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 Table of Contents  
Year : 2021  |  Volume : 8  |  Issue : 4  |  Page : 43-45

Primary Osseous Solitary Fibrous Tumor of C3 Vertebral Body: A Rare Case Report and Literature Review

1 Department of Neurosurgery, SGPGIMS, Lucknow, Uttar Pradesh, India
2 Department of Pathology, SGPGIMS, Lucknow, Uttar Pradesh, India, Lucknow

Date of Submission15-Oct-2021
Date of Acceptance28-Oct-2021
Date of Web Publication24-Dec-2021

Correspondence Address:
Arun K Srivastava
Department of Neurosurgery, SGPGIMS, Lucknow, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/joss.joss_17_21

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Solitary fibrous tumor (SFT) is a recently adopted nomenclature in the 2021 WHO Classification of Central Nervous System Tumors, for a group previously recognized by the hybrid term “hemangiopericytoma/SFT.” This is the third case report of primary osseous cervical SFT and the first case wherein the vertebral body was the origin site. We reviewed the literature for its clinical profile and operative nuances. We also reviewed the possible role of the Ki-67 proliferation index in deciding the need for adjuvant therapy in these cases.

Keywords: Cervical vertebrae, Ki-67 proliferation index, solitary fibrous tumor

How to cite this article:
Kumar A, Srivastava AK, Sahu V, Sardhara J, Pal L, Bhaisora KS, Maurya VP, Behari S. Primary Osseous Solitary Fibrous Tumor of C3 Vertebral Body: A Rare Case Report and Literature Review. J Spinal Surg 2021;8:43-5

How to cite this URL:
Kumar A, Srivastava AK, Sahu V, Sardhara J, Pal L, Bhaisora KS, Maurya VP, Behari S. Primary Osseous Solitary Fibrous Tumor of C3 Vertebral Body: A Rare Case Report and Literature Review. J Spinal Surg [serial online] 2021 [cited 2022 Jan 21];8:43-5. Available from: http://www.jossworld.org/text.asp?2021/8/4/43/333614

  Introduction Top

Solitary fibrous tumors (SFTs) are mesenchymal, nonmeningothelial tumors. The earlier hybrid term for these tumors has been discarded in the recent 2021 WHO Classification of Central Nervous System (CNS) Tumors.[1] These tumors are rare in the brain, while occurrence in the spine is still rarer.[2] This is the third case of primary osseous cervical SFT and the first case report of SFT arising from a vertebral body.[3],[4],[5]

  Case Report Top

A 25-year-old male presented with neck pain and radiating pain along the left shoulder and arm for 4 months. There were no focal neurological deficits. A contrast-enhanced chemotherapy (CT) of the cervical spine showed a well-defined, heterogeneously contrast-enhancing, lytic lesion arising from the body and left pedicle of C3 [Figure 1]a and [Figure 1]b. It was extending into the posterolateral paraesophageal space. The C2–C3 and C3–C4 intervertebral foramina on the left were also involved, with the destruction of the left posterior inferolateral edge of the C2 body. The left vertebral artery was seen encased by the tumor, while the spinal canal was not invaded [Figure 1]c and [Figure 1]d. On magnetic resonance imaging (MRI), the lesion was T1 hypointense and T2 hyperintense. The differential diagnosis included bony tumors such as chondroma or chondrosarcoma.
Figure 1: (a) Plain chemotherapy cervical spine showing C3 vertebrae with the destruction of the left side of the vertebral body and part of the pedicle. (b) Patchy enhancement of the tumor was seen on contrast chemotherapy. (c) The left vertebral artery was involved within the tumor. There was the destruction of the inferior part of the body of C2 vertebrae also. (d) On magnetic resonance imaging, the tumor was seen completely extradural with encasement of the left vertebral artery. (e) Postoperative magnetic resonance imaging (at 18 months) was suggestive of a small residual lesion. The vertebral artery was not visualized (thrombosed)

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The patient was operated on through a left anterior cervical approach with an incision along the anterior border of sternocleidomastoid muscle from the mastoid tip to the cricoid cartilage. The level IIA and level III neck node levels were exposed and dissected. The spinal accessory nerve was identified and preserved. The carotid sheath with the internal jugular vein, common carotid artery, external carotid artery, internal carotid artery, and vagus nerve was exposed and retracted medially. The cervical sympathetic chain was identified posterior to the carotid sheath and retracted medially. The tumor was found involving the body of C2 and C3 on retracting the longus colli muscles. A near-total tumor decompression was done, leaving a small portion along the vertebral artery and C3 nerve root. The tumor was moderately vascular and fibrotic, needing piecemeal resection.

Postoperatively, the patient had left partial ptosis, miosis, and anhidrosis (Horner's syndrome). There were no focal neurological deficits with relief in neck pain and radicular pain. Histopathology was suggestive of a moderately cellular tumor with pleomorphic cells having oval to spindle-shaped nuclei, fine chromatin, inconspicuous nucleoli, and scant cytoplasm. Occasional mitotic figures were noted. On immunohistochemistry, the tumor cells showed positivity for vimentin along with focal expression of BCL-2, confirming SFT [Figure 2]. The Ki-67 proliferation index was 8%–10%. No postoperative adjuvant therapy was given. At 18 months of follow-up, the patient is asymptomatic, except for mild paresthesia over the left side of the neck and shoulder. The postoperative contrast-enhanced MRI was suggestive of a small residual lesion [Figure 1]e.
Figure 2: (a) H and E-stained section showing a spindle cell tumor arranged in fascicles with few thin-walled interspersed blood vessels (×200). (b) Reticulin stain showing intertumoral reticulin deposition (×200). (c) Vimentin immunohistochemistry showing strong vimentin positivity (×200)

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  Discussion Top

Hemangiopericytoma (HPC) and SFT were earlier recognized as distinct pathological entities. Then in 2016 WHO Classification of Tumors (an update of the fourth classification), both these categories were integrated and termed as “HPC/SFT.” However, in the recent 2021 Classification of CNS Tumors, the fifth edition (WHO CNS5), the term HPC has been retired and the term SFT was used to denote these tumors.[1] These tumors are characterized by genetic aberrations involving inversion of 12q13, causing fusion of NAB2 and STAT6 genes.[6]

Differential diagnosis

Due to similar radiological findings, most of these tumors are diagnosed as meningioma or hemangioblastoma preoperatively. In this case, as the site of tumor origin was the vertebral body, we considered bony tumors such as chondroma and chondrosarcoma as the possible differential diagnosis. Most of the previous reports did not mention the site of tumor origin. The classification suggested by Liu et al. is also based on tumor extension.[3] Among the cases where the tumor origin was mentioned, we found this case to be the first case where SFT occurred in the cervical vertebral body. This affected the preoperative differential diagnosis and the surgical approach.

Operative nuances

In this case, we preferred an anterior cervical approach. The left vertebral artery and C3 nerve root were completely encased. Most authors reported that recurrence and overall survival were not affected by the extent of resection.[3],[7] Thus, a safe maximum possible resection was done. Postoperatively, Horner's syndrome was present as an approach-associated complication, possibly due to stretching of the sympathetic chain during retraction.

Adjuvant therapy

Due to the rarity of the disease, there exists no level 1 evidence for adjuvant therapy. Results have been variable in different studies of radiotherapy (RT) and CT in improving overall survival or preventing/delaying tumor recurrence.[8],[9],[10] Muñoz et al. reported that histopathological examination findings of SFT may not correlate with their clinical behavior.[11] Thus, a dilemma persists regarding optimum postoperative adjuvant therapy for spinal SFTs. In this case, as a near-total excision was achieved and the Ki-67 index was 8%–10%, we did not subject the patient to RT and opted for close follow-up. Even at 18 months, there is no evidence of tumor recurrence. In a series of 10 cases of spinal HPC/SFT reported by Singla et al., three patients had Ki-67 index ≥10%.[7] One patient died following tumor recurrence. This patient was operated on three times for tumor recurrence and received RT only after the third excision (subtotal resection). Of the other six operated patients with Ki-67 index ≤10%, three patients did not get RT and continued to be recurrence-free at follow-up. None of these patients reported tumor recurrence. The findings of this study and other case reports, including ours, suggest that there is a possible role of the Ki-67 index in formulating the postoperative management plan of spinal SFTs.[7],[12]

  Conclusion Top

A primary osseous SFT is a rare differential diagnosis of cervical vertebral body tumors. The extent of resection should be weighed against possible postoperative complications. Possible predictors for the postresection indication for adjuvant therapy need further evaluation.

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.

  References Top

Louis DN, Perry A, Wesseling P, Brat DJ, Cree IA, Figarella-Branger D, et al. The 2021 WHO classification of tumors of the central nervous system: A summary. Neuro Oncol 2021;23:1231-51.  Back to cited text no. 1
Kim JH, Jung HW, Kim YS, Kim CJ, Hwang SK, Paek SH, et al. Meningeal hemangiopericytomas: Long-term outcome and biological behavior. Surg Neurol 2003;59:47-53.  Back to cited text no. 2
Liu HG, Yang AC, Chen N, Yang J, Qiu XG, Zhang JG. Hemangiopericytomas in the spine: Clinical features, classification, treatment, and long-term follow-up in 26 patients. Neurosurgery 2013;72:16-24.  Back to cited text no. 3
Nakashima H, Imagama S, Sakai Y, Nakamura H, Katayama Y, Ito Z, et al. Dumbbell-type hemangiopericytoma in the cervical spine: A case report and review. J Orthop Sci 2013;18:849-55.  Back to cited text no. 4
Ramdasi RV, Nadkarni TD, Goel NA. Hemangiopericytoma of the cervical spine. J Craniovertebr Junction Spine 2014;5:95-8.  Back to cited text no. 5
Fritchie KJ, Jin L, Rubin BP, Burger PC, Jenkins SM, Barthelmeß S, et al. NAB2-STAT6 gene fusion in meningeal hemangiopericytoma and solitary fibrous tumor. J Neuropathol Exp Neurol 2016;75:263-71.  Back to cited text no. 6
Singla R, Singh PK, Khanna G, Suri V, Agarwal D, Chandra PS, et al. An institutional review of 10 cases of spinal hemangiopericytoma/solitary fibrous tumor. Neurol India 2020;68:448-53.  Back to cited text no. 7
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Combs SE, Thilmann C, Debus J, Schulz-Ertner D. Precision radiotherapy for hemangiopericytomas of the central nervous system. Cancer 2005;104:2457-65.  Back to cited text no. 8
Jia Q, Zhou Z, Zhang D, Yang J, Liu C, Wang T, et al. Surgical management of spinal solitary fibrous tumor/hemangiopericytoma: A case series of 20 patients. Eur Spine J 2018;27:891-901.  Back to cited text no. 9
Cohen Y, Lichtig C, Robinson E. Combination chemotherapy in the treatment of metastatic hemangiopericytoma. Oncology 1972;26:180-7.  Back to cited text no. 10
Muñoz E, Prat A, Adamo B, Peralta S, Ramón y Cajal S, Valverde C. A rare case of malignant solitary fibrous tumor of the spinal cord. Spine (Phila Pa 1976) 2008;33:E397-9.  Back to cited text no. 11
Koduru MR, Ail S, Shetty J, Shetty V. Solitary fibrous tumour of cervical spinal cord. Ann Afr Med 2020;19:144-6.  Back to cited text no. 12
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