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38 Osteosarcoma
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Fig. 38.1 A 19-year-old female patient’s T2-weighted sagittal and axial thoracic MR images
revealed a signicant canal involvement by a soft tissue mass originated from vertebral body
223
mineralization (Chap. 56). In addition, aggressive growth patterns with surrounding
soft tissue invasion, expansile remodeling, periosteal reaction, cortical destruction,
associated peritumoral soft tissue mass, spinal canal violation, and pathologic fractures can be seen (Fig.38.1).
38.5 Differential Diagnosis
Diagnosis is made with biopsy (Video 38.8). Metastatic lesion to the bone (Chap.
63), Ewing’s sarcoma (Chap. 39), aneurysmal bone cyst (Chap. 56), leukemia, lym-
phoma, eosinophilic granuloma (Chap. 35), osteomyelitis, and spondylodiscitis
(Chap. 65) and spondylodiscitis are the most common entities in differential
diagnosis.
38.6 Treatment Options
Musculoskeletal sarcoma is best treated with wide resection and neoadjuvant/adjuvant therapy if the tumor histology is compatible. Currently, OS is treated with
multimodality therapy including neoadjuvant therapy followed by surgery and then
adjuvant therapy.

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Fig. 38.2 After
conrmation diagnosis
with a percutaneous
biopsy, en bloc
vertebrectomy was done
M. Çetinkaya and A. Şenköylü
After the diagnosis with biopsy (percutaneous or open), the primary goal of the
surgical treatment is complete resection of the tumor with tumor-free margins
(Video 38.8). However, complete tumor resection may cause signicant local and
systemic morbidity, depending on the tumor size, location, and the basal health
status of the patient. Aggressive resection is known to be improving the neurological and functional status, local tumor control, and long-term survival. As well, inadequate excision leads to high rates of metastasis and local recurrence since
osteosarcomas (OS) are locally very aggressive. The optimal excision technique is
the en bloc resection of the vertebral body with tumor-free margins and reconstruction of the defective level(s) with an appropriate size cage (Figs. 38.2, 38.3).
However, because of the local aggressiveness of the tumor and the anatomical constraints, en bloc total resection is not always possible. Radiotherapy is rarely used
since OS are almost always resistant to it.
38.7 Expected Outcomes
Despite aggressive treatment, the expected outcome (survival) is frequently poor.

38 Osteosarcoma
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225
a
b
Fig. 38.3 A-P (a) and lateral (b) X-ray view after reconstruction of the vertebral column with a
mash cage and posterior pedicle screw xation
38.8 Potential Complications
Besides the natural poor prognosis and the fatal progress of the disease, there are
multiple complications secondary to the aggressive treatment options which include
chemotherapy, surgery, and radiotherapy. Radiotherapy is rarely used in OS as it has
local side effects like surrounding healthy tissue damage, postsurgical bony fusion
delay, and wound healing trouble, while chemotherapy has mostly systemic effects.
Surgical intervention has several potential complications as well including spinal
cord injury, dural tears and cerebrospinal uid leakage and stula, nonunion or
delayed union, implant failure, and major vascular structure injury.

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M. Çetinkaya and A. Şenköylü
38.9 What Should Patient andFamily Know?
OS of the spine has a poor prognosis. Even with appropriate treatment, poor outcome is not unfrequent. All caregivers involved in the care of patients with OS
(spine surgeon, oncologist, other specialists) must warn patient and family about the
prognosis of the disease and what are the available treatment options, the outcome,
and the need for additional surgical procedures.
Further Readings
1. Mukherjee D, etal. Survival of patients with malignant primary osseous spinal neoplasms:
results from the Surveillance, Epidemiology, and End Results (SEER) database from 1973 to
2003. J Neurosurg Spine. 2011;14(2):143–50.
2. Shives TC, etal. Osteosarcoma of the spine. J Bone Joint Surg Am. 1986;68(5):660–8.

Ewing’s Sarcoma
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39
PeterPalVarga andAronLazary
39.1 Definition
Ewing’s sarcoma (ES) is the second most common primary malignant bone tumor
in children and adolescents although the spinal localization is not frequent. The
tumor arises from bone marrow-derived mesenchymal stem cells, and a genetic
translocation between chromosomes 11 and 22 (EWS and FLI1 genes) is found in
most (>80%) cases. Extraskeletal (lung, kidney, etc.) localizations of primary ES
are also possible. Therapy for ES includes aggressive multimodal therapy with chemotherapy, surgery, and radiation which allows 50% to 60% of those without metastases to achieve long-term relapse-free survival. The role of neoadjuvant
chemotherapy is essential, even in a spinal case with neurological compromise, but
the timing and type of surgery and dose of radiation are still questioned.
39.2 Natural History
The median age of diagnosis is approximately 15years. The spine can be affected
by the disease both as the primary site and more commonly as a metastatic progression (Chap. 63); prognosis is poorer in the latter case. Depending on the extent and
localization of the primary tumor, the spinal lesion can be symptom-free and can be
Supplementary Information The online version contains supplementary material available at
(https://doi.org/10.1007/978- 3- 030- 80356- 8_39).
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@bhc.hu; 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 Spine Surgery,
https://doi.org/10.1007/978-3-030-80356-8_39
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discovered during follow-up screening imaging studies of limb ES.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 (Chaps. 11 and 41). Functional and neurological deterioration can
be caused by the tumor mass or pathological fracture. Alarming symptoms are
intractable pain, progressing neurological decit (Video 39.4), cauda equina syndrome, or signs of spinal cord compression.
P. P. Varga and A. Lazary
39.3 Physical Examination
Standard spinal physical examination of the patient is crucial to identify conditions
requiring emergency surgery; however, it is rare in ES. Laboratory ndings are
nonspecic.
39.4 Imaging
In imaging studies, ES is an aggressive lesion with mostly mixed lytic-sclerotic pattern with large soft tissue component. Periosteal reactions like onion-skinned, spiculae, “sunburst,” or Codman’s triangle are rarer in spinal localization. For bony
structures, computed tomography (CT) scan is more sensitive than standard radiographs, but the primary imaging modality for local staging and surgical planning is
magnetic resonance imaging (MRI). Bone scintigraphy and positron emission
tomography (PET) are useful to detect distant metastases which occur in 25% of all
cases at the time of the initial diagnosis. Regular imaging studies (MRI, CT) are
advised during the follow-up postoperatively to assess local control.
39.5 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 39.8).
39.6 Treatment Options
Treatment of ES is multimodal. Except for emergency cases, where an urgent
decompression of the spinal cord can be required, the treatment process is planned
by the oncologist and started with chemotherapy (vincristine, doxorubicin, and
cyclophosphamide; VDC), alternating with ifosfamide and etoposide (IE) or VDC/

39 Ewing’s Sarcoma
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IE. Surgical resection of the remnant tumor mass and radiotherapy are crucial to
achieve local control. Based on Enneking’s principles, a wide en bloc resection of
the high-grade malignant bone tumor would be required for the optimal oncological
outcome; however, it is extremely difcult to achieve such outcome in spine ES
without dramatic functional loss. On the other hand, intralesional resection of the
tumor is associated with a higher rate of local recurrence and shorter survival. The
recommended surgical treatment for such lesions is an en bloc resection with wide
or marginal margins, if technically possible, followed by radiotherapy. It requires
advanced technical skills and experience in spinal tumor surgery; as it; these patients
must be treated in spinal tumor centers. Meticulous surgical planning is mandatory:
resection, stabilization/reconstruction, and soft tissue procedures must be meticulously planned. Involvement of professional partners—as vascular surgeon, chest
surgeon, and plastic surgeon—can be required depending on the localization of the
ES and the surgical plan. Ideally, treatment should be done in a few dedicated centers of the country gathering all competencies in one unique place (Fig.39.1).
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39.7 Expected Outcomes
Survival in spinal ES is poorer compared to extremity cases. A 5-year survival is
about 60% in spinal ES, and it is related to the surgical resection (according to
Enneking’s principles) and timing of chemotherapy. The local recurrence rate is
high, about 30% within 5years after the index procedure. Local recurrence is associated with intralesional margins and previous tumor surgery. The functional outcome depends on the neurological functional loss caused by the tumor itself, the
consequence, or the complication of the surgery. Loss of spinal stability can result
in chronic, progressing pain decreasing the function and quality of life of the patient.
39.8 Potential Complications
The most frequent perioperative complications are wound healing problems, deep
surgical site infections, and neurological deterioration. Long-term complications
are implant loosening and development of secondary spinal instability.
39.9 What Should Patient andFamily Know?
ES is a high-grade malignant disease, where survival is relatively poor also in case
of effective multimodal therapy. Survival, local recurrence rate, and functional outcome (quality of life) are related to the outcome of surgery (proper resection, margins); however, performing adequate surgery is frequently challenging and is
characterized by a high complication rate. The proper oncological treatment before
and after the surgery is essential.

230
bd
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P. P. Varga and A. Lazary
a
e
i
f
j
k
c
g
l
h
m
n
Fig. 39.1 Thoracic ES surgically treated with en bloc resection, stabilization, and reconstruction.
The 48-year-old male patient was operated 3years ago because of a left humerus ES.Follow-up
imaging showed a thoracic tumor, affecting the Th9–11 level causing mild pain without neurological decit nor spinal instability (a, b). Neoadjuvant chemotherapy and irradiation resulted in the
shrinking of the tumor mass (c, d). Planning of the Enneking’s appropriate en bloc resection (e–h)
and stabilization. Postoperative X-ray after the surgery (i). A PMMA bone cement spacer anchored
to the posterior rod was used to reconstruct the anterior column (j, k). A “sliding” latissimus ap
was used for soft tissue reconstruction that resulted in perfect wound healing (l). The surgery and
adjuvant radiation therapy provided local control (m, n—1-y follow-up)
Further Readings
Arshi A, Sharim J, Park DY, etal. Prognostic determinants and treatment outcomes analysis of
osteosarcoma and Ewing sarcoma of the spine. Spine J. 2017;17(5):645–55.
Charest-Morin R, Dirks MS, Patel S, etal. Ewing’s sarcoma of the spine: prognostic variables for
survival and local control in surgically treated patients. Spine. 2018;43(9):622–9.
Sewee MD, Tan KA, Quraishi NA, etal. Systematic review of en bloc resection in the management
of Ewing’s sarcoma of the mobile spine with respect to local control and disease-free survival.
Medicine. 2015;94(27):e1019.

Discitis inPediatric Spine
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40
Yat-WaWong
40.1 Definition
Discitis, vertebral osteomyelitis, and spondylodiscitis refer to the infection of the
intervertebral disk, vertebral body, and bony vertebrae with the intervening disk,
respectively. They are the spectrums of spinal infections that are named according
to the primary site of infection. However, pure discitis is rare because most infections start in the metaphyseal region of the vertebral body as a result of rich blood
supply. Pediatric spinal infections can also be divided into pyogenic (most common), granulomatous, fungal, or parasitic origins.
40.2 Natural History
Pediatric spinal infections are relatively rare [1]. It is notoriously difcult to catch
the causative microorganisms, and inammatory markers do not usually elevate to
high levels. Some patients may even recover spontaneously. Therefore, in the past,
many clinicians did not believe the existence of discitis in children. Missing the
diagnosis may sometimes be catastrophic. The mean delay in diagnosis is
28weeks [2].
Supplementary Information The online version contains supplementary material available at
(https://doi.org/10.1007/978- 3- 030- 80356- 8_40).
Y.-W. Wong (*)
Chief of Spine Division, The University of Hong Kong, Queen Marry Hospital,
Hong Kong, China
e-mail: yatwa@hku.hk
© 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_40
231

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Y.-W. Wong
40.3 Physical Examination
Infants or very young children cannot present their symptoms well, and it imposes
diagnostic difculties. Isolated spinal infections in infants are rare, but the isolated spinal infection may be a part of disseminated sepsis. Although toddlers and
young children may complain of back pain, they usually present with irritability
and refusal to walk. Many such patients are afebrile on admission. Neurological
decit is uncommon until at a late stage (Video 40.4). The diagnosis of spinal
infections in older children and adolescents is similar to that in adults, because
older children and adolescents can present their symptoms well and have a higher
incidence of fever. Non-pyogenic spinal infections tend to have a subacute or
chronic course. In the tuberculous (TB) spine, the children may present with
round or angular kyphosis, torticollis, dysphagia, stridor, or respiratory obstructions due to huge cold abscesses.
40.4 Imaging
Radiographic changes may not be apparent in the rst two weeks except for soft
tissue edema. Typical radiological ndings of spondylodiscitis, regardless of pyogenic or TB infections, are narrowing of the intervertebral disk space and adjacent
vertebral end plate destruction. For spondylitis, the only destruction of the vertebral
body with a normal looking intervertebral disk is seen. Cold abscesses of TB spine
appear as paraspinal soft tissue shadow, but patients are typically not septic looking.
Other radiological features are described in the chapter on TB spine (Chap. 63).
Magnetic resonance imaging is the most sensitive in diagnosing spinal infections.
Its sensitivity and specicity are approximately 96% and 93%, respectively. For
patients with atypical presentations, FDG-PET may distinguish infections from
neoplasms.
40.5 Differential Diagnosis
For pyogenic spinal infections, an elevation of white blood cell count, CRP, and ESR
was only found in 41%, 57%, and 86% of the cases, respectively [2]. Positive blood
culture only appears in 8% of the cases. The positive yield from spinal biopsies or
aspirations is 40%. With such a low detection rate, some clinicians recommend
empirical antibiotics and reserving image-guided biopsies (Videos 40.7 and 40.8) or
aspirations for patients who do not respond to empirical antibiotics, who may be
infected by atypical microorganisms, or who have features of tumoral lesions.
Staphylococcus aureus is the most common cause of pediatric infections for all
age groups. Spinal infections in the rst 6months of life are rare because of the
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