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A. Kale and H. Emmez
evaluated for metastatic spread by spine MRI with contrast-enhanced examinations
and also CSF cytology [1, 3].
36.3 Physical Examination
The clinical ndings of the patients can be divided into two groups. The rst belongs
to the region where the disease is primarily located: focal neurological decits,
signs of increased intracranial pressure, and epileptic seizures. The second is similar
to what can be observed in other spinal cord lesions: pain, motor decit, sensory
changes, and sphincter dysfunction (Video 36.4). The pain usually ts the location of
the tumor and tends to increase at night and with movement (Chap. 11). Motor and
sensory changes can vary considerably, depending on the location and extent [2, 3].
36.4 Imaging
Spinal metastases can be intramedullary or extramedullary. Intramedullary localization is less common and occurs by hematogenous or direct spread from the
leptomeninges. Extramedullary, intradural metastasis is much more frequent and
usually results from CSF seeding from primary CNS neoplasms which are in
close proximity or within the CSF compartments. MRI is considered the gold
standard imaging modality to diagnose spinal intramedullary tumors. The lumbosacral region is most frequently affected with a nodular and irregular, contrastenhancing thickening of the thecal sac and nerve roots. The surface of the spinal
cord may be coated, also known as “sugar-coating” [2]. Contrast-enhanced
T1-weighted sequences are mandatory. Figure36.1 shows a patient with lumbar
intradural medulloblastoma.
Fig. 36.1 MRI of patient
with lumbar intradurally
medulloblastoma (white
arrow)
36 Medulloblastoma andOther Seeding Tumors
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Medulloblastoma patients were classied as average or high risk by disease staging using the Chang classication, which included age, post-resection tumor size,
CSF cytology, and CNS and extra-CNS metastases (Fig.36.1). Also, it has recently
been subclassied with histological and genomic ndings [1].
The sensitivity of MRI for the detection of disseminated tumor is 83% compared
with 60% for individual contemporaneous CSF samples and 78% for multiple CSF
samples, over time.
In the assessment of leptomeningeal metastases, CSF collection for cytology or
ow cytometry differs between studies in terms of timing, collection site, and volume of CSF collected.
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36.5 Differential Diagnosis
Intramedullary and extramedullary pathologies should be kept in mind in the differential diagnosis of spinal medulloblastomas. The most common ones are astrocytoma (Chap. 37), ependymoma (Chap. 60), and seeding choroid plexus tumors and
germinomas. Spinal involvement due to hematological malignancies should not be
forgotten, especially in the childhood age group. Spinal masses observed in patients
with a previous diagnosis of primary CNS tumor should be considered in favor of
metastasis.
36.6 Treatment Options
Aggressive treatment in medulloblastoma results in better survival for these patients.
After gross total tumor resection, radiation is given to the posterior fossa. In children older than 3years of age, a lower dose of radiotherapy is given to the rest of the
head and spinal cord to treat a macro- or microscopic tumor that has spread along
the CSF pathways. In children younger than 3years of age, radiation therapy is
usually delayed until after the rst postoperative chemotherapy to reduce neurotoxicity. In addition to adjuvant chemotherapy, bone marrow transplant and high-dose
chemotherapy have also been shown to improve survival in some patients [1, 3].
It has been reported that medulloblastoma surgery often causes tumor cells to
spill into CSF.The presence of tumor cells seen in early postoperative CSF samples,
however, does not always indicate that the cells are capable of establishing distal
implants. Therefore, spinal MRI and CSF samples obtained more than 2weeks after
surgery will reduce the incidence of false-positive samples.
Given the paucity of literature, the management of intramedullary spinal metastasis remains controversial. The case reports available so far only suggest biopsy
and chemotherapy with or without radiation.
Early diagnosis of disseminated tumor is important for the initiation of prompt
treatment that may prevent neurologic deterioration, produce symptomatic improvement, and improve or prolong survival.
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A. Kale and H. Emmez
36.7 Expected Outcomes
Widespread spinal involvement is observed in 33% of the patients followed up with
MRI after medulloblastoma surgery. This spread occurs predominantly in the leptomeninges and rarely in the vertebral marrow. Spinal MRI has been reported to have
higher diagnostic accuracy than CSF cytological analysis for early detection of the
disseminated tumors [1]. The presence of disseminated disease seen with MRI is
associated with a poor prognosis.
36.8 Potential Complications
In addition to general complications of the surgery, the progression of neurological
decit and development of instability/deformity may occur after removal of the
tumor (kyphosis post laminectomy, in particular). Besides, the negative effects of
adjuvant chemotherapy and radiotherapy should be kept in mind, including the loss
of spinal growth in younger patients.
36.9 What Should Patient andFamily Know?
It should be noted that the prognosis of the patients mainly depends on the extent of
the disease. In diseases spread by spinal insemination, the treatment process is quite
difcult, and tumor control is generally not possible. The possibility of recurrence
should be kept in mind, and strict follow-up and controls should not be neglected.
Further Readings
1. Warren KE, et al. Response assessment in medulloblastoma and leptomeningeal seeding
tumors: recommendations from the Response Assessment in Pediatric Neuro-Oncology com-
mittee. Neuro Oncol. 2018;20(1):13–23.
2. Huisman TA.Pediatric tumors of the spine. Cancer Imaging. 2009;9:S45–8.
3. Goyal A, etal. Surgical treatment of intramedullary spinal metastasis in medulloblastoma: case
report and review of the literature. World Neurosurg. 2018;118:42–6.
Spinal Astrocytoma
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37
AydemirKale andHakanEmmez
37.1 Definition
Spinal cord tumors represent 6% to 8% of all central nervous system tumors combined and are relatively rare compared to intracranial neoplasms. Astrocytomas are
the most common intramedullary spinal cord tumor in childhood that develops from
astrocytic glial cells [1]. The peak incidence of spinal astrocytomas occurs in the
third decade and is more common in males. It is usually diagnosed a few months
after symptoms begin. The thoracic and cervical regions of the spinal cord are more
frequently affected, and usually multisegmental involvement occurs [1, 2]. It can be
diffuse or limited, and cystic areas can be found in half of the cases.
37.2 Natural History
Three-quarters of spinal astrocytomas are low grade. They tend to grow quickly or
slowly depending on the aggressiveness of the tumor [3]. If left untreated, they can
cause serious disability. The severity of preoperative neurological decits is correlated with poor postoperative outcomes. An increased incidence is observed in
patients with NF-1 (Chap. 23).
Supplementary Information The online version contains supplementary material available at
(https://doi.org/10.1007/978- 3- 030- 80356- 8_37).
A. Kale (*) · H. Emmez
Department of Neurosurgery, Gazi University Faculty of Medicine, 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_37
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A. Kale and H. Emmez
37.3 Physical Examination
Symptoms in intramedullary spinal cord tumors usually develop slowly, over a long
period of time. The most commonly reported symptoms are pain, motor decit,
sensory changes, and sphincter dysfunction [1] (Video 37.4). The pain usually ts
the location of the tumor and tends to increase at night and with movement. Scoliosis
may accompany the thoracic region. Motor and sensory changes can vary considerably, depending on the location and extent.
37.4 Imaging
Astrocytomas are usually intramedullary masses that cause a diffuse expansion in
the spinal cord. If osseous remodeling has not developed yet, radiographs and computed tomography (CT) scans are normal. Magnetic resonance imaging (MRI) is
considered the gold standard imaging modality to diagnose spinal intramedullary
tumors (Fig. 37.1). They are generally detected as hyperintense lesions in
T2-weighted images and as iso-hypointense lesions in T1-weighted images in
MRI.Its borders are not clear, and there is heterogeneous contrast enhancement [2].
The true size of the mass may not be understood due to edema in the cord, and
contrast-enhanced examinations help in the distinction. There may be a cystic component as well as intratumoral hemorrhage. Figure37.1 shows a patient with cervical intramedullary grade 2 astrocytoma.
Fig. 37.1 MRI of patient
with astrocytoma (cervical
spine)
37 Spinal Astrocytoma
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37.5 Differential Diagnosis
The most important differential diagnosis is ependymoma (Chap. 60). It is observed
more frequently in adults. More frequent observation of scoliosis and bone remodeling, location of the central part of the spinal canal, well-circumscribed nature,
high tendency to hemorrhage, homogeneous contrast involvement in focal intense,
and more frequent and prominent cystic components are characteristics of ependymomas [3]. In addition, other intramedullary masses such as hemangioblastoma,
epidermoid cyst, ganglioglioma, and metastasis should be kept in mind as potential
differential diagnosis.
37.6 Treatment Options
Treatment protocol choices for intramedullary astrocytomas remain controversial
but usually include surgery, radiotherapy (RT), and chemotherapy [1–3]. Among
surgical options are gross total resection, subtotal resection, and biopsy (Video
37.8). The main treatment for spinal astrocytomas is surgical removal. Figure37.2
shows the surgical view of cervical intramedullary grade 2 astrocytoma (Fig.37.2).
However, it is generally not possible to remove the tumor completely due to its
inltrative nature. Concerning the surgical approach, some spine surgeons recommend the following cleavage, while some others recommend removing the tumor
from the inside to the outside of the glial plane. CUSA is extremely helpful in the
removal of astrocytomas because low-grade astrocytomas tend to be hard and sticky.
Intraoperative ultrasonography is an extremely helpful method for both surgical
strategies. Somatosensory evoked potentials taken with the help of electrodes placed
on the dorsal columns are another functional auxiliary method during surgery. Since
the motor pathways can be damaged independently of the sensory system, motor
Fig. 37.2 Surgical view of
cervical intramedullary
grade 2 astrocytoma
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evoked potentials can be monitored by taking transcortical stimulation and epidural
recording to eliminate this risk. Survival rates of patients with gross total or subtotal
excision are much better than those with biopsy. Therefore, postoperative adjuvant
chemotherapy and RT are used for tumor control. RT can signicantly prolong survival time. On the contrary, the spinal cord is sensitive to the effects of radiation.
Overdosage of RT treatment has been proven to lead to the occurrence of radiationrelated tumors. Considering this, overdosage must be avoided to reduce the risk of
radiation-related tumors. Although temozolomide and bevacizumab are the most
widely used chemotherapeutic agents, their effect on survival in high-grade astrocytomas is controversial.
A. Kale and H. Emmez
37.7 Expected Outcomes
Histological grading is the most important predictor of survival in spinal cord astrocytomas. Mortality risk increased 14 times in high-grade gliomas compared to lowgrade gliomas. Young patients have a better prognosis than the elderly population.
The prognosis of spinal glioblastoma cases was extraordinarily gloomy, with a
mean survival of 14.3months, and only 14.1% of patients still survive 24months
after initial diagnosis. Also, gender does not have a signicant effect on prognosis.
Since total resection rates of lesions located in the cervical region are better, the
prognosis is better, while the possibility of permanent neurological decit in the
thoracic region is much higher. Surgery performed with mild neurological decits
at the onset of symptoms has better results. As much tumor resection as possible
improves the prognosis. Postoperative RT increases mortality rates in low-grade
gliomas, while the opposite prolongs survival in high-grade gliomas. While chemotherapy is within the standard treatment protocol for high-grade gliomas, its effectiveness has also been reported in low-grade gliomas, recently. Although there is not
much data about the use of RT in combination with chemotherapy, it is reported that
it has little effect on long-term survival [3].
37.8 Potential Complications
In addition to general complications of the surgery, the progression of neurological
decit and development of instability and/or deformity may occur after removal of
the tumor. Besides, the negative effects of adjuvant chemotherapy and RT should be
kept in mind.
37.9 What Should Patient andFamily Know?
It should be known that the prognosis of the patients depends on many factors. Very
good results can be obtained, or the rest of their life can be maintained as a nursing
patient. The possibility of recurrence should be kept in mind, and strict follow-up
and controls should not be neglected.
37 Spinal Astrocytoma
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219
Further Readings
1. Azad TD, etal. Surgical outcomes of pediatric spinal cord astrocytomas: systematic review and
meta-analysis. J Neurosurg Pediatr. 2018;22(4):404–10.
2. Garber ST, et al. Pediatric spinal pilomyxoid astrocytoma. J Neurosurg Pediatr.
2013;12(5):511–6.
3. Hamilton KR, etal. A systematic review of outcome in intramedullary ependymoma and astro-
cytoma. J Clin Neurosci. 2019;63:168–75.
Osteosarcoma
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38
MehmetÇetinkaya andAlpaslanŞenköylü
38.1 Definition
The most common malignant bone tumors of the spine are metastasis (metastatic
disease; Chap. 63). However, OS is the second most common primary malignant
bone tumor after multiple myeloma, of which only 3% to 5% occur in the spine. OS
mostly affects patients during their second and seventh decade of life (peak incidence). OS is a very aggressive high-grade malignant bone tumor with a poor prognosis. Radiotherapy, chemotherapy, surgery, and combined treatment options have
been developed to manage patients with OS.Recent advances in treatment, including aggressive en bloc resection and the use of adjuvant and neoadjuvant chemotherapy, provided longer survival times and better local control of the disease.
Inadequate excision leads to high rates of metastasis and local recurrence since OS
is extremely aggressive locally. The optimal excision technique is the en bloc resection of the vertebral body with tumor-free margins. However, because of the local
aggressiveness of the tumor and the anatomical constraints, en bloc total resection
is not always possible.
Supplementary Information The online version contains supplementary material available at
(https://doi.org/10.1007/978- 3- 030- 80356- 8_38).
M. Çetinkaya
Department of Orthopaedics, Spinal Unit, Stellenbosch University Faculty of Medicine and
Health Sciences, Western Cape, South Africa
A. Şenköylü (*)
Department of Orthopaedics & Traumatology, Gazi University School of Medicine,
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_38
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M. Çetinkaya and A. Şenköylü
38.2 Natural History
OS of the spine has a poor prognosis with relatively short-term survival. It ends up
with death when untreated in the early term. Even with the appropriate treatment
and maximum precautions, poor outcomes are not unexpected in highly aggressive
tumors. Mukherjee etal. [1] reviewed the National Cancer Institute’s Surveillance,
Epidemiology, and End Results (SEER) database in 2011 for 1892 patients with
spinal neoplasms. Their report included 430 patients with OS who underwent surgical treatment and radiotherapy. According to the results of their study, 78% of those
patients had died during their SEER follow-up period and 28% developed metastasis. In agreement with Mukherjee’s study, Shives etal. [2] documented 27 cases
with spinal OS and found that 26 (96.3%) of the patients had died of the disease
1 to 18months after surgery.
38.3 Physical Examination
On physical examination, inspection mostly gives no information unless there is an
obvious spinal deformity, invasion to the skin, stula, constitutional symptom, or
any symptom secondary to second organ metastasis. The spinous process of the
affected vertebral level may show some tenderness on palpation, particularly when
there is signicant posterior element invasion. Since the structural integrity and
weight-bearing capacity of the vertebral body is lost, mechanical pain is provoked
by anterior/posterior/side bending, spinal column rotation, and long-distance walking (Chap. 41). Neurologic functions can be impaired due to canal violation by the
tumor, and it may range from single nerve root pathology to para/tetraplegia (Videos
38.4 and 38.9).
38.4 Imaging
Plain radiographs of the spine usually show a blastic lesion that occasionally
appears as an “ivory body.” However, OS can also present as a lytic lesion. The
pure lytic pattern is seen in various subtypes, such as telangiectatic OS with predominant cystic architecture simulating aneurysmal bone cyst (ABC; Chap. 56). In
20% of the cases, mixed type may be found, while 5% has no typical radiographic
features.
Computed tomography (CT) is obviously superior to plain radiographs in depicting the cortical destruction and the matrix mineralization pattern of lytic lesions
which is found in about 80% of osteolytic cases.
Magnetic resonance imaging (MRI) can show the dense mineralization as a low
signal intensity on all pulse sequences. Particularly in the telangiectatic OS, uiduid level nding can be demonstrated on T2 sequences which differs from that of
ABC with thick, solid tissue surrounding the cystic spaces and matrix
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