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Fig. 62.7 Chondroid
chordoma. Chondroid
tumor islets are seen
among the tumor cells
M. A. Deveci and S. A. Şimşek
chondrosarcoma forming malign cartilage (Fig.62.7); chondroid chordomas are
common in lesions located in the skull base [1]. Besides its classical physaliphorous appearance, it is characterized by ability to stain immunohistochemically
with epithelial markers such as chordoma S-100 and epithelial membrane antigen (MUC1) and cytokeratins [4]. In some studies, the transcription factor for
notochord development, brachyury, has been shown to be a good marker for
chordomas.
The differential diagnosis of chordoma include chondrosarcoma, myeloma
(Chap. 61), lymphoma, giant cell tumor (Chap. 57), aneurysmal bone cyst (Chap.
56), and metastases (Chap. 63). Since both chondrosarcomas and chordomas are
S-100 positive, it should not be forgotten that if cytokeratin evaluation cannot be
made clear in small biopsy samples, there may be problems in differentiating chondrosarcoma from chordoma.
62.6 Treatment
Surgical Treatment. In the 1970s, Stener and Gunterberg introduced the idea of en
bloc resection for the treatment of sacral tumors [2]. It is very difcult to obtain a
wide surgical margin due to the complex anatomy and the challenges of reconstruction, in particular lling the dead space and the potential neurological decits (especially above S2 level). Risk of recurrence is twice as high if the tumor capsule is
damaged during resection; the local recurrence of sacral chordomas is about 2 years
in patients undergoing radical resection, and it is 8months in the other patients.
The posterior transperineal approach alone is often sufcient for chordomas
located below the sacroiliac joint. Resection is more difcult in lesions located in
the caudal part of the sacroiliac joint which require both anterior and posterior
approaches. If S2 roots can be preserved, it is possible to obtain normal bladder and
anal sphincter functions in about 50% of cases. Loss of more than 50% of the
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383
sacroiliac joint causes vertical and rotational instability in the lumbopelvic region
and requires lumbopelvic stabilization.
In lesions located in the sacrum, aps such as gluteus maximus ap and vertical
rectus abdominis muscle (VRAM) ap should be used to ll the dead space after
total resection (Fig.62.8).
Fig. 62.8 A 69-year-old male with sacral chordoma. Wide resection with closure of the defect
with gluteal ap
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M. A. Deveci and S. A. Şimşek
Fig. 62.8 (continued)
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385
Fig. 62.8 (continued)
Medical Treatment. Despite the advances in surgical treatment, en bloc resection
is only possible in only 50% of sacral tumors; this percentage is lower in tumors of
the mobile spine and the skull base.
Chordomas are known as radioresistant tumors and require doses above 60Gy.
Conventional radiotherapy applied with high-energy photons can only achieve
50–55Gy and cannot provide local control. With the development of methods using
particle radiation (especially protons) and improvements in the preservation of normal structures by imaging, planning, and directing radiotherapy to target tissues,
better tumor control has been achieved with fewer side effects [4]. Especially in
cervical lesions or lesions located at the skull base, where extensive surgeries are
not possible, better results are obtained with razoxane use, which increases radiosensitivity. There are also limited studies on radiotherapy with particles heavier than
protons such as helium and carbon ions. Compared with helium ions, carbon ions
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M. A. Deveci and S. A. Şimşek
have been shown to have more biological advantages and are more effective in the
treatment of chordoma [5].
Chordomas are not sensitive to chemotherapy like all other low-grade tumors.
The effectiveness of chemotherapy has been demonstrated in dedifferentiated chordomas, which constitute only 5% of chordomas.
In recent years, molecular-targeted agents have also begun to be tried in the treatment of chordomas. It has been shown that chordomas use the platelet-derived
growth factor receptor beta (PDGFR-ß) and its phosphorylated form for activation,
and tumor shrinkage has been shown as a result of targeted treatments. With the use
of 850mg of imatinib mesylate, which is a tyrosine kinase inhibitor, a decrease in
tissue activity has been demonstrated [6]. The epidermal growth factor receptor
pathway may also constitute another target for drugs. Studies are carried out on the
combination of cetuximab and getinib.
62.7 Expected Outcomes
Chordomas have metastasized in 5% of cases at the time of diagnosis. Lung, bone,
skin, and brain are the most common areas for metastasis. The metastasis rate is up
to 65% in advanced disease or recurrent lesions. The survival rate is affected by the
presence of metastases rather than local progression. It has been shown that the
average survival after the development of distant metastasis is less than 12months.
Metastases are often stable, and the patient is lost due to local tumor spread and
invasion of vital organs. Although it has been shown that the prognosis is not
affected by the histological type, there is information proving that chondroid chordomas have a better prognosis.
62.8 What Should Patient andFamily Know?
Although chordoma is a low-grade tumor, its clinical course resembles malignant
tumors because of its high recurrence rate and inltrative growth pattern; the prognosis is generally poor. The overall survival rate is 6 years on average, and local
recurrence is extremely frequent.
Further Readings
1. Chugh R, Tawbi H, Lucas DR, Biermann JS, Schuetze S, Baker LH.Chordoma: the nonsar-
coma primary bone tumor. Oncologist. 2007;12:1344–50.
2. Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT, Fereira MJ.Chordoma: current
concepts, management, and future directions. Lancet. 2012;13:69–76.
3. Yang XR, Ng D, Alcotra DA, etal. T (brachyury) gene duplication confers major susceptibility
to familial chordoma. Nat Genet. 2009;41:1176–8.
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4. Srivastava A, Vischioni B, Fiore MR, Vitolo V, Fossati P, Iannal A, et al. Quality of life in
patients with chordomas/chondrosarcomas during treatment with proton beam therapy. J
Radiat Res. 2013;54:i43–8.
5. Schultz-Ertner D, Nikoghosyan A, Thilmann C, etal. Results of carbon ion radiotherapy in 152
patients. Int J Radiat Oncol Biol Phys. 2004;58:631–40.
6. Casali PG, Messina A, Stacchiotti S, et al. Imatinib mesylate in chordoma. Cancer.
2004;101:2086–97.
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Metastatic Lesions oftheSpine
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63
VanessaHubertus, PeterVajkoczy, andJuliaSophieOnken
63.1 Definition
Metastatic lesions of the spine commonly occur in advanced malignant disease.
More than 50% of tumor patients suffer from bone metastases, with the spine presenting the most common site. Spinal metastases can be symptomatic or asymptomatic. At autopsy, 30% to 90% of tumor patients present with previously undiagnosed
spinal metastases. Around 5% of patients with spinal metastases present with acute
neurological decits due to metastatic epidural spinal cord compression (MESCC),
thus drastically reducing their health-related quality of life (HRQOL) and their statistic life expectancy [1].
63.2 Natural History
Malignancies underlying spinal metastases present a heterogenic group; the most
common are prostate, breast, kidney, and lung cancers. A signicant role also play
hematologic diseases such as multiple myeloma. In the spinal column, the thoracic
spine is the most frequent localization for metastatic lesions, followed by the lumbar spine. The least frequent localization presents the cervical spine, although
Supplementary Information The online version contains supplementary material available at
[https://doi.org/10.1007/978- 3- 030- 80356- 8_63].
V. Hubertus · P. Vajkoczy (*) · J. S. Onken
Charité - Universitätsmedizin Berlin, Berlin, Germany
e-mail: Peter.Vajkoczy@charite.de
© 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_63
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clinically highly relevant due to a higher occurrence of neurological decits in this
region. Special attention in the surgical treatment of spinal metastases must be paid
to metastases at the junctional zones—the craniocervical, cervicothoracic, thoracolumbar, and lumbosacral junctions, for their role in spinal stability.
V. Hubertus et al.
63.3 Physical Examination
In the examination of a patient potentially suffering from spinal metastases, specic
red ags must be assessed: Does the patient suffer from a new neurological (motor
or sensory) decit, bladder or bowel dysfunction, ataxia, new axial or radicular
pain, or paraparesis / paraplegia? It is important to assess the acuity of the symptoms in order to speed up the diagnosis process accordingly. In MESCC, a classic
spinal emergency, “time is spine.” The faster surgical decompression is applied, the
better is the neurological outcome of the patient. Next to the neurologic examination (Video 63.4), assessment of clinical status using the Karnofsky performance
status (KPS) and comorbidities is necessary, as is the clinical assessment, a thorough history of tumor diagnosis, staging, and prior treatment.
63.4 Imaging
Magnetic resonance imaging (MRI) is the gold standard with the highest sensitivity
in detecting spinal metastases. Usually, contrast-enhanced T1-weighted protocols
are used to display the extent and limits of the metastatic lesion and their invasion
into the spinal canal. In patients with spinal metastases, MRI of the whole spine
should be performed, as metastatic lesions often occur multifocally. To assess bone
involvement, with differentiation of osteoblastic and osteoclastic lesions, additional
high-resolution computed tomography (CT) is necessary. Alternative imaging like
bone scintigraphy or positron emission tomography (PET) is commonly used in
staging examinations and shows a lower resolution than CT or MRI; therefore, they
cannot replace these imaging modalities. The use of spinal angiography is restricted
to the preoperative display and embolization of highly vascularized metastases,
such as originating from renal cell or thyroid carcinomas.
63.5 Differential Diagnosis
One important differential diagnosis to rule out before surgery is a primary bone
tumor, such as chordoma (Chap. 62), Ewing sarcoma (Chap. 39), and chondro- or
osteosarcoma (Chap. 38). These tumors are quite rare but necessitate a gross tumor
resection due to their aggressive growth behavior, radioresistance, and potential for
tumor cell distribution during surgery. In suspected cases, tumor biopsy should precede denite surgery (Video 63.8).
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391
63.6 Treatment Options
The primary goals of surgery for spinal metastases are the preservation of neurological function, pain relief, mechanical stabilization, and local tumor control (Video
63.1). Ideally, surgical decision-making is based on an interdisciplinary approach,
considering four factors: neurological and oncological status, mechanical stability,
and systemic disease (the NOMS-decision framework by Laufer etal., Table63.1)
[2]. Evaluation of oncological status should include the expected response to nonsurgical tumor treatment and radiation sensitivity according to the tumor’s histopathology. Spinal stability is assessed according to the Spinal Instability Neoplastic Score
(SINS) [3], using CT and MR imaging of the spinal lesion (Table63.2). Systemic
disease assessment includes the calculation of expected patient prognosis according
to tumor and treatment history and comorbidities. Different prognostic scores were
established for this purpose, like the Tokuhashi and the Tomita scoring systems—
their clinical applicability though remaining limited.
Surgical options include decompression and stabilization surgery, dependent on
spinal stability according to SINS.Biopsies are functional for differential diagnosis
and can be combined with either decompression, stabilization, kyphoplasty or vertebroplasty. In case of extensive lytic disease or disease at junctional zones, 360°
stabilization and decompression with vertebral body replacement might be necessary (Fig.63.1). To reduce surgical risks, minimally invasive percutaneous stabilization using spinal navigation is trending. When instrumentation and fusion of the
Table 63.1 The Neurologic Oncologic Mechanical Systemic (NOMS) decision framework by
Laufer etal. [2]
Neurologic
Low-grade
MESCC
without
myelopathy
High-grade
MESCC
with/
without
myelopathy
Oncologic Mechanical Systemic
Radiosensitive Stable EBRT
Unstable Stabilization+cEBRT
Radioresistant Stable SRS
Unstable Stabilization+SRS
Radiosensitive Stable EBRT
Unstable Stabilization+cEBRT
Radioresistant Stable Able to
tolerate
surgery
Stable Unable
to
tolerate
surgery
Unstable
Unstable Unable
Able to
tolerate
surgery
to
tolerate
surgery
Decision
Decompression+Stabilization+SRS
cEBRT
Decompression+stabilization+SRS
Stabilization (MIS/
cement-augmentation)+cEBRT
cEBRT
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Table 63.2 The SINS by Fisher etal. with SINS 0–6 points stable, SINS 7–12 points potentially
unstable, and SINS 13–18 points dened as unstable lesions [3]
Element of the SINS
Location Junctional (C0–C2; C7–Th2; Th 11–L1; L5–S1)
Mechanical pain Ye s
Bone lesion Lytic
Alignment Subluxation/translation
Vertebral body involvement >50% collapse
Posterolateral involvement Bilateral
Characteristics
Mobile (C3–C6; L2–L4)
Semirigid (Th 3–Th 10)
Rigid (S2–S5)
No (occasional nonmechanical)
Pain free lesion
Mixed
Blastic
De novo deformity
Normal
<50% collapse
No collapse with >50% vertebral body involvement
None of the above
Unilateral
None
V. Hubertus et al.
Score
3
2
1
0
3
1
0
2
1
0
4
2
0
3
2
1
0
3
1
0
a
b
d
c
Fig. 63.1 Example of a 67-year-old female suffering from metastatic renal cell carcinoma with
osteolytic spinal metastasis at Th11, clinically presenting with pain and ataxia. Following angiographic embolization of the metastasis, 360° decompression and stabilization were performed via
vertebral body replacement Th11 with cage implantation and posterior stabilization with navigated
pedicle screw placement Th9–L1 using PEEK carbon pedicle screws for better MRI follow-up and
adjuvant radiation planning. Preoperative images a–c, postoperative image d
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