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Fibrous Dysplasia of The Thoracic Spine: Sciencedirect
Fibrous Dysplasia of The Thoracic Spine: Sciencedirect
ScienceDirect
CASE REPORT
a
Division of Neurosurgery, Department of Surgery, Kaohsiung Medical University Hospital, Kaohsiung,
Taiwan
b
Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
c
Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung,
Taiwan
Received 3 September 2013; received in revised form 27 November 2013; accepted 15 January 2014
Available online 15 May 2014
KEYWORDS Summary Fibrous dysplasia occurs rarely in the spine, especially in the thoracic spine. Accord-
Fibrous dysplasia; ing to the literature, less than 20 cases have been reported. We report in this paper a 48-year-old
fusion; female patient with fibrous dysplasia of the first thoracic spine and left side of the first rib with
no recurrence; left thoracic first root compression. She complained of severe pain of the left arm, numbness on
radical resection; the ulnar side of the left forearm, and weakness of the middle to little fingers. Computed tomog-
thoracic spine raphy and magnetic resonance imaging showed the characteristic appearance of fibrous
dysplasia, which includes ground-glass opacity, an expansile nature, and lytic lesions with scle-
rotic rims. She underwent surgical intervention with radical tumor resection combined with sta-
bilization and fusion with a mesh graft. The pain was relieved. The thoracic first root compression
signs improved after the operation. Nineteen months after the operation, there was no recur-
rence of the tumor. We suggest that radical tumor resection combined with stabilization and
fusion with an anterior or posterior approach using a mesh graft for thoracic fibrous dysplasia
can achieve definite decompression and prevent tumor recurrence.
Copyright ª 2014, Taiwan Surgical Association. Published by Elsevier Taiwan LLC. All rights
reserved.
http://dx.doi.org/10.1016/j.fjs.2014.01.001
1682-606X/Copyright ª 2014, Taiwan Surgical Association. Published by Elsevier Taiwan LLC. All rights reserved.
Fibrous dysplasia of the thoracic spine 123
2. Case Report
Figure 3 T1-weighted magnetic resonance image (MRI) in the axial plane shows a hypointense multiloculated lesion (black arrow)
with no evidence of spinal cord compression, but the left nerve root is encased (left). Postcontrast axial spine T2-weighted MRI (on
the right) in the same plane shows mild enhancement of the lesion (black arrow).
The patient’s postoperative course was uneventful. the years 1961e2013 revealed only 16 cases of monostotic
Fig. 5 shows the patient’s postoperative radiography. By 6 FD of the thoracic spine.
months postoperatively, the patient’s pain score fell from Fibrous dysplasia has a characteristic radiographic
5e6 to 1e2 (based on the VAS) and the numbness and appearance. Plain radiographs, CT scans, and magnetic
weakness improved (the muscle power increased from 4 to resonance images show the presentation of an eccentric
5). Nineteen months postoperatively, a follow-up MRI lesion with intact cortex bone and marginal sclerosis in
showed no recurrence (Fig. 6). vertebral bodies without involving the vertebral appendix
and extraosseous soft tissue. The radiographic hallmark of
monostotic FD involving the spine is a well-defined multi-
3. Discussion loculated expansion of the medullary cavity with a ground
glass appearance and variable degrees of marginal scle-
Fibrous dysplasia represents approximately 7% of all benign rosis. The CT image and MRI are superior to plain radio-
tumor-like bone lesions.5 However, the spine is affected in graphs in defining the extent of involvement of the spine.
only 2.5% of patients. Fibrous dysplasia of the spine very The MRI may be helpful in demonstrating the characteris-
rarely exists without being present elsewhere in the body.6 tics of fibrous lesions. Fibrous dysplasia usually shows low
Spinal involvement occurs mostly in the polyostotic form of signal intensity on T1-weighted images, heterogenous
FD; it is unusual for it to occur in the monostotic form, mixed signal intensity on T2-weighted images, and mild
especially in the thoracic spine.3,5 A literature review of enhancement after the administration of a contrast mate-
rial. A sclerotic “rind” that encapsulates the expansile bone
lesion is always present in typical cases of FD. Spinal cord
compression can be well demonstrated on MRI.7
There is no standard treatment for FD. Treatment in-
cludes pharmacological therapy and surgical intervention.
Oba et al7 managed FD that involved only the 10th vertebra
with tumor resection, which was followed by bone grafting
with hydroxyapatite material. Arazi et al2 treated FD in the
sixth vertebra with the transthoracic approach and used a
costal graft and interbody fusion after en bloc resection.
Tezer et al6 cite the work of Przybylski et al who performed
a posterior fusion and instrumentation combined with an
anterior strut graft and 360 spinal fusion, after resecting
the tumor in FD located in the thorax, which showed
kyphotic deformation and neurological progression. Cha-
purlat et al5 cite the work of Nabarro and Giblin who
treated thoracic FD with tumor resection with anterior and
posterior approaches in combination with arthrodesis.
In treating FD, stabilization is as important as the excision
Figure 4 The pathology examination shows curvilinear of the lesion. A very important aim in treating spinal tumors
trabeculae of woven bone that lacks conspicuous osteoblastic is to perform one radical surgery and avoid repeated sur-
rimming (black arrow) arising in the background of fibrous geries. For this reason, the lesion should be resected as
tissue. extensively as possible. In large resection sites, stabilization
Fibrous dysplasia of the thoracic spine 125
Figure 5 Postoperative cervical radiography image. Surgical treatment consisted of C5e6 diskectomy with artificial cage fusion
[Bryan (Medtronic Spinal and Biologics, Memphis, Tennessee)] and T1 corpectomy with C7eT2 plate fixation.
Figure 6 Postoperative magnetic resonance image (MRI). No recurrence is present in the postoperative MRI.
and fusion should be performed cautiously to avoid prob- thoracic spine that mimicked a neoplastic or infectious
lems related to pseudoarthrosis.6 With the goal of 360 process. Fibrous dysplasia should be included in the dif-
stabilization and fusion with an anterior or posterior ferential diagnosis of patients with lytic bone lesions. A
approach, using a strut or mesh graft is an effective treat- combination of medical and surgical management can lead
ment in the polyostotic form of FD involving successive to excellent outcomes. As in the present patient, although
vertebrae.8 The prognosis is generally good, although poor located in the thoracic region, cystic lesions in segments of
outcomes are more frequent in younger patients and in the whole spine that are not successive or adjacent to each
patients with polyostotic forms of the disease. The risk of other should be evaluated for the possibility of FD with
malignant transformation is low. detailed radiographical studies. We also suggest radical
An alternative treatment for FD is bisphosphonate drugs. tumor resection combined with stabilization and fusion
Pamidronate is the most effective bisphosphonate and is with an anterior or posterior approach using a mesh graft
used at a dosage of 180 mg every 6 months. Prospective for thoracic FD to achieve definite decompression and
studies have been conducted in increasingly large numbers prevent recurrence.
of patients. However, there are no reports of patients with
monostotic FD of the spine who were treated with
bisphosphonates.2,5,6,8 In addition to the bisphosphonates, References
calcium and vitamin D supplementation may benefit pa-
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Increased expression of the c-fos proto-oncogene in bone
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inflammatory drug (NSAID) and supplementation with cal- 1546e1551.
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operation. No specific complaints were noted. fibrous dysplasia of the thoracic spine: clinicopathological
Fibrous dysplasia of the thoracic spine is rare, especially description and follow up. Case report. J Neurosurg. 2004;100:
in the thoracic spine. Our patient had FD limited to the 378e381.
126 Z.-S. Wu, S.-L. Hwang
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