CONTINUOUS LEARNING LIBRARY

Tumor Pathology

Primary Malignant Bone

Tumors Surgery
Author: Dr William Gemio Jacobsen Teixeira
Editor In Chief: Dr Néstor Fiore
Senior Editor: Dr José María Jiménez Avila
OBJECTIVES

CONTINUOUS LEARNING LIBRARY

Tumor Pathology

Primary Malignant Bone

Tumors Surgery

■ Highlight surgery indications and the technical possibilities to

perform them.

■ Discuss the surgical planning for tumor resection.

■ Describe the treatment of some of the most frequent tumors.

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 2
CONTENTS
1. Introduction...........................................................................................................04
2. Surgery indications....................................................................................05
Indications according to the biological behavior..............................................................05

Possibility of performing the indicated surgery.................................................................06

Surgical planning..........................................................................................................................08

Summary........................................................................................................................................16

3. Specific lesions.................................................................................................17
Osteosarcoma...............................................................................................................................17

Chondrosarcoma.........................................................................................................................18

Chordoma......................................................................................................................................19

Multiple myeloma.......................................................................................................................20

Plasmocytoma..............................................................................................................................22

Summary........................................................................................................................................24

References.......................................................................................................................25

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 3
1 1. INTRODUCTION
Primary malignant spinal tumors are rare and may have different origins and
biological behaviors. The early diagnosis and treatment of primary malignant
tumors have a direct impact on the patient’s prognosis.
In the last few decades, the development of spinal-reconstruction techniques
and instrumentation enabled aggressive spinal tumor resections. Thus, there
is an increase in survival rates and in the probability of curing the disease in
patients without metastatic disease and with resection with free-margins.

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 4
 2 2. SURGERY INDICATIONS
The indication of surgical treatment depends on factors linked to the following
items:
■■ tumor histology
■■ local extension of the tumor
■■ proximity or involvement of neurovascular structures;
Indications according to biological behavior
Based on the biological behavior of musculoskeletal tumors, Enneking developed
a classification system for benign and malignant tumors (Enneking, Spanier and
Goodman, 1980). Tumors are also sub-classified according to the local extension
and presence of metastasis.
■■ presence of metastatic disease Presented next are images of a patient with clival chordoma with recurrence after
■■ tumor sensitivity to adjuvant therapies, such as chemo or radiotherapy the surgical treatment, without metastatic disease

Consequently, the oncological staging must be done prior to the treatment Stage I
decision through the following exams:
Before any therapy Low-grade malignant tumors without metastasis
decision, it is necessary ■■ CT scan of the following regions:
to perform the ■■ chest
Stage IA Tumors contained in a single compartment
histological diagnosis. ■■ abdomen
■■ pelvis Stage IB Tumors that involve another compartment
■■ bone scintigraphy
Usually, these lesions do not have a true capsule. Instead, there is
Adequate imaging exams of the primary tumor location are also essential for a reactive-tissue pseudocapsule penetrated by microscopic tumor
planning. This information provides understanding of the risk and morbidity of the islands (Boriani, Weinstein and Biagini,1997).
surgical treatment.
Patients with primary malignant tumors without metastatic disease usually benefit
from aggressive surgical treatment with en bloc resection. The objective of the Stage II
surgery, in these cases, is to obtain local control and to attempt to cure the
disease. These surgeries have a high complication rate and should be discussed High-grade tumors without metastasis
together with the patient and the oncologist (Boriani et al., 2010).
The surgical treatment can also be indicated in patients with metastatic disease, Stage IIA Intracompartimental tumors
but for palliative purposes. In these cases, the surgery usually has the following
objectives: Stage IIB Tumors with an extracompartimental extension
■■ spinal cord or radicular decompression
■■ treatment of the instability High-grade tumors are fast growing and there is no time to form
■■ pain control reactive tissue around the entire lesion. Thus, it is commonly seen
as a radiolucent and destructive lesion on simple x-rays and CT
scans. High-grade tumors can have metastatic skip lesions in the
same compartment.

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 5
 2
Possibility of performing the indicated surgery
Although resection with wide margins is desirable in primary malignant tumors
without metastasis, the procedure cannot always be performed due to the local
extension of the disease and its relation to adjacent neurovascular structures.
Presented next are images of a patient with clival chordoma with recurrence after
Stage III the surgical treatment, without metastatic disease
Whenever possible,
malignant tumors
classified as IA, IB, IIA Low or high-grade tumors with distant metastatic disease
and IIB should preferably
be treated with en bloc
resection surgeries with If a wide margin cannot be obtained, marginal resection should be attempted.
wide margins (Chan et al., When the margin is compromised and a resection with an intralesional margin
2009). is performed, there is an increased risk of local recurrence (Hsieh et al., 200;
Rao et al., 2008).

In Stage III tumors, the

surgical treatment can be done for palliative
purposes, as it does not influence the survival
prognosis. The efficacy of adjuvant treatments on
the tumor is what will determine the prognosis,
and not the resection margin.

This factor must be considered when planning the surgical procedure. Limited
surgeries with intralesional resection and stabilization may be more adequate
for patients with a short survival prognosis.
View of a tumor recurrence. Although en bloc resection with wide mar-
Nevertheless, even in patients with metastatic disease, en bloc resection may
gin is desirable, the local extension does not allow for the execution of
be the best choice, especially in patients with the following conditions:
the ideal procedure.
■■ good general status
CT scan with sagittal reconstruction of a clival chordoma
■■ oligometastatic disease
■■ slow-growing primary tumors
■■ low response expected from adjuvant therapies Boriani et al. (1979) developed a local staging system for spine tumors called
WBB, which helps plan the surgical treatment. This classification was modified by
Chan et al. (2009) and is used to date.
Usually, the objective of the en bloc resection for metastasis patients is
not the oncological cure, but the possibility of adequate local control to
avoid complications that may happen before systemic compromise, as a
result of the progression of the disease.

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 6
2
7 6
7 6

8
A 5 8
A
5
7 6
B
A B
A. Extraosseous soft tissue
B 8 5
B 9
C
9 4 B
B. Superficial intraosseous C E
4
9 4
C
C. Deep intraosseous E E
D. Extradural extraosseous 10
D 3 10
D
3
E
3
C D
10
E. Intradural extraosseous C

11 2
11 2
11 2
12 1
12 1
12 1

Tumor located exclusively in the vertebral body between zones 5-8.

The vertebra is divided into twelve rays and five levels from the epidural space Both pedicles can be cut without contaminating the surgical field.
to the extravertebral region of the soft tissue. The longitudinal extension is Schematic showing a case where it is possible to perform the
described according to the number of levels involved. oncological resection
WBB surgical classification 7 6

8
A 5
WBB allows the surgeon to understand the most adequate way to perform the en 7 6

A
bloc resection and decide the best osteotomy points. 8 5
B
B

9
B 4
B
For an en bloc vertebrectomy to be performed, it is necessary that the posterior 9 4 E
arch is cut into two different regions, allowing the posterior and anterior elements 10
D

E
to be removed, maintaining the integrity of the spinal chord. The excision of a D E
10 3 3
vertebral-body tumor can be performed with appropriate margins if the tumor C
10
D
11 C 2
is in the 4-8 or 5-9 zones (Sundaresan, Rosen and Boriani, 2009). The bone
resections can be performed through both pedicles or one pedicle and the 11 2
11 2
contralateral lamina (Bohinski and Rhines, 2003).
12 1
12 1

Tumor invading the right pedicle in zones 5-9. The osteotomy can be done
on the lamina on the right side, and through the pedicle on the left side.

Schematic showing a case in which the oncological resection can be

performed, despite the invasion of one pedicle

There are situations in which the lesion compromises both pedicles, which does
not allow for cutting the posterior arc without violating the tumor while also
preserving the chord integrity. In these cases, it is acceptable to perform an en
bloc resection with an intentional, planned intralesional cut.

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 7
2
7 6 Intralesional Violation of the tumor margins
8
A 5
7 6

8
A 5 B
Marginal Dissection along the pseudocapsule
9 4
B
Dissection out of the pseudocapsule,
9 4 E
Wide removing the tumor with a considerable
E
E
amount of healthy, continuous tissue
D
10 3 D
10 3

11 2
11 2 Surgical planning
12 1
12 1
En bloc resection of thoracic and lumbar-spine lesions can be performed through
the following techniques (Boriani et al., 1997; Sundaresan et al., 2009):
Lesion with a compromised vertebral body and both pedicles in zones 1 ■■ vertebrectomy
through 11. It is necessary to do an intralesional cut to allow for the extraction
■■ sagittal resection
of the posterior vertebral-body elements, preserving the chord.
■■ posterior-arch resection
Schematic showing a case where it is not possible to perform the
oncological resection

Lesions involving zones 2-5 or 7-11 may be submitted to sagittal resection. If the Planning the approach is important to obtain a good outcome and
tumor involves zones 10-3, it is recommended to resect the posterior arch. reduce surgery risks.

The choice of the approach should consider the following points:

■■ region of the compromised vertebrae
■■ presence of extracompartimental extension
■■ relation of the tumor with neurovascular structures
■■ issues related to spine stability and reconstructions
■■ the patient’s clinical status

In thoracic-spine lesions, an en bloc vertebrectomy can be performed through a

single posterior or combined approach.
A chondrosarcoma can be observed
on the T-spine, involving zones 9, 10 View of the resected specimen.
In the lumbar region, the nerve roots must be preserved and the space available
and 11 of two adjacent levels.
to resect the vertebral body is reduced through the isolated posterior approach.
Thoracic-spine chondrosarcoma in a location Thus, a double approach is recommended.
that allows for a sagittal resection

The resection material of a primary bone tumor must always be submitted to

adequate macroscopic and histologic assessment to define if the planned margin
was obtained. The resection will be classified as intralesional, marginal or wide.

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 8
2
A double approach is also recommended when there is contact of the lesion with
vascular structures, or when there is anterior extracompartimental extension, as it
allows for greater safety during the dissection.
■■ The dural sac should be separated from the adhesions in the ventral region
with the aid of a dissector.
■■ In the thoracic region, the ribs in the same level and the level below the
lesion should be resected to allow for the release of the vertebral body.

In summary, the choice of the approach depends on the surgeon’s

experience, the situation of the tumor and the compromised levels (Tomita, En bloc resection through an exclusively posterior
Kawahara, Murakami and Demura, 2006). approach
En bloc vertebral resection through an exclusively posterior approach should
be done whenever possible, as it has the advantage of enabling a direct view
Regardless of picking the single-posterior or double approach, the first step of the of the spinal cord throughout the entire resection and reconstruction. The main
surgery involves the resection of the posterior elements. disadvantage is the greater difficulty in dissecting the great vessels ventral to the
vertebral body.
■■ The midline incision should be done by resecting the biopsy trajectory.
IIn the thoracic region, the ribs must be osteotomized 3 to 4 cm lateral to the
■■ The paravertebral musculature should be separated from the spinous costovertebral joint to enable access to the anterior region of the vertebral body.
processes, laminae and transverse processes. It is necessary to dissect
enough to expose 4-5 cm laterally to the costovertebral joint.
■■ Posterior fixation must be done with pedicular screws in the levels cranial and
caudal to the vertebrectomy site. In the cases where the resection of only
one vertebral body is planned, the fixation of two levels above and two levels
below is enough. If there is the need to resect two or three vertebrae, it is
recommended to do a fixation of three vertebrae above and three below the
resection level (Kawahara, Tomita, Murakami and Demura, 2009). 3–4 cm
■■ If the tumor is located in the posterior elements, en bloc resection is
desirable. For such, it is necessary to perform a laminectomy of the cranial
and caudal levels in the resection zone, in such a way that it allows for the
release of the flavum ligament and articular facets of the adjacent levels.
■■ The pedicles can be carefully cut using a Gigli saw or an osteotome.
■■ If there is no tumor in the posterior elements, the laminectomy can be
performed in fragments.
■■ In the posterior approach, one must be sure to perform the following actions:
■■ cut the posterior longitudinal ligament
The compromise of the vertebra is shown, as well as the resection zone
■■ resect the posterior and lateral portions of the fibrous annulus
of two ribs on either side.
■■ perform the discectomy or vertebral osteotomy above and under
CT reconstruction of the thoracic region
the lesion.

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 9
2
The thoracic spine nerve roots should be ligated and cut bilaterally to facilitate the
procedure and reduce the risk of nerve root avulsion or excessive traction of the
spinal cord
■■ The prevertebral vessels are carefully released through digital dissection.
The resection of the vertebral body will cause severe instability. Thus, it
is extremely important to perform a temporary stabilization with at least
one temporary rod on one of the sides to avoid the risk of neurological
damage due to instability.

Using a Gigli saw or chisel, it is possible to cut the adjacent

View of the separation of the View of the dissection

costal arch pleura to perform the of one thoracic root.
costectomy.

Once the temporary stabilization of the The segmental artery that must
spine is performed, the parietal pleura is be ligated is identified.
separated from the body through blunt
dissection.

View of the ligation of one thoracic root.

Costal resection and articular ligation (Teixeira et al., 2010) View of the
dissection
■■ Once the posterior fixation is done, blunt dissection of both sides is anterior to the
performed between the vertebral body and the pleura, identifying and vertebral bodies.
ligating the segmental arteries. Vertebral body dissection (Teixeira et al., 2010)

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 10
2
■■ vertebrate end plates or disk and cut the anterior longitudinal ligament. The
vertebral body is then free to be carefully rotated and removed through the
defect of the thoracic wall (Kawahara et al., 2009).
Anterior approach during a double approach
To resect the vertebral body en bloc through an anterior approach, it is important
for the posterior elements to have been released through a posterior approach,
including the posterior half of the adjacent discs and the posterior longitudinal
ligament, and the lumbar roots must have been previously completely dissected
and released.

In the lumbar region, the anterior approach can be performed

View of the osteotomy of the vertebral View of the rotation of the

body using the Gigli saw. vertebral body to remove it from View of the
the thoracic wall. dissection of the
lumbar roots.
Phase after a double approach on the lumbar spine
(Uhlendorff, Narazaki, Teixeira, Martins and Teixeira, 2011)

Inferior plate. Superior plate.

Surgical specimen.
Caudal vertebra osteotomy (Uhlendorff et al., 2011)
Body resection (Teixeira et al., 2010)

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 11
2
■■ through an anterolateral extraperitoneal or transperitoneal approach, It is necessary to recall that vertebrectomies are indicated for patients with a long
according to the location of the lesion. In the thoracic region, it is done survival prognosis and methods to improve the rate of fusion should be
through a thoracotomy. used to avoid the failure of the fixation in the long term.
■■ The dissection of the lateral borders of the vertebral bodies and ligation of
■■ The reconstruction of the anterior spine can be performed by using titanium
the segmental arteries of the compromised level should be performed.
cages filled with autologous bone graft or allograft.
■■ The vertebral body is removed and extracted after cutting the anterior
longitudinal ligament and the anterior portion of the discs.
In cases in which an exclusively posterior resection is performed

Reconstruction posterior to the vertebrectomy

Anterior view
of the dural sac
and roots after Allograft tibia.
performing the
corpectomy.
Phase previous to a double approach (Uhlendorff et al., 2011)

Titanium cage filled with autologous graft.

Examples of elements used to reconstruct the anterior spine

Intraoperative image. X-ray.

Surgical specimen (Uhlendorff et al., 2011)

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 12
2
■■ it is possible to compress a titanium cage through the rods. ■■ In patients submitted to reconstruction through an anterior approach,
the use of expandable cages has the advantage of enabling the
positioning of the cage under compression in the defect.

Intraoperative aspect with View of the positioning of the

the right rod in place. titanium cage with autologous graft
for the anterior reconstruction. This cage can be placed through a posterior approach and expanded
to apply compression between the adjacent end plates.
Reconstruction after a vertebrectomy of three vertebrae done exclusively
through a posterior approach (Teixeira et al., 2010) Example of the use of an expandable cage

Palliative surgeries
Palliative surgery is the one performed aiming at preserving the function.
It is indicated for spinal cord decompression or fracture stabilization.
There is no intention of improving survival with palliative surgical treatments.
Thus, intralesional tumor resection is acceptable.

Post-op CT reconstruction

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 13
2
CLINICAL CASE Pre-operative procedures
29-year-old patient with cervical mesenchymal sarcoma and lung metastasis.
Chemotherapy
The indication
Neoadjuvant chemothe-rapy is used initially should be decided
in the attempt to reduce the local extension together with the
of the tumor, allowing for a better surgery and oncologist and
increasing the efficacy of the treatment. should consider the
For illustration purposes, presented next expected response
are images of a patient with sacrum Ewing of the tumor to
sarcoma with extracompartimental extension chemotherapy.
and no distant metastasis (Enneking IIB).

View of the tumor causing spinal cord View of the extensive pulmonary
compression. metastasis.
Coronal T2-sequence MRI Chest x-ray

The patient presented progressive motor deficit. Despite the advanced

oncological disease, the patient was submitted to spinal cord decompression and
occipitocervical fixation.

Aspect of the lesion before the treatment.

View of the occiptocervical fixation Intralesional resection of the tumor in

after the spinal cord decompression. fragments.
Intraoperative image Resected material
View of a considerable reduction of the tumor mass after chemotherapy.
The patient recovered the capacity to walk and was discharged on the third
post-op day, but died three months after the surgery due to progression of Pre and post neo-ancillary-chemotherapy MRI
the pulmonary disease. Despite the short survival, the patient maintained his
autonomy and had his quality of life improved.

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 14
 2
The treatment also aims to treat the micrometastasis that may not have been
identified at the time of the diagnosis. There is also the possibility that the
pathologist will determine the degree of necrosis caused by the chemotherapy
on the resected specimen and can assess the patient’s response to the treatment.

Embolization
Surgical treatment of vertebral spinal tumors is associated with the risk of severe
Pre-operative hemorrhage, especially in the presence of hypervascular tumors.
embolization may be
used to reduce the Findings from the imaging exams may help predict the risk of bleeding
bleeding (Kawahara et al., (Truumees, Dodwad and Kazmierczak, 2010):
2009) and facilitate the
■■ presence of elevated contrast intake by the tumor
resection of the tumor,
■■ presence of intralesional hemorrhage signs Tumor hypervascularization.
improving the vision and
reducing the length of the ■■ fast-growing and locally aggressive tumors Pre-embolization angiography
surgery.

Ideally, it should be performed

between 48 and 72 hours
before the surgical procedure.

After 72 hours, the reduced bleeding benefit may be lost due to the establishment
of new collateral circulation.

Presented next are images of a patient with alveolar sarcoma in the sacrum.

Post-embolization control

To perform en bloc vertebrectomies, Kawahara et al. (2009) also defend pre-op

embolization as a means of reducing the bleeding during the procedure. Some
authors also recommend bilateral embolization of the segmental arteries in the
level of the lesion and the cranial and caudal vertebrae (Fujimaki, Kawahara,
Tomita, Murakami and Ueda, 2006; Kawahara, et al., 2009; Tomita et al., 2006).
The segmental arteries that supply the anterior spinal artery should not be
Apparent lesion.
embolized (Kawahara, et al., 2009).
Axial T2-sequence MRI

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 15
2
One must bear in mind that embolization is not efficient in controlling the epidural
venous plexus bleeding (Truumees et al., 2010). If necessary, the injection
of fibrin glue in the epidural space during surgery may help in controlling the
bleeding (Sekhar, Natarajan, Manning and Bhagawati, 2007).

Embolization is relatively safe. Nevertheless, there is the risk of severe

neurological lesions. Care must also be taken with the indication and
execution of embolization in patients with kidney failure, which may
be worsened by the use of contrast.

Summary:
SURGERY INDICATIONS
The adequate surgical treatment of primary malignant spine tumors is
complex and essential for a good prognosis.

Ideally,
Patients need complete it should
staging and be
theperformed
anatomopathological diagnosis
must be known in between 48 and
order to plan 72 hours
the most adequate treatment. In most
beforeofthe
cases, en bloc resection thesurgical
tumor isprocedure.
associated with a better prognosis.t
least these three tests before deciding on a biopsy and/or surgery.

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 16
3 3. SPECIFIC LESIONS
Osteosarcoma
Osteosarcoma includes several histological subtypes differentiated by the type of
dominant cell which have the production of osteoid tissue in common.
Pre-operative treatment

In most osteosarcoma histological subtypes, pre-op chemotherapy must be

performed with the following objectives:
■■ early treatment of the micromestastases
■■ reduction of the size of the lesion to facilitate the surgical treatment
They are more frequent in the lumbosacral region and
■■ reduction of the risk of local recurrence of the disease
compromise the vertebral body in most cases.

Surgery
Clinic
Surgery plays an essential role in the treatment An en bloc
The main symptom associated with osteosarcoma is pain. It is also common to of osteosarcoma. resection
have signs and symptoms of medullary or radicular compression in the initial
must be
presentation, due to the fast growth of the lesion (Shives, Dahlin, Sim, Pritchard Nonetheless, although the lesion is frequently performed with
and Earle, 1986). diagnosed at an early stage, the prognosis is a wide margin
usually poor due to the technical difficulty in whenever
obtaining a resection with adequate margins possible.
Complementary studies during surgery.

Radiologically, osteosarcoma may have different appearances, according to the

histological type and degree of mineralization of the matrix. Post-op follow up and control
In most cases, it is possible to identify a mineralized matrix with a mixed pattern.
On the other hand, a lithic pattern may be found in some cases, especially in After the surgical treatment, it is important for the patient to be treated with
telangiectatic osteosarcoma (Ilaslan, Sundaram, Unni and Shives, 2004). adjuvant chemotherapy.
The presence of an intense periosteal reaction and extracompartimental extension
of the tumor into the epidural space are common (Ilaslan et al., 2004).
Conventional chemotherapy should not be used as a single treatment and
should be kept for palliative treatment (Sundaresan et al., 2009). It could
also be used as an adjuvant measure to treat residual microscopic disease
Bone scintigraphy with technetium is useful to identify bone metastasis at a
(DeLaney et al., 2005).
distance and scattered lesions.

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 17
3
The assessment of the osteosarcoma treatment outcome is difficult due to the Complementary studies
rarity of the disease. If compared to limb osteosarcoma, osteosarcoma located
in the spine is associated with a worse prognosis. The factors related to a worse In the imaging exams, the lesions are usually mixed and with intralesional
prognosis are as follows: calcification.
■■ presence of metastasis at the time of the diagnosis
■■ large tumors
■■ sacral location

Chondrosarcoma
Chondrosarcoma is a rare primary malignant tumor that produces a cartilaginous
matrix. It presents varied clinical behaviors according to the histological degree.

Chondrosarcoma may appear anywhere in the spine, but is

predominantly found in the thoracic spine (York et al., 1999).
There is a predominance of lesions located on the vertebral body, but
chondrosarcomas originated from benign chondral lesions which usually originate
from the posterior elements (York et al., 1999). When located in the sacrum, they
frequently present an eccentric location on the upper portion of the sacrum.
View of an eccentric lesion with areas of intralesional calcification.
CT scan of a sacrum chondrosarcoma
In low-grade lesions, cortical bone remodeling or cortical expansion can be found.
In high-grade lesions, the cortex may erode and form a large extraosseous mass
with diffuse calcification areas.

Clinic Pre-operative treatment

The most common symptom of chondrosarcoma is pain in the site of the tumor.
The symptoms are usually insidious and may be present for months or years
(Stuckey and Marco, 2011). In approximately one-third of patients, it is possible
to identify a palpable mass, and in approximately 25% of the cases, it is possible
to identify neurological signs and symptoms.
Chondrosarcoma is a tumor highly resistant to radiotherapy, which should not
be used as an exclusive treatment (Boriani, Saravanja, Biagini and Fisher, 2009).
Most chondrosarcomas are not sensitive to chemotherapy and the neoadjuvant
treatment is not recommended (Stuckey and Marco, 2011).

Surgery Considering the lack

of response to radio
Surgery is an essential tool in the treatment and chemotherapy,
of these tumors. chondrosarcoma is
mainly a disease for
surgical treatment.

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 18
 3
The proximity of the tumor to neurovascular structures may make the en bloc Chordoma
resection more difficult. When the en bloc resection can be performed with wide
or marginal margins, there are the following advantages (Boriani et al., 2009): Chordoma is a malignant primary tumor originating from notochord remnants.
Usually, it involves the sacrococcygeal or spheno-occypital region of the axial
• reduction of local recurrence rates skeleton.
• greater interval free from the disease
• reduction in the mortality related to the disease
Differently from other primary malignant tumors, chordoma usually
does not happen out of the spine. It is a slow-growing tumor with a low
tendency for metastasis.
When the surgery is intralesional, there is recurrence and progression of
the disease in most cases (Boriani et al., 2000; Katonis et al., 2011).

Clinic

It could be slow growing with a gradual installment of the signs and symptoms,
Post-op follow up and control being common for the tumors to be large at the time of the diagnosis.

In patients with an adequate oncological margin, adjuvant treatment may not be Non-specific, slow evolving lower-back pain is the most common symptom in
necessary. sacrum lesions. Besides pain, constipation symptoms and spasms are frequent. In
most patients, the tumor can be palpated in a rectal exam.

High doses of radiotherapy (60 to 65 Gy) may be indicated as an The symptoms usually appear early in chordomas of the mobile spine. Most
adjuvant treatment in cases of incomplete resection or intralesional patients complain of axial pain, usually with a radicular component.
margins (Boriani et al., 2009; Foweraker et al., 2007).

Complementary studies
Nonetheless, the patient has a shorter survival when compared to patients who
The chordoma diagnosis through simple x-rays is not easy at the initial stages of
underwent en bloc resection with a free margin (Boriani et al., 2000; Katonis et
The post-op follow up the disease. Unless there are many signs of calcification within the lesion, the
al., 2011).
must be extended. Most soft-tissue mass associated with the tumor may not be recognized.
recurrences happen
The treatment with chemotherapy also plays a limited role after surgery. Some
before five years from the
forms of chondrosarcoma, such as mesenchymal chondrosarcoma, present some
treatment, but there are The CT and MRI aid in the assessment. In the sacrum, it is common to
response to chemotherapy (Katonis et al., 2011).
cases of late recurrence, find a median pre-sacral mass, characteristic of the disease.
up to ten years after the
When there is post-op recurrence after a surgery with an intralesional margin,
surgery (Katonis et al.,
there may be an indication for a new surgery with the intent of obtaining adequate
2011).
margins (Katonis et al., 2011).
In the MRI, the chordoma usually presents a lobulated mass with a myxoid or
mucinoid consistency.

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 19
 3
Post-op follow up and control

There is no efficient chemotherapy treatment against chordoma.

Adjuvant treatment with high doses of radiotherapy may be considered in

chordomas in which the incomplete resection or an intralesional margin was
performed (Boriani et al., 2009; Moojen, Vleggeert-Lankamp, Krol and
Dijkstra, 2011).
■■ Distant metastases happen in approximately 25% of the cases (Moojen et
al., 2011).
■■ The average 10-year survival rate in patients with chordoma varies between
40% and 52% (Moojen et al., 2011).
■■ Craniocervical-transition chordomas have a worse prognosis due to the
technical difficulty in performing a wide resection and its proximity to vital
neurovascular structures.

Multiple myeloma
Multiple myeloma is a tumor characterized by the malignant transformation of
View of a very bulky pre-sacral mass.
B-cell-derived plasmocytes found in the bone marrow.
Sagittal T2 sequence MRI of a sacrum chordoma

Clinic
Pre-operative treatment
The clinical status of multiple myelomas is varied. Patients may present the
Chordomas have a poor response to radiotherapy and it is not indicated as the following symptoms:
primary treatment of the lesion if an adequate surgery can be done (Boriani et al., ■■ fatigue
2009). There are no efficient chemotherapy schemes to treat the disease.
■■ dyspnea
■■ nauseas
The best treatment for
chordoma is en bloc Surgery ■■ vomiting
resection with wide ■■ constipation
Surgery is essential in treating the tumor.
margins. ■■ thirst
Like with chondrosarcoma, when an adequate margin is obtained, there are the ■■ polyuria
following advantages (Boriani et al., 2009): ■■ lethargy
■■ reduction of local recurrence rates ■■ recurrent infections
■■ greater interval free from the disease
■■ reduction in the mortality related to the disease

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 20
3
■■ bleeding related with some of the following causes: Radiographic studies should be performed with x-rays of the following regions:
■■ ­anemia ■■ skull
■■ hypercalcemia ■■ total spine
■■ ­kidney failure ■■ pelvis
■■ ­thrombocytopenia ■■ ribs
■■ ­hyperviscosity syndrome ■■ long bones

In the musculoskeletal system, multiple myeloma attacks mainly the axial skeleton
and the proximal portion of long bones. Approximately two thirds of patients The bone marrow biopsy is important to confirm the diagnosis.
present pain in the bones. Pain is frequently related to movement and activities,
improving during rest. The presence of pain at night is uncommon. Pain is usually
the result of a pathological fracture, or could be a sign of instability.
In the simple x-ray, it is common to find multiple osteolitic bone lesions with
Some patients may suffer vertebral fractures without pain. Moreover, they may or without fractures. Some patients may present signs of diffuse osteoporosis
develop progressive kyphotic deformity and have a reduction in height. without identifiable lithic lesions. Over 50% of patients present compression
vertebral fractures, due to tumor infiltration or osteoporosis (Kyle, 1975).
Medullary or radicular compression may occur in the evolution of multiple
myeloma due to the following causes: The CT or MRI allows for a better understanding of the extent of the disease.

■■ pathological fracture
■■ deformity
■■ growth of the epidural mass

Complementary studies

For patients with the suspicion of multiple myeloma, the following exams should
be requested:
■■ complete blood work-up
■■ renal function assessment
■■ serum and urinary protein electrophoresis
■■ serum immunoglobulin dosage
■■ sedimentation speed
■■ electrolytes

View of multifocal lithic lesions, compatible with the multiple

myeloma diagnosis.
CT scan, sagittal reconstruction

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 21
 3
Post-op follow up and control
Bone scintigraphy with technetium is a useful exam to assess polyostotic
diseases, but in multiple myeloma false positives may appear. Patients with multiple myeloma should be periodically monitored due to the
risk of fractures. The use of bisphosphonates can reduce the frequency of bone
complications, including vertebral fractures (Berenson et al., 1998; Kyle et
al., 2007). Such agents inhibit osteoclastic activity and reduce or inhibit bone
Pre-operative treatment
reabsorption.
Multiple myeloma is highly sensitive to chemo and radiotherapy, which allows for
adequate control of the pain without the need for surgery, in most cases.
Radio and chemotherapy
are the most indicated Plasmacytoma
treatments. Surgery
Plasmacytoma is a monoclonal neoplastic mass that may occur in bone or soft
As it is a multifocal disease highly sensitive to chemo and radiotherapy, the surgical tissues. The axial skeleton is the most common compromised location in solitary
treatment has no impact in the oncological treatment per se. Even in patients bone plasmacytoma.
with medullary compression due to an epidural mass, exclusive radiotherapy
treatment is adequate, as long as there are no signs of instability.
It may represent a
frustrated form of
Therefore, the indication for surgery is based on treating the instability. myeloma or can be a
Surgery is also useful in patients at a high risk of pathologic fractures. true localized lesion.

Persistent and intense pain, which worsens with movement, may be a fracture-
risk sign. The fracture risk also increases with the extension of bone compromise.
In the case of destruction of more than 50% of the diameter of the bone, the
estimated risk of a pathological fracture is of 80%.
In patients who suffer progressive deformity or incapacity due to pain resulting
from instability, the treatment with vertebral reinforcement through vertebroplasty
or kyphoplasty allows for the relief of pain with a low risk of having complications.
Vertebral reinforcement procedures may provide fast pain relief and allow for the
partial recovery of the sagittal height and alignment when well indicated (Khanna,
Neubauer, Togawa, Kay Reinhardt and Lieberman, 2005). The contraindications
pertaining to the procedure include fracture of the posterior wall and medullary
compression.
Clinic
The most common solitary bone-plasmacytoma symptom is local pain. The
presence of pathological fractures in the spine may cause intense pain and
spasms of the paravertebral musculature.
Patients with a severely compromised spine may also present signs and
symptoms of radicular or medullary compression. Occasionally, plasmacytoma
may be asymptomatic and recognized in imaging exams performed for other
reasons (Dimopoulos, Moulopoulos, Maniatis and Alexanian, 2000).

The presence of disseminated bone lesions throughout the spine and

osteoporosis make instrumentation a challenge. Circumferential reconstruction
techniques and the use of cement to reinforce the posterior fixation may help
reduce the risk of mechanical failure.

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 22
 3
Complementary studies Surgery

In the suspicion of solitary plasmacytoma, the patient must be submitted to Surgical treatment of solitary spine plasmacytoma is not indicated to improve the
several exams (Soutar et al., 2004): oncological outcome (Knobel et al., 2006).
■■ Lab work-up:
■■ ­complete blood work Surgical treatment indications are as follows:
■■ ­electrolytes ■■ presence of a pathological fracture with signs of instability;
■■ ­serum immunoglobulin dosage ■■ medullary compression by bone fragments or vertebral deformity;
■■ ­serum and urinary protein electrophoresis ■■ persistent pain.
■■ imaging exams for radiographic tracing:
■■ ­long bones
■■ ­spine
■■ ­skull and pelvis
■■ ­myelogram

To diagnose solitary bone plasmacytoma, it is necessary to satisfy certain criteria

(Soutar et al., 2004; Dimopoulos et al., 2000). There should be a single bone
destruction area by monoclonal plasmocytes. The infiltration of plasmocytes
in the bone marrow should not exceed 5% of the total nucleated cells and
there should not be anemia, hypercalcemia or renal dysfunction attributed to
the myeloma. In the case of monoclonal paraproteins in the blood or urine, they
should be in a low concentration.

In the imaging exams, the plasmacytoma lesion is purely lithic. The spine CT
and MRI are more reliable than simple x-rays to understand the extension of the
disease. In the MRI, there is the presence of a focal bone-marrow substitution
area. The signal intensity tends to be similar to that of the musculature in the
T1-weighted sequence and present a hypersignal in the T2-weighted sequence.
In some cases, there is also an epidural component that may cause medullary or
radicular compression symptoms.

Pre-operative treatment
The treatment of
choice for isolated View of a pathological fracture due to plasmacytoma with
The adjuvant methods are very important for the treatment of this disease.
bone plasmacytoma is medullary compression.
radiotherapy. The treatment field should include normal tissue margins (Dimopoulos et al., Sagittal T2-sequence MRI
2000). In the spine, it is possible to include one normal vertebra above and
below the lesion.

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 23
3
Summary:
SPECIFIC LESIONS
Knowledge of the biologic behavior and response of primary malignant
tumors to chemo and radiotherapy is important to plan treatment
strategies and understand the prognosis. Due to the complexity of the
disease, the treatment should always be multidisciplinary and involve the
oncologist to decide what the best treatment is.

View of 360° decompression through an exclusive posterior approach and

reconstruction with a titanium cage and posterior-approach fixation.
Intraoperative image

Post-op follow up and control

After adequate radiotherapy treatment, the patients present relief from the
symptoms. In most cases, local control is obtained, being defined as long-term
clinical and radiographic stability (Dimopoulos et al., 2000; Knobel et al., 2006).

On the other hand, approximately 50% of the patients develop multiple myeloma
within two to three years on average (Dimopoulos et al., 2000; Knobel et al.,
2006; Soutar et al., 2004).

Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira 24
REFERENCES
Berenson, J. R., Lichtenstein, A., Porter, L., Dimopoulos, M. A., Bordoni, R.,
George, S. et al. (1998) Long-term pamidronate treatment of advanced
multiple myeloma patients reduces skeletal events. Myeloma Aredia Study
Group. J Clin Oncol, 16(2), 593-602.
Bohinski, R. J. and Rhines, L. D. (2003) Principles and techniques of en bloc
vertebrectomy for bone tumors of the thoracolumbar spine: an overview.
Neurosurgical focus, 15(5), E7.
Boriani, S., Bandiera, S., Donthineni, R., Amendola, L., Cappuccio, M., De Iure,
F., et al. (2010) Morbidity of en bloc resections in the spine. Eur Spine J,
19(2), 231-41.
Boriani, S., De Iure, F., Bandiera, S., Campanacci, L., Biagini, R., Di Fiore, M. et
al. (2000) Chondrosarcoma of the mobile spine: report on 22 cases. Spine,
25(7), 804-812.
Boriani, S., Saravanja, D., Biagini, R., and Fisher, C. G. (2009) Challenges of
local recurrence and cure in low grade malignant tumors of the spine. Spine,
34(22), 25-32.
Boriani, S., Weinstein, J. N. and Biagini, R. (1997) Primary bone tumors of the
spine: terminology and surgical staging. Spine, 22(9), 1036.
Chan, P., Boriani, S., Fourney, D. R., Biagini, R., Dekutoski, M. B., Fehlings,
M. G. et al. (2009) An assessment of the reliability of the Enneking and
Weinstein-Boriani-Biagini classifications for staging of primary spinal tumors
by the Spine Oncology Study Group. Spine (Phila Pa 1976), 34(4), 384-391.
DeLaney, T. F., Park, L., Goldberg, S. I., Hug, E. B., Liebsch, N. J., Munzenrider,
J. E. et al. (2005). Radiotherapy for local control of osteosarcoma. Int J Radiat
Oncol Biol Phys, 61(2), 492-498.
Dimopoulos, M. A., Moulopoulos, L. A., Maniatis, A. and Alexanian, R. (2000).
Solitary plasmacytoma of bone and asymptomatic multiple myeloma. Blood,
96(6), 2037-2044.
Enneking, W. F., Spanier, S. S., and Goodman, M. A. (1980). A system for the
surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res, 153,
106-20.
Primary Malignant Bone Tumors Surgery. Author: Dr. William Gemio Jacobsen Teixeira
Foweraker, K. L., Burton, K. E., Maynard, S. E., Jena, R., Jefferies, S. J., Laing, R.
J. C., et al. (2007). High-dose radiotherapy in the management of chordoma
and chondrosarcoma of the skull base and cervical spine: Part 1--Clinical
outcomes. Clin Oncol (R Coll Radiol), 19(7), 509-516.
Fujimaki, Y., Kawahara, N., Tomita, K., Murakami, H. and Ueda, Y. (2006).
How many ligations of bilateral segmental arteries cause ischemic spinal
cord dysfunction? An experimental study using a dog model. Spine, 31(21),
E781-E789.
Hsieh, P. C., Xu, R., Sciubba, D. M., McGirt, M. J., Nelson, C., Witham, T. F. et al.
(2009). Long-term clinical outcomes following en bloc resections for sacral
chordomas and chondrosarcomas: a series of twenty consecutive patients.
Spine, 34(20), 2233-2239.
Ilaslan, H., Sundaram, M., Unni, K. K. and Shives, T. C. (2004). Primary
vertebral osteosarcoma: imaging findings. Radiology, 230(3), 697-702.
Katonis, P., Alpantaki, K., Michail, K., Lianoudakis, S., Christoforakis, Z.,
Tzanakakis, G. et al. (2011) Spinalchondrosarcoma: a review. Sarcoma, 2011,
378957.
Kawahara, N., Tomita, K., Murakami, H. and Demura, S. (2009) Total en bloc
spondylectomy for spinal tumors: surgical techniques and related basic
background. Orthop Clin North Am, 40(1), 47-63.
Khanna, A. J., Neubauer, P., Togawa, D., Kay Reinhardt, M. and Lieberman,
I. H. (2005) Kyphoplasty and vertebroplasty for the treatment of spinal
metastases. Supportive Cancer Ther, 3(1), 21-25.
Knobel, D., Zouhair, A., Tsang, R. W., Poortmans, P., Belkacémi, Y., Bolla, M.
et al. (2006) Prognostic factors in solitary plasmacytoma of the bone: a
multicenter Rare Cancer Network study. BMC Cancer, 6, 118.
Kyle, R. A. (1975) Multiple myeloma: review of 869 case. Mayo Clin Proc,
50(1), 29–40.
Kyle, R. A., Yee, G. C., Somerfield, M. R., Flynn, P. J., Halabi, S., Jagannath, S.
et al. (2007) American Society of Clinical Oncology 2007 clinical practice
guideline update on the role of bisphosphonates in multiple myeloma. J Clin
Oncol, 25(17), 2464-2472.
25
Moojen, W. A, Vleggeert-Lankamp, C. L., Krol, A. D. G. and Dijkstra, S. P. D.
(2011) Long-term results: adjuvant radiotherapy in en bloc resection of
sacrococcygeal chordoma is advisable. Spine, 36(10), E656-E661.
Rao, G., Suki, D., Chakrabarti, I., Feiz-Erfan, I., Mody, M. G., McCutcheon, I. E.
et al. (2008) Surgical management of primary and metastatic sarcoma of the
mobile spine. Journal of neurosurgery. Spine, 9(2), 120-8.
Sekhar, L. N., Natarajan, S. K., Manning, T. and Bhagawati, D. (2007) The use
of fibrin glue to stop venous bleeding in the epidural space, vertebral venous
plexus, and anterior cavernous sinus: technical note. Neurosurgery, 61(3),
E51.
Uhlendorff, E. V., Narazaki, D. K., Teixeira, W. G. J., Martins, S. K.
and Teixeira, M. J. (2011) En bloc resection of a L3 chordoma by a
double approach. AOSpine Case Base. Visited on March 1st, 2012 at
http://aospine.org/casedetails.aspx?id=4106cdc9-e1f5-43c4-a8c4-
07ca12152f3e&from=CASEPG_CASEINFO&extend=id|equal|4106cdc9-e1f5-
43c4-a8c4-07ca12152f3e|and|from|equal|PG_HOME_CASES
York, J. E., Berk, R. H., Fuller, G. N., Rao, J. S., Abi-Said, D., Wildrick, D. M. et
al. (1999) Chondrosarcoma of the spine: 1954 to 1997. J Neurosurg, 90(1),
73-78.

Shives, T. C., Dahlin, D. C., Sim, F. H., Pritchard, D. J. and Earle,

J. D. (1986) Osteosarcoma of the spine. J Bone Joint Surg Am, 68, Project coordination
660-668. Néstor Fiore
Luciana Braga Garcia
Soutar, R., Lucraft, H., Jackson, G., Reece, A., Bird, J., Low, E. et al. (2004)
Guidelines on the diagnosis and management of solitary plasmacytoma of Instructional design and educational review
bone and solitary extramedullary plasmacytoma. Clin Oncol (R Coll Radiol), María Alejandra Zangara
16(6), 405-13. María Eugenia Bregliano

Stuckey, R. M. and Marco, R. (2011) Chondrosarcoma of the mobile spine Project and graphic design
and sacrum. Sarcoma, 2011, 274281. André Secundino

Sundaresan, N., Rosen, G. and Boriani, S. (2009) Primary Malignant Tumors Illustrations
of the Spine. Orthop Clin North Am, 40, 21-36. Gustavo Francesconi

Teixeira, W. G. J., Terra, R., Lara Jr., N., Narazaki, D. K., Ribas, E. S. C., Teixeira,
M. J. et al. (2010) Triple en bloc spondylectomy from a single posterior
approach for a metastatic spinal chondrosarcom. AOSpine Case Base. Visited
on March 1st, 2012 at http://aospine.org/casedetails.aspx?id=04a5f182-
afa4-41a1-84bc-43d9fe74ed78&from=CASEPG_CASEINFO&extend=id|equal
|04a5f182-afa4-41a1-84bc-43d9fe74ed78|and|from|equal|PG_HOME_CASES

Tomita, K., Kawahara, N., Murakami, H. and Demura, S. (2006) Total en bloc
spondylectomy for spinal tumors: improvement of the technique and its
associated basic background. J Orthop Sci, 11(1), 3-12.

Truumees, E., Dodwad, S. N. and Kazmierczak, C. D. (2010) Preoperative

embolization in the treatment of spinal metastasis. J Am Acad Orthop Surg,
18(8), 449-453.

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