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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 2  |  Issue : 2  |  Page : 179-183

A rare case of giant cell tumor of body of axis: Surgical management with staged posterior occipitocervical fusion, anterior excision and reconstruction through anterior mandibulotomy


1 Department of Spine, Stavya Spine Hospital and Research Institute, Ahmedabad, Gujarat, India
2 Department of Orthopaedics, IMS and SUM Hospital, Bhubaneswar, Odisha, India

Date of Web Publication23-Jul-2019

Correspondence Address:
Dr. Devanand Degulmadi
Stavya Spine Hospital and Research Institute, Ahmedabad, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/isj.isj_21_19

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  Abstract 


Giant cell tumor (GCT) constitutes around 5% of all the skeletal tumors which usually occur between second and fourth decade. Cervical spine GCT is very rare, and only a few case reports have been reported. In view of complex anatomy, variable aggressiveness, and scanty literature, there is a lack of clear consensus in the evaluation and management of high cervical GCT. We present a 30-year-old male patient, a case of GCT involving C2 vertebral body with severe neck pain, C1–C2 instability, and neurological deficit (visual analog scale [VAS] - 10/10, Nurick Grade-IV, NDI - 97.7%) managed by staged procedures. Posterior occipitocervical stabilization followed by anterior corpectomy, intralesional excision of the mass through anterior mandibulotomy, and reconstruction with iliac crest bone graft was performed. Postoperatively, the patient received adjuvant Denosumab therapy for 6 months. Complete neurological improvement was seen by 3 months. The VAS and NDI scores were 1/10 and 11.1%, respectively, at 1-year follow-up. No radiological recurrence was seen on radiograph and magnetic resonance imaging at 2-year follow-up. C2 GCT managed by intralesional excision and global stabilization combined with Denosumab therapy provides good clinical improvement without recurrence on medium-term follow-up. We believe that extended transoral transmandibular approach provides an excellent wide field for excision of high cervical aggressive tumors.

Keywords: Anterior mandibulotomy, axis vertebra, Denosumab, extended transoral transpharyngeal approach, giant cell tumor


How to cite this article:
Dave BR, Behera G, Krishnan A, Degulmadi D. A rare case of giant cell tumor of body of axis: Surgical management with staged posterior occipitocervical fusion, anterior excision and reconstruction through anterior mandibulotomy. Indian Spine J 2019;2:179-83

How to cite this URL:
Dave BR, Behera G, Krishnan A, Degulmadi D. A rare case of giant cell tumor of body of axis: Surgical management with staged posterior occipitocervical fusion, anterior excision and reconstruction through anterior mandibulotomy. Indian Spine J [serial online] 2019 [cited 2019 Aug 21];2:179-83. Available from: http://www.isjonline.com/text.asp?2019/2/2/179/263274




  Introduction Top


Giant cell tumor (GCT) of high cervical spine is very rare, and only few cases are reported in the literature. Complex anatomy of upper cervical spine, variable aggressive nature of GCT, and high recurrence rates are peculiar features.[1] Anterior approach to the upper cervical spine is a challenging task because of anatomical complexity and proximity of important neurovascular structures.[2] En bloc resection with tumor-free margins is not always possible in cases of high cervical spine.[3] We discuss a case of GCT involving body of axis vertebra with neurological impairment, surgically managed by staged posterior and anterior approach with a brief review of the literature.


  Case Report Top


A 30-year-old male presented to the outpatient department with a 4 months' history of severe neck pain, bilateral upper and lower limb weakness. Progressive weakness with gradual difficulty in independent ambulation was noticed by the patient over a period of 2 weeks. Bowel and bladder habits were normal. Sleep was disturbed because of pain. On physical examination, he was supporting his head by both the hands over the chin and was not able to walk without support (visual analog scale [VAS] - 10/10, Nurick Grade-IV, NDI - 97.7%). He had bilateral positive Hoffman's sign, positive Babinski sign, and hyperreflexia of all the four limbs. Anteroposterior open mouth plain radiograph of the cervical spine showed ill-defined lytic lesion involving the body of C2, and lateral flexion-extension views showed instability at C1–C2 [Figure 1]a, [Figure 1]b, [Figure 1]c. Computed tomography (CT) scan depicted an expansile lytic lesion involving the body of the axis and sparing the dens [Figure 2]a, [Figure 2]b, [Figure 2]c. CT angiography showed normal vertebral arteries. Magnetic resonance imaging (MRI) of the cervical spine showed heterogeneous mixed intensity on T2 and homogeneous, isointense expansile lesion on T1 images [Figure 3]a, [Figure 3]b, [Figure 3]c. Differentials considered were aneurysmal bone cyst (ABC), GCT, and tuberculosis of body of axis. Transoral CT-guided biopsy report was suggestive of GCT. As per Campanacci system of classification, the GCT was staged as an intraosseous lesion with cortical thinning and expansile borders (Grade II).[4]
Figure 1: (a-c): Anteroposterior and lateral (flexion-extension) radiographs of cervical spine showing ill-defined lytic lesion involving the body of C2 and instability at C1–C2. (d and e): Postoperative X-ray showing posterior occipitocervical fixation with occipital plate and lateral mass screws

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Figure 2: (a-c): Computed tomography scan of cervical spine showing an expansile lytic lesion involving the body of the axis sparing the dens. (d and e): Postoperative computed tomography scan demonstrating reconstruction of anterior column with iliac crest bone graft and cervical buttress plate along with the remnant of the odontoid process

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Figure 3: (a-c): Magnetic resonance imaging of the cervical spine showing heterogeneous mixed intensity on T2 and homogeneous, isointense expansile lesion on T1 images

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Staged posterior and anterior procedure was planned. In the first stage, posterior occipitocervical fusion (O-C5) was done with occipital plate and cervical lateral mass screws [Figure 1]d and [Figure 1]e. One week later, the second stage of surgery was planned after tracheostomy. Multidisciplinary approach was adopted with maxillofacial surgeons and spine surgeons. Through an extended transoral transpharyngeal approach and anterior mandibulotomy, intralesional excision of the tumor mass was performed in piecemeal [Figure 4]a, [Figure 4]b, [Figure 4]c, [Figure 4]d. The tumor mass was highly vascular, friable, and soft in consistency. Reconstruction with tricortical iliac crest bone graft and anterior plate was done retaining the normal odontoid process [Figure 2]d, [Figures 2]e and [Figure 4]e, [Figure 4]f, [Figure 4]g, [Figure 4]h, [Figure 4]i. Histopathology of the tumor mass confirmed GCT. Postoperatively, Philadelphia collar was used for 3 months. He was advised monthly subcutaneous Denosumab 120 mg along with daily supplements of calcium (1000 mg) and Vitamin D (400 IU). Complete neurological improvement was seen by 3 months and the patient started walking independently. The VAS and NDI scores improved to 1/10 and 11.1%, respectively at 1-year follow-up. No radiological recurrence was seen on radiograph and MRI at 2-year follow-up [Figure 5]a, [Figure 5]b, [Figure 5]c, [Figure 5]d, [Figure 5]e.
Figure 4: (a-i) Intraoperative picture showing anterior mandibulotomy and extended transoral transpharyngeal approach, intralesional excision of the tumor mass, and reconstruction of anterior column with iliac crest bone graft and cervical buttress plate

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Figure 5: (a-e): No evidence of recurrence of giant cell tumor on radiograph and magnetic resonance imaging at 2-year follow-up

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  Discussion Top


GCT is a benign but locally aggressive bone tumor with high recurrence rates.[1] Excluding sacrum, GCT of the spine is rare, and only 2–3% cases affect the spine above sacrum.[5] The incidence in cervical spine is still rare (<1%).[6] Pain and different grades of neurological deficits are usual presentations in upper cervical GCTs. In their case series of five patients with axial vertebra GCTs, Chen et al. reported that all cases had different grades of upper neck pain, paresthesias, and features of cord compression such as shaky gait, hypertonia, hyperreflexia, positive Babinski and positive Hoffman's sign.[7] Our patient presented with a typical posture of restricted neck movements by holding at chin that is observed in patients with upper cervical pathology. All the signs of cervical myelopathy were present that clinically support a high cervical lesion.

Radiographs show expansile lytic lesions often with destroyed cortices and soft tissue mass. CT has a characteristic soap bubble appearance and also gives a clear idea about bony cortices.[8] On MRI, GCTs show heterogeneous signal intensity, low to intermediate signal intensity on T1 images, and low to similar intensity to the spinal cord on T2 images.[9] Since GCT does not have specific MR imaging features, differentials such as ABC, chondroblastoma, brown tumor of hyperparathyroidism, and secondary metastasis should be considered.[8] In the present case, radiographs revealed C1-2 instability and expansile lytic lesion in C2 vertebral body. CT and MRI added details to the pathology that helped us in planning the surgery. Primarily, posterior surgery was planned in view of listhesis of C1 over C2. Total en bloc resection with tumor-free margins appears to be the best standard in providing continuous disease-free course with excellent clinical outcome.[1] However, due to relatively complex anatomical structure of the upper cervical spine and proximity to neurovascular structures, an en-bloc resection of the mass is very difficult to accomplish. Extended transoral approach with mandibulotomy provides a direct wider surgical field for upper cervical spine mass excision as demonstrated by Ortega-Porcayo et al. in their series of two cases of high cervical spine (C2, C2–C4) chordoma [Table 1].[10] Chen et al. reported their case series of 5 cases of axis vertebra GCT treated by intralesional curettage, reconstruction with bone graft, anterior and posterior spinal instrumentation [Table 1].[7] Preservation of the odontoid process is important in terms of anatomy and functional preservation of atlantoaxial vertical joint.
Table 1: Comparison of similar reported cases of high cervical spine tumorous conditions (giant cell tumor, chordoma), surgical management, and outcome

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In case of failure of total excision, the local recurrence rate was supposed to be very high (25%–45%), demanding careful management.[11] Adjuvant radiotherapy should be considered in such cases.[12] However, a risk of sarcomatous transformation is reported, and thus, it should be reserved for patients with incomplete excision.[13] Goldschlager et al. in a multicentric prospective series of 5 patients having GCT of the spine treated with Denosumab demonstrated a clinically beneficial radiological and histological response in most except one.[14] Our patient was advised monthly Denosumab injection for 6 months. There was complete neurological improvement by 3 months. No local recurrence or implant-related issues were noticed at the end of 2-year follow-up [Figure 5]a-e].[15]


  Conclusion Top


The present case shows a successful management of C2 GCT by intralesional excision and global stabilization combined with Denosumab therapy with good clinical improvement and no radiological recurrence at 2-year follow-up. We believe that extended transoral transmandibular approach provides an excellent wide field for excision of high cervical aggressive tumors. Both anterior and posterior cervical fixation in upper cervical GCT allows early ambulation.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Boriani S, Bandiera S, Casadei R, Boriani L, Donthineni R, Gasbarrini A, et al. Giant cell tumor of the mobile spine: A review of 49 cases. Spine (Phila Pa 1976) 2012;37:E37-45.  Back to cited text no. 1
    
2.
Cheung JP, Luk KD. Complications of anterior and posterior cervical spine surgery. Asian Spine J 2016;10:385-400.  Back to cited text no. 2
    
3.
Fisher CG, Keynan O, Boyd MC, Dvorak MF. The surgical management of primary tumorsof the spine: Initial results of an ongoing prospective cohort study. Spine (Phila Pa 1976) 2005;30:1899-908.  Back to cited text no. 3
    
4.
Campanacci M. Giant cell tumor. In: Campanacci M, Enneking WF, editors. Bone and Soft Tissue Tumors: Clinical Features, Imaging, Pathology and Treatment. 2nd ed. New York: Springer-Verlag; 1999. p. 99-136.  Back to cited text no. 4
    
5.
Abdelwahab IF, Camins MB, Hermann G, Klein MJ, Mosesson RE, Casden AM, et al. Giant cell tumour of the seventh cervical vertebra. Can Assoc Radiol J 1995;46:454-7.  Back to cited text no. 5
    
6.
Bidwell JK, Young JW, Khalluff E. Giant cell tumor of the spine: Computed tomography appearance and review of the literature. J Comput Tomogr 1987;11:307-11.  Back to cited text no. 6
    
7.
Chen G, Li J, Li X, Fan H, Guo Z, Wang Z. Giant cell tumor of axial vertebra: Surgical experience of five cases and a review of the literature. World J Surg Oncol 2015;13:62.  Back to cited text no. 7
    
8.
Shi LS, Li YQ, Wu WJ, Zhang ZK, Gao F, Latif M. Imaging appearance of giant cell tumour of the spine above the sacrum. Br J Radiol 2015;88:20140566.  Back to cited text no. 8
    
9.
Kwon JW, Chung HW, Cho EY, Hong SH, Choi SH, Yoon YC, et al. MRI findings of giant cell tumors of the spine. AJR Am J Roentgenol 2007;189:246-50.  Back to cited text no. 9
    
10.
Ortega-Porcayo LA, Cabrera-Aldana EE, Arriada-Mendicoa N, Gómez-Amador JL, Granados-García M, Barges-Coll J. Operative technique for en bloc resection of upper cervical chordomas: Extended transoral transmandibular approach and multilevel reconstruction. Asian Spine J 2014;8:820-6.  Back to cited text no. 10
    
11.
Hart RA, Boriani S, Biagini R, Currier B, Weinstein JN. A system for surgical staging and management of spine tumors. A clinical outcome study of giant cell tumors of the spine. Spine (Phila Pa 1976) 1997;22:1773-82.  Back to cited text no. 11
    
12.
Caudell JJ, Ballo MT, Zagars GK, Lewis VO, Weber KL, Lin PP, et al. Radiotherapy in the management of giant cell tumor of bone. Int J Radiat Oncol Biol Phys 2003;57:158-65.  Back to cited text no. 12
    
13.
Feigenberg SJ, Marcus RB Jr., Zlotecki RA, Scarborough MT, Berrey BH, Enneking WF. Radiation therapy for giant cell tumors of bone. Clin Orthop Relat Res 2003;411:207-16.  Back to cited text no. 13
    
14.
Goldschlager T, Dea N, Boyd M, Reynolds J, Patel S, Rhines LD, et al. Giant cell tumors of the spine: Has denosumab changed the treatment paradigm? J Neurosurg Spine 2015;22:526-33  Back to cited text no. 14
    
15.
Afsoun S, Saied SA, Amir N, Hamed J. En-bloc resection of a giant cell tumor causing cervical vertebral collapse. Asian J Neurosurg 2018;13:150-3.  Back to cited text no. 15
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