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CASE REPORT
Year : 2020  |  Volume : 13  |  Issue : 4  |  Page : 399-402  

Giant cell tumor of the cervico-dorsal junction with compressive myelopathy: A rare presentation with review of literature


1 Department of Neurosurgery, R and R Hospital, New Delhi, India
2 Department of Neurosurgery, Armed Forces Medical College, Pune, Maharashtra, India

Date of Submission24-May-2019
Date of Decision15-Aug-2019
Date of Acceptance15-Oct-2019
Date of Web Publication20-Jul-2020

Correspondence Address:
Vikas Maheshwari
Department of Neurosurgery, Armed Forces Medical College, Pune - 411 040, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.mjdrdypu_126_19

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  Abstract 


Giant cell tumor (GCT) of bone, although benign in its nature, is aggressive in its course. It has a predilection for the epiphysis of long bones. Although it can affect any vertebra in the body, it has propensity to affect the fixed spine more than the mobile segments. It occurs infrequently above the sacrum, and involvement of the cervical spine is still rarer. However, sparse case reports of upper cervical spine GCT have been reported in literature. We report a case of GCT involving the C7 and D1 vertebrae in a 50-year-old woman who presented with the features of compressive myelopathy. She underwent corpectomy of C7–D1 with cage fixation and cervical plating via an anterior approach. The patient showed significant neurological improvement postoperatively.

Keywords: Cervico-dorsal junction, corpectomy, giant cell tumor


How to cite this article:
Gill M, Maheshwari V, Mukherjee A, Gadhavi R. Giant cell tumor of the cervico-dorsal junction with compressive myelopathy: A rare presentation with review of literature. Med J DY Patil Vidyapeeth 2020;13:399-402

How to cite this URL:
Gill M, Maheshwari V, Mukherjee A, Gadhavi R. Giant cell tumor of the cervico-dorsal junction with compressive myelopathy: A rare presentation with review of literature. Med J DY Patil Vidyapeeth [serial online] 2020 [cited 2020 Dec 3];13:399-402. Available from: https://www.mjdrdypv.org/text.asp?2020/13/4/399/290156




  Introduction Top


Giant cell tumor (GCT) of bone, although a benign tumor, is aggressive in its nature.[1] It accounts for 5% of all primary bone tumors in adults and occurs most frequently in the ends of long bones.[2],[3] It occurs infrequently in the spine and has a predilection for the fixed spine. The mobile spine is affected in only 1.4%–9.4% of cases.[4] They rarely affect the spine above the sacrum, and their incidence in the cervical spine is still rarer.[5] They mostly occur in the third to fourth decades of life. Although they are locally malignant, metastasis can occur in up to 3% of these tumors. Lung is the most common site of metastasis.[6] They mostly involve the vertebral body; however, posterior elements can also be involved. Modalities of treatment are varied and range from intralesional excision to en bloc spondylectomy. Embolization and medical management with denosumab and bisphosphonates are also known to be effective.[7] GCT of the cervico-dorsal junction is unique in that it is not only rare in literature, but also a challenge to deal with surgically.[7] We report a case of GCT involving the C7 and D1 vertebrae in a 50-year-old woman who presented to us with paraparesis and underwent corpectomy of C7–D1 along cage fixation via an anterior approach. The patient showed significant neurological improvement postoperatively.


  Case Report Top


A 50-year-old woman presented with insidious-onset, gradually progressive pain over the left side of the chest and the back of the neck of 6 months' duration. She had progressively increasing weakness and paresthesias of bilateral lower limb (left > right) for 4 months. There was a history of urinary incontinence and constipation of 2 months' duration. She also had a history of weight loss of 5 kg over 2 months and intermittent fever. Clinically, she had increased tone in both lower limbs (Modified Ashworth Scale III). Power was Medical Research Council (MRC) Grade 2/5 in both lower limbs. Bilateral ankle and knee jerks were exaggerated. There was ankle clonus, and both plantars were extensor. There was 60%–70% sensory loss in C8, D1, and D2 dermatomes. X-ray of the cervico-dorsal spine showed lytic lesion of the cervico-dorsal junction. Noncontrast computed tomography cervico-dorsal spine with three-dimensional reconstruction revealed an expansile lytic lesion epicentered at the D1 vertebra with characteristic soap bubble appearance [Figure 1]a and [Figure 1]b.
Figure 1: (a) Noncontrast computed tomography cervico-dorsal spine sagittal cuts showing the lesion centered at D1, (b) characteristic soap bubble appearance (white arrow) in the axial section

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Contrast-enhanced magnetic resonance imaging (CEMRI) cervico-dorsal spine revealed well–defined, contrast-enhancing expansile lytic lesion causing complete collapse of the D1 vertebral body with severe cord compression [Figure 2]a and [Figure 2]b.
Figure 2: (a) Contrast-enhanced magnetic resonance imaging sagittal section showing contrast enhancing expansile lytic lesion measuring 40 mm × 56 mm × 39 mm involving the D1 vertebral body, (b) axial section showing the lesion causing severe cord compression involving the D1 vertebral body

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Anterior approach to cervicothoracic junction was done by right-sided Smith–Robinson approach with skin incision reaching up to the sternal notch[8] [Figure 3]. The sternomastoid, sternohyoid, and sternothyroid muscles were sectioned, allowing a connection of the lower cervical region to the upper thoracic spine. Through this approach, T2 is readily accessible in 88% of cases and T1 in almost all cases. The patient underwent corpectomy in the C7 and D1 vertebrae along with dural decompression. Intraoperatively, the tumor was bluish-red, was moderately vascular, was bony hard in consistency, and caused severe cord compression. Reconstruction was done with expandable cage, and anterior plating from C6 to D2 was done [Figure 4].
Figure 3: Skin incision

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Figure 4: Postoperative radiograph showing the implant in situ

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The postoperative recovery was uneventful. At 4-week follow-up, her lower limb power improved to MRC grade 4/5. She regained her urinary continence and was ambulant with support. She was given 45 Gy for 5 days a week for 4 weeks, and two such cycles were given. At 3-month follow-up, she was ambulant without support. Her histopathology report showed GCT Grade I (Jaffe et al.) [Figure 5]. Postoperative CEMRI showed gross total excision of tumor with no epidural compression [Figure 6].
Figure 5: Photomicrograph of the lesion (H and E, ×400) showing multinucleated osteoclast giant cells (white arrow) in a background stroma of mononuclear spindle cells (black arrows)

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Figure 6: Postoperative magnetic resonance imaging sagittal section showing gross total excision with expanded cord

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There has been no locoregional recurrence at 1-year follow-up.


  Discussion Top


GCT is known to affect the epiphysis of the long bones in the younger population. There can be infrequent involvement of spine, and literature mentions sacrum as the most common site.[9] Involvement of the spine above the sacrum is rare and that of the cervical spine is still rarer as was seen in our case.[9] GCTs, although are benign, slow-growing tumors, can have an aggressive course. They occur more commonly in the third and fourth decades of life and are more common in women.[10] Our patient, though a female, was in her fifth decade. GCT of the spinal region can present with localized pain, weakness, and paresthesias due to compression of the nerve roots or features of myelopathy. Our patient had the features of myelo-radiculopathy along with severe bony pain. Histologically, an abundance of mononuclear stromal cells and multi-nucleated giant osteoclast-like cells distributed evenly in the tissue characterizes these tumors. Although GCT is a benign tumor, 1%–9% of cases can undergo malignant transformation.[11],[12],[13],[14] Distant metastasis can occur in 2%–9% of cases, with lung being the most common site.[15] Radiologically, these need to be distinguished from aneurysmal bone cyst, Brown tumor of hyperparathyroidism, osteoid osteoma, and plasmocytoma.[11],[16] Surgical options include radical en bloc resection or intralesional curettage tailored according to the nature of the lesion and the proximity of the surrounding vital structures. Intralesional curettage can be done for low-grade lesions; however, high-grade lesions require en bloc resection.[6]En bloc spondylectomy can be done at cervical region. However, the cervico-thoracic region entails special challenges and en bloc spondylectomy may not be technically feasible in all cases.

There is no clear evidence in literature of the benefit of en bloc resection over intralesional resection along with adjuvant treatment in regions with difficult access. Conservative resection of GCT of the spine has been recommended by various authors with satisfactory results. Jaffe et al. proposed intralesional resection followed by radiation to be an acceptable form of management in GCTs of the cervical spine.[17] According to Hart et al. in tumors extending into the posterior elements or into soft tissues as seen in our case, achieving en bloc removal is more difficult with higher recurrence rates.[10] In our case, en bloc spondylectomy was not feasible as the tumor had already breached the capsule and was causing compression of the C7 and D1 nerve roots with significant epidural extension. Gross total resection along with dural decompression and reconstruction was done in our patient. She was given adjuvant radiotherapy. Although radiotherapy is the preferred mode of adjuvant therapy, some authors doubt its effectiveness, whereas others believe that it could cause malignant transformation of GCT.[9],[10] Other adjuvant therapies in the form of cryotherapy, preoperative embolization, bone cement injections, biphosphonates, and denosumab have shown encouraging results. However, literature reviews showing their effectiveness are hardly extensive.[18],[19],[20] Cervico-thoracic junction is a rare site for GCT and while the inaccessibility of the region makes intralesional removal the most feasible option, it leaves a definite risk of recurrence of GCT. This requires radiation or other adjuvant modalities. While en bloc spondylectomy is ideal, neurological outcome of the patient should take precedence in the management of GCTs located in relatively difficult-to-access regions of the spine such as the cervico-thoracic region.


  Conclusion Top


The cervico-thoracic region is an uncommon location of a GCT with very few case reports in literature. The relative inaccessibility of the region makes it surgically challenging. Aggressive en bloc resection of the tumor remains a gold standard. Intralesional resection is a suitable alternative for multisegment GCTs of the cervico-thoracic region. For GCTs of this region, neurological outcome is of paramount importance and should not be sacrificed for the sake of en bloc resection. Adjuvant radiotherapy and a close follow-up is essential for the prevention of recurrence.

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.
Szmygin P, Kulesza B, Grochowski C, Litak J, Janusz W. Upper cervical spine giant cell tumour of the vertebra – Case report. J Pre Clin Clin Res 2016;10:133-5.  Back to cited text no. 1
    
2.
Mendenhall WM, Zlotecki RA, Scarborough MT, Gibbs CP, Mendenhall NP. Giant cell tumor of bone. Am J Clin Oncol 2006;29:96-9.  Back to cited text no. 2
    
3.
Turcotte RE, Sim FH, Unni KK. Giant cell tumor of the sacrum. Clin Orthop Relat Res 1993;291:215-21.  Back to cited text no. 3
    
4.
Turcotte RE. Giant cell tumor of bone. Orthop Clin North Am 2006;37:35-51.  Back to cited text no. 4
    
5.
Hunter CL, Pacione D, Hornyak M, Murali R. Giant-cell tumors of the cervical spine: Case report. Neurosurgery 2006;59:E1142-3.  Back to cited text no. 5
    
6.
Eckardt JJ, Grogan TJ. Giant cell tumor of bone. Clin Orthop Relat Res 1986;204:45-58.  Back to cited text no. 6
    
7.
Mahajan R, Chhabra HS, Tandon V, Venkatesh R. Giant cell tumor of cervicothoracic region treated by triple corpectomy from posterior only approach: A case report with review of literature. J Craniovertebr Junction Spine 2015;6:212-5.  Back to cited text no. 7
    
8.
Huang YX, Ni WF, Wang S, Xu H, Wang XY, Xu HZ, et al. Anterior approaches to the cervicothoracic junction: A study on the surgical accessibility of three different corridors based on the CT images. Eur Spine J 2010;19:1936-41.  Back to cited text no. 8
    
9.
Sanjay BK, Sim FH, Unni KK, McLeod RA, Klassen RA. Giant-cell tumours of the spine. J Bone Joint Surg Br 1993;75:148-54.  Back to cited text no. 9
    
10.
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. 10
    
11.
Bertoni F, Present D, Sudanese A, Baldini N, Bacchini P, Campanacci M. Giant-cell tumor of bone with pulmonary metastases. Six case reports and a review of the literature. Clin Orthop Relat Res 1988;237:275-85.  Back to cited text no. 11
    
12.
Pai SB, Lalitha RM, Prasad K, Rao SG, Harish K. Giant cell tumor of the temporal bone – A case report. BMC Ear Nose Throat Disord 2005;5:8.  Back to cited text no. 12
    
13.
Siebenrock KA, Unni KK, Rock MG. Giant-cell tumour of bone metastasising to the lungs. A long-term follow-up. J Bone Joint Surg Br 1998;80:43-7.  Back to cited text no. 13
    
14.
Werner M. Giant cell tumour of bone: Morphological, biological and histogenetical aspects. Int Orthop 2006;30:484-9.  Back to cited text no. 14
    
15.
Lackman RD, Khoury LD, Esmail A, Donthineni-Rao R. The treatment of sacral giant-cell tumours by serial arterial embolisation. J Bone Joint Surg Br 2002;84:873-7.  Back to cited text no. 15
    
16.
Hudson TM. Fluid levels in aneurysmal bone cysts: A CT feature. AJR Am J Roentgenol 1984;142:1001-4.  Back to cited text no. 16
    
17.
Jaffe HL, Lichtenstein L, Portis RB. GCT of bone, its pathological appearance, grading, supposed variants and treatment. Arch Pathol 1940;30:993.  Back to cited text no. 17
    
18.
Gille O, Oliveira Bde A, Guerin P, Lepreux S, Richez C, Vital JM. Regression of giant cell tumor of the cervical spine with bisphosphonate as single therapy. Spine (Phila Pa 1976) 2012;37:E396-9.  Back to cited text no. 18
    
19.
Lee CG, Kim SH, Kim DM, Kim SW. Giant cell tumor of upper thoracic spine. J Korean Neurosurg Soc 2014;55:167-9.  Back to cited text no. 19
    
20.
Marcove RC, Lyden JP, Huvos AG, Bullough PB. Giant-cell tumors treated by cryosurgery. A report of twenty-five cases. J Bone Joint Surg Am 1973;55:1633-44.  Back to cited text no. 20
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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