Elsevier

World Neurosurgery

Volume 114, June 2018, Pages 155-160
World Neurosurgery

Case Report
Spinal Sclerosing Epithelioid Fibrosarcoma at the Cervicothoracic Junction

https://doi.org/10.1016/j.wneu.2018.03.105Get rights and content

Highlights

  • Spine is a very rare primary site for Sclerosing epithelioid fibrosarcoma.

  • En bolc resection of tumor in cervicothoracic junction can be challenging.

  • Because of the anatomic nature of the cervicothoracic junction and critical neurovascular structures that the tumor involves, a true en bloc resection may not be possible.

Background

Sclerosing epithelioid fibrosarcoma (SEF) is an unusual variant of fibrosarcoma that was previously considered to be a low-grade tumor with an indolent course. To our knowledge, the spine is a rare primary site for SEF, with only a few cases reported to date.

Case Description

We report a case of SEF occurring in the cervicothoracic spine of a 64-year-old man with complaints of neck pain and right hand tingling for 6 months before clinical presentation. Imaging revealed a large mass that extended from C5 to T1 in the right posterior paraspinal soft tissues, with involvement of the posterior elements from C6 to T1 and infiltration of the T1 vertebral body. We performed an en bloc spondylectomy and gross total resection of the tumor. Pathologic and immunohistochemical characteristics confirmed the diagnosis of SEF.

Conclusions

Because of the anatomic nature of the cervicothoracic spine and critical neurovascular structures that the tumor involves, a true en bloc resection might not be possible, but an oncologically sound excision is ideal.

Section snippets

Background

Sclerosing epithelioid fibrosarcoma is a rare soft tissue tumor first described by Meis-Kindblom et al.1, 2 in 1995. The tumor occurs mostly in the deep musculature, and it is frequently associated with the adjacent fascia or periosteum.3 The spine is rarely a primary site for sclerosing epithelioid fibrosarcoma (SEF), with only a few cases reported to date. SEF can arise from any single part of the spine4, 5, 6 or occur at multiple spinal sites.7 Our case was a histopathologically confirmed

Case Description

The patient is a 64-year-old, left-handed man with right hand tingling in the 4th and 5th digits for 6 months. Magnetic resonance imaging (MRI) showed a heterogeneous, predominantly T1 and T2 hypointense mass with peripheral enhancement; it extended from C5 to T1 in the right posterior paraspinal soft tissue and involved the posterior elements from C6 to T1. It infiltrated the T1 vertebral body and extended into the right C7-T1 neural foramen (Figure 1). The tingling had worsened, and he

Discussion

Sclerosing epithelioid fibrosarcoma is a rare tumor consisting of epithelioid cells arranged in strands, nests, and sheets, embedded in a fibrotic and extensively hyalinized stroma.3 It was first described in 1995 by Meis-Kindblom et al.2, 4 as a distinct and rare tumor of the soft tissue occurring primarily in the deep musculature and often involving the fascia or periosteum. The disease usually occurs in adults; it has no significant preponderance for one sex over another, and the mean age of

Conclusion

SEF is a rare malignant tumor, and its occurrence in the spine is even rarer. Because it is a sarcoma, we determined that an en bloc resection would give the patient the best chance of lowering the risk of local recurrence. Because of the anatomic nature of the spine and critical neurovascular structures that the tumor involves, a true en bloc resection may not be possible. In addition, adjuvant radiation therapy will also play a role in treatment; therefore, it is important to consider this

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  • Supplementary digital content available online.

    Conflict of interest statement: Dean Chou is a consultant for Medtronic and Globus, and he collects royalties from Globus.

    Both authors collaborated in the study design and diagnosing and managing the patient. Jing Zhang prepared the first manuscript draft and Dean Chou modified the manuscript subsequently. Both authors approved the manuscript.

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