States of America; Department of Orthopedics Inje University Busan Paik Hospital, Busan, Korea
|Date of Web Publication||20-Dec-2019|
Dr. Huibing Ruan
505 Parnassus Ave, Box 0112, San Francisco, CA - 94143
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Ruan H, Mehra RN, Park D, Chou D. Intradural Invasion of Cervical Chordoma in a 34 Year Old Patient. Neurol India 2019;67:1545-8
Chordomas are rare and aggressive primary bone tumors. They are slow growing, malignant tumors that arise from embryonal remnants of the primitive notochord. Chordomas make up 1% to 4% of all primary malignant bone tumors, and 10% of chordomas are found in the cervical spine., Other common locations are the skull base and sacrococcygeal region. Prognosis remains poor secondary to vital structure invasion (nerve roots, spinal cord, vessels) and to propensity for local recurrence. The surgical goal is en bloc resection for the most favorable outcomes and disease-free intervals., However, nearby critical structures often make en bloc resection challenging, particularly in the cervical spine where essential nerve roots and the vertebral arteries are often involved. Cases of successful multilevel spondylectomy for en bloc excision do exist. However, true en bloc spondylectomy is not often possible, and parasagittal osteotomies may be needed to achieve en bloc tumor excision.,, Because they are tumors that originate in the spinal column, they rarely invade the dura. However, we present a technically challenging case of cervical spine chordoma with intradural invasion in a 34 year-old patient with video showing the intradural involvement of the tumor.
A 34-year-old female presented with progressive loss of balance, gait disturbance, left-sided neck pain, loss of bilateral upper extremity dexterity, and urinary urgency. Her past medical history included developmental delay, obesity, diabetes mellitus and an inflammatory pituitary mass resected during adolescence. Her neurologic examination revealed a shuffling gait, bilateral paresis in the intrinsic hand muscles, tibialis anterior, extensor hallicus longus, and gastrocnemius muscle groups. Magnetic Resonance Imaging (MRI) of the cervical spine revealed a multilobular, T2 hyperintense heterogenously enhancing cystic epidural mass, ventral to the cord at C2-3. This extended into the left neural foramen, foramen transversarium, and prevertebral space with compression of the cervical spinal cord [Figure 1]. Computerized Tomography (CT) revealed expansion and remodeling of the C2, C3, and C4 central canal and foramina. CT angiography revealed the left vertebral artery (VA) to be dominant, and both VAs to be patent [Figure 2]. MRI of the entire neuroaxis did not reveal any additional lesions.
|Figure 1: Pre-operative MRI demonstrating cystic epidural mass ventral to the cord at C2-3 with extension to the left neural foramen and foramen transversarium. Figures 1a and 1b: T2W MRI. Figures 1c and 1d: post-gadolinium MRI|
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|Figure 2: Pre-operative CT Angiogram demonstrating the bony destruction of the tumor at C2 and C3, with patency of both vertebral arteries, as seen in the axial (a), coronal (b), and sagittal (c) planes|
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A CT guided biopsy was performed demonstrating chordoma. Discussion was held with the patient and a surgical plan for circumferential approach and en bloc excision was made. Because the patient's tumor was on the left and her left VA was dominant, left VA sacrifice was not an option. Thus, dissection of the left VA away from the tumor with parasagittal osteotomy was planned. First, a left anterior approach to the cervical spine was performed [Figure 3]. Parasagittal osteotomies were performed at C2 and C3; a midline cut was made in the vertebral body of C3, and a C2 cut was made disconnecting the odontoid tip and C1 complex from the body. The left vertebral artery was carefully skeletonized away from the tumor. An anterior cervical discectomy was performed at C3-4. The patient was allowed to recover in the hospital, and the posterior stage was planned for another day during the same hospitalization.
|Figure 3: Intraoperative photograph demonstrating the exposed anterior cervical spine after a left-sided approach and performing the anterior parasagittal osteotomy|
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During the second stage, a standard posterior midline incision was used for exposure of the occiput down to C7 [Figure 4]. An occipital plate was placed in addition to lateral mass screws down to C7 (skipping some segments secondary to bony anomalies and for planned tumor excision). A laminectomy was performed from C2 to C4 to expose the tumor; these posterior elements did not have tumor involvement. It was apparent at this point that there was intradural invasion of the tumor along the C3 nerve root. It was not possible to perform en bloc resection of the intradural portions of tumor. However, we pulled the tumor out as much as possible before ligating the C3 nerve root. The left VA lateral to the C2 and C3 bodies was identified with Doppler, and it was carefully dissected away. The left C2 and C4 nerve roots were also sacrificed in order to achieve en bloc excision of the extradural portions of the tumor. Utilizing the previously performed anterior parasagittal osteotomies, the tumor and vertebral bodies of C2 and C3 were carefully rolled en bloc laterally and dorsally through the corridor between the left VA and the spinal cord. No cord injury or left VA injury occurred during this maneuver, and there were no changes to the neuromonitoring signals. Under microscopic inspection, there was no remaining extradural tumor. Due to the large defect between the remnant of C2 and C4 ventrally, an expandable cage was posteriorly placed under fluoroscopic guidance to reconstruct the anterior spinal column. Posterior segmental instrumentation was completed with the rod and screw construct from the occiput to C7 [Figure 5].
|Figure 4: Intraoperative photograph demonstrating the posterior cervical spine with the occipital plate and lateral mass screws in place|
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|Figure 5: Post-operative standing radiographs in the lateral (a) and AP (b) dimensions, demonstrating the circumferential reconstruction of the spinal column|
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Histopathology demonstrated vacuolated cells in chord-like clusters with scattered physaliphorous cells. Evidence of osteonecrosis with pathologic fracture and invasion into the bone was present. Immunohistochemical staining revealed positive Brachyury in the tumor nuclei and S100 in the nuclei and cytoplasm. Cytologic and immunohistochemical diagnosis confirmed the tumor to be chordoma.
Post-operatively, the patient was extubated and discharged to rehabilitation, and eventually, home. She had an improvement in her lower extremity and intrinsic hand strength at time of discharge. She stated that her neck and radicular pain had resolved and she was ambulating without assistance. We have 8 months of follow-up data. A post-operative MRI showed good decompression of the spinal cord and no evidence of gross tumor [Figure 6]. Proton beam therapy after 12 months was recommended to allow for fusion.
|Figure 6: Post-operative MRI demonstrating good decompression of the spinal cord on T2W images in the sagittal (a) and axial (b) planes, and no residual enhancing mass with gadolinium administration in the sagittal (c) and axial (d) planes|
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Cervical chordomas can be challenging surgical cases secondary to their location and involvement of vital structures. It has been established that en bloc excision for chordoma is the optimal treatment for long-term local control compared to intralesional resection.,, Excision via total en bloc spondylectomy may require sacrifice of the VA or cervical nerve roots, and careful surgical planning needs to be undertaken. Pre operative discussion with the patient regarding potential neurologic sequelae and devastating complications need to be had. In addition, baseline neurologic status assessment and angiography are helpful tools that should be considered. In most cases, sacrifice of C1-4 nerve roots can be done safely with little neurologic sequelae, as was done in this case; although the patient should be warned of a possible post operative hemidiaphragm. VA sacrifice was not performed in our case secondary to the left vertebral artery dominance. The VA was safely preserved during the en bloc resection of the extradural tumor, and no negative neurologic or respiratory effects were seen from unilateral C2 to C4 nerve root sacrifice. Although single level spondylectomies and corpectomies can be performed in the absence of VA involvement, lateralized tumors that span multiple levels and encase the VA may present a greater challenge. The described technique for making multiple anterior parasagittal osteotomies prior to posterior en bloc extradural excision was utilized in our case. It is important to keep in mind that no tumor was removed or encountered during the first stage. The bony and ligamentous structures around the affected vertebral bodies were disconnected and the VA was skeletonized during the first stage. This allowed the tumor to be excised completely from a posterior approach.
Intraoperatively, it was discovered that this tumor demonstrated intradural invasion. This was difficult to assess based on pre operative imaging because of the severe cord compression, and it was an unexpected finding. Intradural chordomas are difficult to distinguish from neurogenic tumors, such as schwannomas or neurofibromas, especially when they extend into the foramina and take on a dumbbell shape. It is known that chordomas arise from persistent notochordal rests, which are intraosseous. Thus, chordomas of the spine are generally found in the bone and not in the intradural space.
Intradural invasion by chordomas creates a challenging problem and makes true en bloc resection rarely feasible. Zhang et al. reported a case of an intradural chordoma with diffuse leptomeningeal spread. They performed a literature review and also found 12 reported cases of intradural spinal chordoma. They make a distinction between chordomas with an intradural component (such as this case), and those that are purely extraosseous. Their reported cases were all purely extraosseus. Kirschenbaum and Yang reported a cervical spinal chordoma with intradural invasion in 1983. Although they performed a corpectomy for lesional excision and reconstruction of the anterior spinal column, our case required a more complex approach for complete excision due to the VA involvement and extent of tumor. Regardless of whether the chordoma is purely extraosseous or not, intradural spread can lead to cerebrospinal fluid seeding and dissemination; which need to be monitored closely in follow-up.
This is a technically challenging case of cervical chordoma with intradural extension. It required complex surgical management with parasagittal osteotomies, VA skeletonization, nerve root sacrifice, reconstruction of the anterior spinal column from a posterior approach. In our case, the patient was only 34 years old. Thus, unlike traditional chordomas, which may have been present for much longer in older patients, this chordoma most likely represented a more aggressive subtype in a younger patient.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
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