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B. Karaaslan and A. Ö. Börcek
carcinoma, and medullary thyroid carcinoma should be considered in the differential diagnosis (sclerotic lesions; Chap. 63) (Video 55.8). Additionally, hematological malignancies such as lymphoma and myeloma should also be considered.
55.6 Treatment Options
As in every pathology, treatment should be tailored to the patient. Asymptomatic
patients can be followed up with no intervention. In case of neurological decits or
signicant pain, there are various treatment options. Radiotherapy, endovascular
embolization, percutaneous polymethyl methacrylate (PMMA – vertebroplasty),
and ethanol injection are the main management options. In case of signicant cord
compression and acute ndings, surgical resection of the lesion and spinal
stabilization can be considered although signicant blood loss can complicate the
surgical procedure. In symptomatic cases, embolization techniques and surgical
decompression may be combined according to the patients’ needs. Of note, young
patients with asymptomatic lesions should be followed clinically and/or
radiologically. Treatment options should be considered according to the extension
of the involvement, clinical picture, and age of the patient.
55.7 Potential Complications
Acute cord compression, pathological spinal fractures, and related deformity are
potential complications. In case of surgical intervention, surgeons should be
prepared for intraoperative bleeding that can cause signicant morbidity and in
some cases mortality.
55.8 What Should Patient andFamily Know?
VHs are usually benign and indolent lesions; successful treatment with the current
medical intervention techniques is possible. Only a small fraction of VH poses
clinical difculties, and successful treatment with the current medical intervention
techniques is possible.
Further Readings
Acosta FL, etal. Current treatment strategies and outcomes in the management of symptomatic
vertebral hemangiomas. Neurosurgery. 2006;58(2):287–95.
Fox MW, etal. The natural history and management of symptomatic and asymptomatic vertebral
hemangiomas. J Neurosurg. 1993;78:36–45.
Pastushyn AI, et al. Vetebral hemangiomas: diagnosis, management, natural history and
clinicopathological correlates in 86 patients. Surg Neurol. 1998;50:535–47.
Aneurysmal Bone Cyst
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MuratSongür andAlpaslanŞenköylü
56.1 Definition
Aneurysmal bone cyst of the spine (ABC) was formerly accepted as a pseudotumoral lesion of unknown origin; it is now considered to be a benign bone tumor.
ABC is characterized as multiple cysts containing blood and separated by a brous
septae. ABC is a rare tumor consisting of about 1% of primary bone tumors.
Although being a naturally benign lesion, some ABCs may have aggressive behavior. Success of index treatment is extremely important, because recurrence frequently results in a high level of structural problems and neurological decit.
56.2 Natural History
ABC has a benign nature without the risk of sarcomatous transformation. The cervical spine and sacrum (Fig. 56.1) are the most frequent locations of ABCs.
Pathological fracture, neurological involvement, and instability are rare although
possible.
Supplementary Information The online version contains supplementary material available at
(https://doi.org/10.1007/978- 3- 030- 80356- 8_56).
M. Songür
Zonguldak Bulent Ecevit University, Zonguldak, Turkey
e-mail: murat.songur@beun.edu.tr
A. Şenköylü (*)
Department of Orthopaedics and Traumatology, Gazi University, Ankara, Turkey
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2022
A. Şenköylü, F. Canavese (eds.), Essentials of Spine Surgery,
https://doi.org/10.1007/978-3-030-80356-8_56
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M. Songür and A. Şenköylü
a
b
c
Fig. 56.1 ABC of left S1–S2 (a) in a 14-year-old boy. T2-weighted and post-contrast MRI images
(b) reveal multiple cysts with uid-uid image at left S1 and S2 growing ventrally and dorsally.
Partial sacrectomy with sacral canal decompression followed by autogenous bone grafting was
performed (c)
56.3 Physical Examination
ABCs usually involve children and young adults. Patients present with localized
pain and swelling, with or without tenderness (Chap. 11). Spine motion can be
restricted at the involved region. Neurological impairment is rare (Videos 56.4
and 56.9). Signs and symptoms of myelopathy or single root involvement should
be evaluated carefully in cervical lesions, since these ndings may be overlooked
in healthy teens.
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56.4 Imaging
Plain radiographs reveal cystic-lytic soap bubble-like lesions with smooth nonsclerotic margins. Computerized tomography (CT) shows similar imaging.
T2-weighted magnetic resonance imaging scan typically demonstrates multiple
separated cysts with vertical uid-uid levels. Cervical lesions tend to involve posterior elements but may progress anteriorly interfering vertebral artery and involve
epidural space. Therefore, axial scans help involvement of vertebral artery and epidural space and also facilitate staging and decision-making.
56.5 Differential Diagnosis
Although ABCs have unique radiological features, tissue sampling is mandatory,
and it can be facilitated as CT-guided or open biopsy (Video 56.8). Telangiectatic
osteosarcoma may present with a similar radiographic pattern although it usually
progresses with completely different clinical course and outcome.
56.6 Treatment Options
Several treatment options are available for ABCs. Successful outcome is directly
related to the success of the index procedure. Therefore, selection of appropriate
initial treatment is of paramount importance. All treatment modalities have inherent
advantages and disadvantages. En bloc resection of the lesion may provide safe
margins with low risk of recurrence; the increased surgical morbidity is usually
accepted by many authors as “overtreatment.” Intralesional curettage (with or without adjuvant) has about a 30% risk of recurrence, and it usually necessitates preoperative embolization in order to avoid intraoperative hemorrhage. Selective arterial
embolization (SAE) can be considered as a rst-line treatment for lesions not
involving neural structures and without risk of pathological fracture. Preoperative
angiography is required to prevent sclerosing agent leakage into the spinal canal or
nervous system. Intra-cystic injections with acrylic sclerosing agents or biologics
are other treatment options. Especially bone marrow-derived mesenchymal stem
cell (MSC) concentrate injection under uoroscopy control gives promising results
although it is generally avoided in children because of the risk of late malignancy.
Radiation therapy (RT) is another treatment option with variable outcomes.
Since most of the literature on spine ABCs is represented by case series involving different treatment protocols, such studies outline favorable outcomes that are
not related to the type of treatment. Therefore, the least morbid type of treatment
should be performed.
Eventually, decision-making involves size, location, stability, neural involvement, and surgeon’s and interventional radiologist’s preference. Each case should
be evaluated separately, and treatment options should be tailored individually.
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M. Songür and A. Şenköylü
SAE is a common method for most lesions, especially extending a wide portion
of spine at both mobile segments and sacrum. If equipment and interventional radiologist are available, this treatment approach may be accepted as the rst-line treatment option in lesions carrying a high recurrence rate and requiring morbid surgical
approach. Angiography is indicated for almost all ABCs of the spine requiring treatment. Principles of embolization for ABCs may be summarized as microcatheterization of feeding vessel and injection of sclerosing agent to not only
feeding level but also segmental arteries bilaterally two levels above and below the
lesion (for thoracic and lumbar lesions). If the catheterized segmental artery is associated with anterior spinal artery, embolization is aborted at that level, since this
approach will eventually result in cord ischemia. Selective arterial coil embolization
is a method of choice for cervical lesions. Unlike sclerosing agent injection embolization performed at thoracic and lumbar levels, this procedure is performed unilaterally. Vertebral artery embolization from C7 to posterior inferior cerebellar artery
is common practice with good results. As the patient is awake during the procedure,
temporary obstruction of vertebral artery is performed to evaluate the patency of the
contralateral vertebral artery. Patients are followed for progression- regression of the
disease, ossication of the lesion, or revascularization of the lesion by CT scan and/
or angiography. SEA can be repeated until signs of healing are achieved. Despite
efforts, an increase in tumor size, deterioration of neurological status, and development of instability may indicate surgery. Another advantage of SEA is decreased
blood loss during surgery.
Intralesional bone marrow-derived MSC aspirate is another minimally invasive
procedure (Video 56.7), usually reserved for lesions not suitable for
SAE. Percutaneous puncture of the cysts and MSC aspirate injection theoretically
can decrease the intra-cystic pressure and facilitate osteoblastic regeneration.
Multiple injections may be required to achieve healing. MSC concentrates can be
mixed with demineralized bone matrix and other scaffolds to enhance osteo-inductivity within the cystic cavity.
The acute neurological decit, responsiveness to minimally invasive procedure,
and instability are the common surgical indications for ABCs. Two types of surgical
treatment are available: (1) wide resection and (2) curettage and bone grafting. Wide
resection is usually performed in posterior element-only cases, and it causes minimal surgical morbidity after resection. However, post-laminectomy kyphosis is an
important complication of posterior element wide resection in the growing spine,
reported between 37 and 95% of cases; in such cases, anterior and posterior surgery
is recommended. Another indication of surgical resection is sacral lesions. Since
achievement of complete embolization is extremely difcult in sacral lesions, surgery is the only viable method (Fig.56.1). Intralesional curettage has up to 30%
recurrence rates; therefore, it is not preferred in spinal lesions.
RT promises successful result in terms of tumor control, but radiotherapyinduced sarcoma, radiation myelopathy, and other side effects (especially in children) limit its use. RT should be reserved when other treatment options fail.
Therefore, multimodal approach is necessary to successfully manage ABCs of
the spine, with collaboration between interventional radiology and spine surgery.
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Future studies will focus on osteoclastic inhibition via RANKL inhibitors for
ABCs. Promising reports are available but not enough for routine use.
56.7 Expected Outcomes
Favorable results are reported with minimally invasive procedures such as selective
angiographic embolization and intralesional injections. Usually necessitating multiple applications, healing can be expected within 12 to 18 months. Surgery, even if
indicated, has a high rate of recurrence; wide resection may have unfavorable effect
on growing spine.
56.8 Potential Complications
Minimally invasive procedures seem to be resulted minimal complications. Open
surgery may have a high rate of complications such as kyphosis and recurrence,
unless it is performed by an experienced surgical team.
56.9 What Should Patient andFamily Know?
Minimally invasive approaches are evolving, and a favorable outcome is possible.
Multiple interventions may be required to achieve complete healing.
Further Readings
Amendola L, etal. Aneurysmal bone cyst of the mobile spine: the therapeutic role of embolization.
Eur Spine J. 2013;22(3):533–41.
Barbanti-Brodano G, etal. Aneurysmal bone cyst of the spine treated by concentrated bone mar-
row: clinical cases and review of the literature. Eur Spine J. 2017;26(Suppl 1):158–66.
Novais EN, etal. Aneurysmal bone cyst of the cervical spine in children. J Bone Joint Surg Am.
2011;93(16):1534–43.
Giant Cell Tumor
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PeterPalVarga andAronLazary
57.1 Definition
Giant cell tumor (GCT) is a benign bone tumor with locally aggressive behavior.
High risk of local recurrence (LR) and low risk for distant (lung) metastasis characterize the disease. Histologically, GCT is composed of multinucleated, osteoclastlike cells, mononuclear cells, and stromal spindle-shaped cells. These latest are the
neoplastic components of the tumor.
According to the Enneking classication, GCT is S2 (active benign) or S3
(aggressive benign) lesion. In S2 tumors, the Enneking appropriate treatment is
intralesional excision, while, in S3 cases, only en bloc resection can provide longterm local control. Both cases can be challenging in the spine because of the proximity of neural elements, the rich blood supply of GCT, and the consequent spinal
instability. The possible complexity and morbidity of the surgical treatment and the
high rate of LR resulted in the development of adjuvant therapeutic options like
local adjuvants (phenol, hydrogen peroxide, cryosurgery, or PMMA) and systemic
drugs (denosumab). Postoperative radiation therapy can be also used for local control; however, it can increase the transformation of GCT to radiation-induced
sarcoma.
Supplementary Information The online version contains supplementary material available at
(https://doi.org/10.1007/978- 3- 030- 80356- 8_57).
P. P. Varga · A. Lazary (*)
National Center for Spinal Disorders, Buda Health Center, Budapest, Hungary
Department of Spine Surgery, Semmelweis University, Budapest, Hungary
e-mail: vpp@vpphome.hu; aron.lazary@bhc.hu
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2022
A. Şenköylü, F. Canavese (eds.), Essentials of Spıne Surgery,
https://doi.org/10.1007/978-3-030-80356-8_57
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P. P. Varga and A. Lazary
57.2 Natural History
GCT is most common in young adults, and females are more frequently affected.
GCT is usually localized in the epiphyseal area of long bones; its spinal appearance
is rare although the aggressive nature of the tumor can cause spinal instability and/
or neurological decit. The most common symptom of a spinal tumor is pain which
can be nonmechanical tumorous pain, mechanical pain in case of spinal neoplastic
instability, or pain related to neurological compression (Chap. 41). Functional dete-
rioration can be related to the pain of neurological decits caused by the tumor mass
or pathological fracture. Alarming symptoms are intractable pain, progressing neurological decit, cauda equina syndrome, or signs of spinal cord compression
(Videos 57.4 and 57.9).
57.3 Physical Examination
Standard spinal physical examination of the patient is crucial to identify conditions
requiring emergency surgery; however, it is rare in primary spinal tumors. Laboratory
ndings are nonspecic.
57.4 Imaging
GCT is an aggressive, lytic lesion. Results of osteolysis can be visible on radiographs and computed tomography (CT) studies, while soft tissue expansion is diagnosed on magnetic resonance imaging (MRI) (Fig.57.1). In spinal GCT, MRI is
crucial to describe the relationship of the tumor to the spinal canal, nerve roots, and
spinal cord. Contrast-enhanced imaging studies are also important to explore the
main vessels and sometimes the feeding artery. Chest CT is indicated to exclude
lung metastases, especially in an advanced stage of the disease. Regular imaging
studies (MRI, CT) are advised during the follow-up postoperatively to assess local
control. Spinal GCT with extraosseous involvement can be categorized as an aggressive tumor.
57.5 Differential Diagnosis
Tumorous lesions and tumor-like lesions of the spine are the most common differential diagnostic issues. Following the rst and most important oncological principle, namely, “tissue is the issue,” the cornerstone of the differential diagnosis is the
histological examination of the lesion. In case of a primary spinal tumor, imagingguided percutaneous biopsy or open biopsy can provide adequate tissue for the
detailed histopathological studies (Video 57.8).
a
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b
c
d
e
f
Fig. 57.1 Thoracic GCT treated with denosumab. The 38-year-old female patient had back pain
for months. Thoracic spine MR showed a large soft tissue mass and lytic tumor at ThIII (a–c).
Biopsy proved the GCT diagnosis. Denosumab treatment was initiated with excellent clinical
result and local regression of the mass; however, a progressive kyphotic deformity developed
8months after the diagnosis (d–f)
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P. P. Varga and A. Lazary
57.6 Treatment Options
57.6.1 Surgery
Enneking appropriate surgical treatment (intralesional resection in S2 and en bloc
resection in S3 GCTs) provides signicantly better oncological outcome compared
to Enneking inappropriate treatment where LR rate is higher. Management of LR
can be difcult, and LR is associated with shorter survival. The en bloc resection of
aggressive spinal/sacral GCTs can be challenging and result in signicant neurological loss of function. Not only tumor resection but the management of spinal
instability and soft tissue reconstruction are important issues of preoperative planning (Fig.57.2).
57.6.2 Embolization
GCTs are vascularized tumors; high blood loss is one of the most common complications in intralesional surgeries. That is why preoperative embolization is strongly
b
Fig. 57.2 Surgical resection, reconstruction, and adjuvant denosumab treatment. The case above
with stable disease suffered from severe back pain because of the increasing kyphosis and pathological fracture (Fig. 57.1d–f). An intralesional surgical resection after embolization was performed followed by anterior column reconstruction with PMMA and posterior stabilization (a).
Denosumab has been followed providing good local control 2 years after the surgery (b–d)
d
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