|Year : 2020 | Volume
| Issue : 1 | Page : 118-122
Surgical management of a case of C2 vertebral chordoma via staged anterior and posterior approach
Nigil S Palliyil, Kedar Deogaonkar, Milind Sankhe
Department of Orthopaedics, P. D. Hinduja Hospital & Medical Research Centre, Mumbai, Maharashtra, India
|Date of Submission||19-Nov-2018|
|Date of Decision||10-Apr-2019|
|Date of Acceptance||05-Sep-2019|
|Date of Web Publication||05-Feb-2020|
Dr. Nigil S Palliyil
Dr. Nigil S Palliyil, Department of Orthopaedics, P. D. Hinduja Hospital & Medical Research Centre, Veer Savarkar Marg, Mahim West, Mumbai-400016, Maharashtra.
Source of Support: None, Conflict of Interest: None
Atlantoaxial chordomas being quite uncommon pose a significant therapeutic challenge to the surgeon due to their critical location and often late presentation. Recurrences are common after intralesional excision. Although en bloc excision is the preferred treatment, it may not be feasible due to anatomical constraints in this location. Hence, multimodality treatment in the form of surgery (maximal tumor excision) followed by targeted chemotherapy and radiotherapy is considered to be the next best treatment option. We present the case of a young male patient with C2 chordoma treated at our institution by multimodality approach, and follow-up for 21 months after surgery.
Keywords: Atlantoaxial chordoma, C2 chordoma, multimodality approach to chordoma
|How to cite this article:|
Palliyil NS, Deogaonkar K, Sankhe M. Surgical management of a case of C2 vertebral chordoma via staged anterior and posterior approach. Indian Spine J 2020;3:118-22
|How to cite this URL:|
Palliyil NS, Deogaonkar K, Sankhe M. Surgical management of a case of C2 vertebral chordoma via staged anterior and posterior approach. Indian Spine J [serial online] 2020 [cited 2021 Jan 19];3:118-22. Available from: https://www.isjonline.com/text.asp?2020/3/1/118/277810
| Introduction|| |
Chordomas are uncommon malignant bone tumors with an incidence of 0.1 per 100,000 per year. They are slow growing malignant tumors arising from embryonic rest cells of the notochord. Chordoma most commonly arise from the sacrum (60%), followed by skull base (25%–30%). The mean age of the affected population is 60 years. However, skull base chordomas can affect younger population. Atlantoaxial chordomas are quite uncommon, posing a significant therapeutic challenge to the surgeon due to their critical location and often late presentation. Recurrences are common after intralesional excision; however, en bloc excisions are not feasible many a times due to anatomical constraints in this location. Hence, multimodality treatment in the form of surgery (maximal tumor excision) followed by targeted chemotherapy and radiotherapy is considered to be the only feasible option. We present a case of a young male patient with C2 chordoma treated at our institution by multimodality approach.
| Case Presentation|| |
A 39-year-old male patient presented with axial neck pain of seven-month duration, associated with occasional paraesthesia over left supraclavicular region, shoulder, and suboccipital region. On physical examination, torticollis was present. Tenderness was noted over upper cervical spine. Rotations were terminally restricted due to pain. Neurological examination was normal.
Plain X-ray cervical spine revealed an ill-defined lytic lesion in C2 vertebral body [Figure 1]. Magnetic resonance imaging (MRI) of cervical spine showed an expansile T2 hyperintense lesion involving C2 body extending to involve left-side lamina and pedicle with extradural extension [Figure 2]. Left vertebral artery and C3 nerve root were noted to be encapsulated within the tumor mass. Anterior extension of lesion was also shown, displacing the left prevertebral muscles. Positron emission tomography–computed tomography (PET CT) scan showed no other lesion elsewhere. CT-guided biopsy of the lesion was performed, with histopathological examination suggestive of chordoma. The tumor was classified according to the WBB (Weinstein–Boriani–Biagini) classification––layers A–D/sectors 1–6, Enneking stage IB.
|Figure 1: Plain X-ray cervical spine showing an ill-defined lytic lesion in C2 vertebral body|
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|Figure 2: T2-weighted sagittal and axial MRI cuts showing an expansile hyperintense lesion involving C2 body extending to involve left-side lamina and pedicle with extradural extension|
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| Procedure|| |
Total en bloc excision was not feasible in this case, considering the large tumor volume, extension in to the soft tissues and spinal canal, encasement of left vertebral artery, and left C3 nerve root. Preoperative angiography was carried out, which revealed that the left vertebral artery was dominant. Trial selective balloon occlusion of the left vertebral artery was performed. The patient remained asymptomatic after balloon occlusion test, suggesting that the collateral circulation was adequate and the left vertebral artery could be sacrificed if needed. We supposed that we would attempt to preserve the vertebral artery, especially as late-onset cerebrovascular accidents can occur following permanent ligation. This is not picked up by trial balloon occlusion test. Intralesional maximal tumor excision followed by adjuvant chemotherapy and IMRT (intensity-modulated radiotherapy) was planned.
First stage (POSTERIOR APPROACH). Through a posterior midline incision, occiput to C5 was exposed. Occipitocervical instrumentation was performed followed by C2 laminectomy. Left vertebral artery was identified and protected. Left C3 nerve root was identified and preserved. Soft and friable tumor tissue was clearly visualized. Tumor tissue was found to be soft and friable. Maximum tumor debulking was performed from the posterior aspect. Most of the tumor anteriorly that could be accessed from posterior aspect was removed. Occipitocervical instrumented fusion was completed. Iliac crest graft was overlaid on the fusion bed.
Interim MRI was carried out, which showed residual tumor in the anterior aspect of C2 body [Figure 3]. Hence, decision was taken to perform the second-stage surgery through the anterior approach.
Second stage (ANTERIOR APPROACH). It was performed six weeks later. Anterior high cervical retropharyngeal approach was used via a transverse skin crease incision. Hypoglossal nerve was identified and protected. MRI-based navigation was used to delineate the tumor. Tumor excision was completed. Navigation was used to confirm the margins of resection. Following the tumour excision, the anterior defect was reconstructed using iliac crest tricortical strut graft, snugly fitting it between C1 anterior arch and C3 body, followed by anterior cervical plating for support [Figure 4].
|Figure 4: Immediate postoperative X-ray showing anterior reconstruction using iliac crest strut graft and plating. Posteriorly occipitocervical fixation seen|
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No neurological deficits were noted postoperatively. Patient was mobilized out of bed from third postoperative day. Patient received 30 sessions of IMRT, along with oral imatinib mesylate 400mg daily. No local recurrence or metastasis has been reported till final follow-up at 21 months [Figure 5A]–[D] and [Figure 6].
|Figure 5: 1.5 years follow-up of: (A) X-ray showing sound anterior and posterior fusion, (B) CT scan, axial section showing union of the graft fragment, (C) CT scan, sagittal section showing union of the graft fragment, and (D) CT scan, coronal section showing union of the graft fragment|
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| Discussion|| |
Chordomas are slow-growing tumors, with metastasis occurring late, and mortality being directly related to local tumor progression. Chordomas of the mobile spine are best managed by total en bloc excision to prevent local recurrence and to offer possible cure. The feasibility of aggressive resections and achieving favorable oncological margins in atlantoaxial region is, however, limited due to a number of anatomical factors including (1) the proximity of the vertebral arteries, (2) the difficulty in cutting the cervical pedicles, (3) encasement of cervical nerve roots, (4) prevertebral muscle invasion. Since the epidural space at atlantoaxial level is quite voluminous, patients often present late. Most patients with atlantoaxial chordomas present at Enneking stage IB at the time of diagnosis, en bloc resection in which case would mean resection of paravertebral muscle, nerve root, vertebral artery, even dura or retropharyngeal mucosa, which is practically impossible. In addition, if the vertebral artery is ligated, it can result in significant neurological deficits, particularly if the dominant vertebral artery is sacrificed with insufficient collateral flow.
Hence, various treatment approaches have been suggested for atlantoaxial chordomas, which includes radiotherapy alone, combined radiotherapy and surgical excision, surgical excision alone. However, surgery remains the mainstay of treatment. Conventionally, chordomas were considered resistant to chemotherapeutic agents. Recently, imatinib (tyrosine kinase inhibitor) has been shown to possess antitumor activity in chordomas. With the advent of newer radiation modalities such as proton beam therapy, IMRT, it is possible to deliver higher doses of radiation to the tumor site with minimal dose to the surrounding vital structures.,
Most authors recommend maximal tumor excision followed by high-dose radiation for tumors located at atlantoaxial region, which has a reasonable morbidity-effectiveness ratio as compared with radical excision procedures.,
Higher incidence of perioperative complications, morbidity, contaminated margins, less favorable outcomes, and increased chance of recurrence have been reported with C1–C2 en bloc excisions.
In upper cervical chordomas, intralesional resection with adjuvant radiation therapy is associated with a 40% recurrence and 67% mortality rate at five postoperative years.
On the contrary, the incidence of postoperative complications following en bloc resection was (54%), compared with intralesional excision (11%). However, according to this study, 92% chordoma recurrences and all chordoma metastases occurred in patients with intralesional resection.
Survival outcomes were mainly influenced by the degree of the first surgical resection, and hence, en bloc resection (wide or marginal) of chordomas is associated with decreased recurrence and mortality rates as compared with intralesional resection where there is breaching of the tumor capsule. However, these authors also described that en bloc resections for C1–C2 tumors were more likely to have violated margins as compared with resection for subaxial (C3–C7) tumors.
In our case, we have a follow-up period of 21 months till now without any tumor recurrence. We understand that the follow-up period is not long enough to draw a meaningful conclusion in terms of tumor recurrence especially in a slow-growing tumor such as chordoma. However, we would suggest multimodality approach (intralesional maximal tumor excision followed by IMRT or proton beam radiotherapy and chemotherapy [imatinib mesylate]), as a plausible option for these challenging atlantoaxial tumors, when en bloc excision is impossible or fraught with severe postoperative morbidity.
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]