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CASE REPORT
Year : 2022  |  Volume : 70  |  Issue : 2  |  Page : 764-766

Localized Giant Cell Tumor of the Tendon Sheath of the Upper Cervical Spine: A Case Report


1 Department of Neurosurgery, West China Hospital, Sichuan University, Ghana
2 Department of Pathology, West China Hospital, Sichuan University, China
3 Department of Medicine, Princefield University, Ghana

Date of Submission12-Dec-2019
Date of Decision04-Mar-2020
Date of Acceptance17-Jul-2020
Date of Web Publication3-May-2022

Correspondence Address:
Dr. Siqing Huang
No 37, Guo Xue Xiang, Chengdu . 610041, Sichuan Province
Ghana
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.344600

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 » Abstract 


Introduction: Giant cell tumor of the tendon sheath (GCTTS) is commonly seen in the appendicular skeleton, and rarely arises from the axial skeleton. We describe a rare case of GCTTS in an adolescent in the upper cervical spine.
Case Presentation: A previously healthy 16-year-old boy presented with a 6-month history of numbness of right upper extremity, and had experienced a neck pain 4 months ago. Spinal MRI demonstrated a small syrinx at C2 level and a well-circumscribed extradural mass with contrast enhancement extending from the posterior arch of C1 to C2. The extradural mass was totally resected, and the syrinx underwent clinical and imaging surveillance.
Discussion: GCTTS should be considered in the differential diagnosis of the axial skeletal lesion although very rare. Gross-total resection is advocated in GCTTS of the upper cervical spine, and subtotal resection with meticulous lesion monitoring should be performed in unresectable cases.


Keywords: Adolescent, cervical spine, giant cell tumor of the tendon sheath, synovial tumor
Key Message: GCTTS should be considered in the differential diagnosis of the axial skeletal lesion although very rare. Gross-total resection is curable, and meticulous lesion monitoring should be performed in unresectable cases.


How to cite this article:
Hu Y, Chen M, Richard SA, Huang S. Localized Giant Cell Tumor of the Tendon Sheath of the Upper Cervical Spine: A Case Report. Neurol India 2022;70:764-6

How to cite this URL:
Hu Y, Chen M, Richard SA, Huang S. Localized Giant Cell Tumor of the Tendon Sheath of the Upper Cervical Spine: A Case Report. Neurol India [serial online] 2022 [cited 2022 Jul 3];70:764-6. Available from: https://www.neurologyindia.com/text.asp?2022/70/2/764/344600




Giant cell tumor of the tendon sheath (GCTTS) belongs to a part of the group of synovial tumors that generally occurs in joints, tendon sheaths, and bursae.[1] GCTTS is commonly seen in the appendicular skeleton, and rarely arises from the axial skeleton.[2] Since the first spinal case described by Kleinman et al. in 1980, approximately 80 cases are reported in the literature.[3],[4],[5] Spinal forms of GCTTS commonly affects cervical spine, followed by the lumbar and thoracic spines.[4] Even though cervical spine was the most common location, the occurrence of GCTTS in the upper cervical spine is rare, and only 10 cases have been reported to our knowledge [Table 1].[1],[2],[3],[4],[6],[7],[8],[9],[10] We describe the case of a 16-year-old boy who was found to have GCTTS of C1-C2, and we could not find any description of GCTTS of the upper cervical spine in an adolescent.
Table 1: Summary of GCTTS of the upper cervical spine

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 » Case History Top


A previously healthy 16-year-old boy presented with a 6-month history of numbness of right upper extremity. He had experienced a neck pain 4 months ago. No significant family history was present. A neurological examination confirmed right upper extremity sensory deficit. Laboratory investigations revealed normal hematologic and biochemical parameters. Spinal MRI demonstrated a small syrinx at C2 level and a well-circumscribed extradural mass with contrast enhancement extending from the posterior arch of C1 to C2 [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d. No  Chiari malformation More Details and tethered cord were observed.
Figure 1: MRI, intraoperative, and pathological features. MRI: T1-weighted (a), T2-weighted (b), post-contrast T1-weighted (c and d); intraoperative finding (e); hematoxylin-eosin staining reveals fibrous connective tissue infiltrated by a large numbers of foamy cells and few multinucleated giant cells (f), the lesion is positive for Ki-67 (approximately 10%) (g) and CD68 (h)

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Concerning the syrinx was not related to the extradural lesion, surgical treatment for the extradural mass was recommended, and clinical and imaging surveillance for the syrinx was performed. A yellowish-brown lesion measured 2.3 cm × 2.0 cm × 1.8 cm was then entirely removed [Figure 1]e. We did not observe any continuity between the lesion and the facet joints during the operation. Microscopically, hematoxylin-eosin staining revealed fibrous connective tissue infiltrated by a large number of foamy cells and few multinucleated giant cells [Figure 1]f. The lesion was positive for Ki-67 (approximately 10%) [Figure 1]g, and was immunoreactive to the macrophage marker CD68 [Figure 1]h. The pathologic findings were compatible with the diagnosis of GCTTS. The neck pain relieved, and the numbness of right upper extremity remained stable 1 year after the operation. MRI revealed no signs of tumor recurrence at that time.


 » Discussion Top


GCTTS is categorized into localized and diffuse forms based on its locations and encapsulation.[1],[10] The localized from is predominantly an extra-articular growth and generally affects the small joints of the hand and feet. The diffuse variant, also termed pigmented villonodular synovitis, mainly grows inside the joints and frequently seen in the large joints, such as knee, hip, and ankle.[2],[10] The diffuse type seems more aggressive than the localized form, because it lacks clear boundaries and tends to invade surrounding structures, tumor recurrence after operation as well as malignant transformation in rare conditions.[2],[3] Our case was a localized one because the lesion was grown outside the joints and well encapsulated.

GCTTS of the upper cervical spine commonly presents in adult life at an average age of 44 years (range: 16–82 years). We could not find any description of upper cervical spinal GCTTS in an adolescent. The male:female ratio was 1:4 (the gender was not available in one case), showing a significant female predominance.[1],[8] Most patients can be asymptomatic or are associated with localized pain at time of presentation, and neurological deficits may also be observed in some cases.[2],[4] In rare conditions, an acute onset of symptoms and/or rapid neurological deterioration may occur because of a pathological fracture due to bone erosion.[2]

Radiologically, GCTTS of spine are often seen as focal soft-tissue mass, which are occasionally associated with bone erosion, periosteal reactions, and intralesional calcifications.[3],[10] Bone erosion has been demonstrated in at least 70% of cases of spinal GCTTS.[2],[4] In the upper cervical spine, 60% of cases (6/10, CT scans or radiographs was not available in one case) show bone destruction on CT scans or radiographs.[2],[3],[7],[8],[9] Facet or atlantoaxial joint involvement was demonstrated in 7 of 11 patients with GCTTS of the upper cervical spine, and 4 patients may originate from a bursa or vertebra membrane.[4],[8],[10] The lesion may display hyperattenuation due to hemosiderin deposition on CT scans.[2],[10] It often demonstrates iso-intense compared with skeletal muscle on T1-weighted images, whereas a heterogeneous lesion of variable intensity is revealed on T2-weighted sequences due to variable content of hemosiderin, liquid, lipids, fibrous tissue as well as hemorrhage.[4],[10] Avid enhancement of the lesion was characteristic and presented in most patients following administration of contrast agent.[2],[8] Positron emission tomography-computerized tomography (PET-CT) has been used in two patients with GCTTS of the upper cervical spine, which shows metabolically active lesion with intense FDG uptake.[9],[10] GCTTS of spine should be considered in the differential diagnosis of patients presenting as a soft tissue mass.[11]

Gross-total resection (GTR) is advocated in patients with spinal GCTTS. A relative high recurrence rate (66.7%) was observed in patients who underwent subtotal resection, while a rate of 7.7% in those after GTR.[3] We achieved GTR in our case because the lesion was a localized one. In two cases of GCTTS of the upper cervical spine where GTR was impossible, the patients have remained stable for nearly 2 years, suggesting subtotal resection may result in disease control.[2],[9] However, meticulous lesion monitoring should be performed in those who underwent subtotal resection. For unresectable or recurrent cases, molecularly targeted therapy, such as imatinib, that targets the colony-stimulating factor 1 receptor of GCTTS may be an alternative treatment option.[4],[10] Whether radiation therapy is effective in the treatment of spinal GCTTS remains unclear, and the therapy should be performed in caution in inoperable or recurrent patients.[2],[10] Cervical instrumentation should be considered if there is any sign of spine instability.[2],[3],[7]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Teixeira WG, Lara NA Jr, Narazaki DK, de Oliveira C, Cavalcanti C, Marins LV, et al. Giant-cell tumor of the tendon sheath in the upper cervical spine. J Clin Oncol 2012;30:e250-3.  Back to cited text no. 1
    
2.
Finn MA, McCall TD, Schmidt MH. Pigmented villonodular synovitis associated with pathological fracture of the odontoid and atlantoaxial instability. Case report and review of the literature. J Neurosurg Spine 2007;7:248-53.  Back to cited text no. 2
    
3.
Wang K, Zhu B, Yang S, Liu Z, Yu M, Liu X. Primary diffuse-type tenosynovial giant cell tumor of the spine: A report of 3 cases and systemic review of the literature. Turk Neurosurg 2014;24:804-13.  Back to cited text no. 3
    
4.
Furuhata R, Iwanami A, Tsuji O, Nagoshi N, Suzuki S, Okada E, et al. Tenosynovial giant cell tumor of the cervical spine: A case report. Spinal Cord Ser Cases 2019;5:23.  Back to cited text no. 4
    
5.
Kleinman GM, Dagi TF, Poletti CE. Villonodular synovitis in the spinal canal: Case report. J Neurosurg 1980;5:846-8.  Back to cited text no. 5
    
6.
Pulitzer DR, Reed RJ. Localized pigmented villonodular synovitis of the vertebral column. Arch Pathol Lab Med 1984;108:228-30.  Back to cited text no. 6
    
7.
Graham EJ, Kuklo TR, Kyriakos M, Rubin DA, Riew KD. Invasive pigmented villonodular synovitis of the atlantoaxial joint: A case report. J Bone Joint Surg Am 2002;84:856-60.  Back to cited text no. 7
    
8.
Blankenbaker DG, Tuite MJ, Koplin SA, Salamat MS, Hafez R. Tenosynovial giant cell tumor of the posterior arch of C1. Skeletal Radiol 2008;37:667-71.  Back to cited text no. 8
    
9.
Lavrador JP, Oliveira E, Gil N, Francisco AF, Livraghi S. C1-C2 pigmented villonodular synovitis and clear cell carcinoma: Unexpected presentation of a rare disease and a review of the literature. Eur Spine J 2015;24:S465-71.  Back to cited text no. 9
    
10.
Yamada S, Oshima K, Hamada K, Sotobori T, Joyama S, Hashimoto N, et al. Giant cell tumor of the tendon sheath arising from a membrane surrounding the posterior arch of C1: A case report. Spine J 2016;16:e353-7.  Back to cited text no. 10
    
11.
Pandey S, Puntambekar A, Sharma V, Singh K, Santhosh D, Ghosh A, et al. Clear cell sarcoma of the paraspinal ligament - A rare tumor at an unusual location: A review. Neurol India 2016;64:802-5.  Back to cited text no. 11
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