Clinical
The most common clinical
presentation associated with all spine tumors is
back pain that causes the patient to seek medical
attention. Back pain is the most frequent symptom
for patients with either benign or malignant
neoplasms of the spine. Neurologic deficits
secondary to compression of the spinal cord or
nerve roots also can be part of the presentation.
The degree of neurologic compromise can vary from
slight weakness or an abnormal reflex to complete
paraplegia, depending on the degree of
encroachment. The loss of bowel or bladder
continence can occur from neurologic compression
or can be secondary to a local mass effect from a
tumor in the sacrococcygeal region of the spine,
as occurs in chordomas. Systemic or constitutional
symptoms tend to be more common with malignant or
metastatic disease than in benign lesions.
Lab studies
For these patients, workup
should include a complete blood count and
differential, a basic serum chemistry profile,
erythrocyte sedimentation rate, or C-reactive
protein to help distinguish between neoplastic and
infectious processes. Elevations in serum calcium
or alkaline phosphatase also can provide evidence
for neoplastic bone processes. Specific studies
such as serum electrophoresis or urine
electrophoresis also can be performed to evaluate
the likelihood of multiple myeloma or plasmacytoma.
Imaging studies
Imaging studies for the workup
of spine tumors include plain x-rays, CT scan, MRI,
and a technetium bone scan.
The first-line imaging study
should be plain x-ray to evaluate the trabecular
architecture of the spine. Anteroposterior (AP),
lateral, and oblique views may be necessary to
evaluate the lesion. These studies should be
evaluated for what the tumor is doing to the bone
and, conversely, for what the bone is doing to the
tumor. The blastic or lytic nature of the lesion
should be noted. The general location of the
lesion within the bone, the integrity of the
cortex, and the presence of fractures or soft
tissue masses are important findings.
The ultimate way to make the
diagnosis and ascertain the specific tumor type is
by performing a biopsy of the spine lesion after
all radiographic studies have been completed.
Biopsies can be performed with open or by
percutaneous image-guided technique. Percutaneous
needle biopsies may not supply adequate tissue for
the diagnosis of a primary tumor of the spine.
The basic principles of biopsy
technique also apply to tumors of the spine; the
surgeon performing the biopsy should take the most
direct route to the tumor with the least potential
to contaminate adjacent compartments. The biopsy
tract should be placed in line with the future
incision site for surgical resection of the tumor
so that the biopsy tract can be excised with the
specimen en bloc. Meticulous hemostasis must be
obtained, and a drain must be placed to prevent
hematoma formation, which can dissect the soft
tissue planes and contaminate adjacent
compartments. The drain should exit the skin in
line with the incision so that it, too, can be
excised with the final specimen.
Histologic findings
The histologic findings vary
according to the tumor types as described above.
The following list revisits the primary tissue
type associated with some of the tumors of the
spine:
- Bone producing tumors of the
spine
- Osteoid osteoma - Benign
and locally self limited
- Osteoblastoma - Benign but
locally expansile and aggressive
The spine consists of 33 vertebrae that form the
bony spinal column. The spinal column can be
divided into the cervical, thoracic, lumbar, and
sacrococcygeal regions. Although morphologically
distinct, each vertebra in the subaxial cervical,
thoracic, and lumbar spine has a complex
architecture, consisting of a vertebral body,
pedicles, laminae, and spinous and transverse
processes. The bony canal provides protection and
support to the fragile spinal cord and nerve roots
within the dural sac. The soft tissues surrounding
the bony spine vary by location from the thick
dorsal paraspinous musculature to the vital organs
and vessels within the mediastinal, thoracic,
peritoneal, and retroperitoneal spaces.
The relevant anatomy discussed
above is frequently the limiting factor when
determining contraindications to surgical excision
of spine tumors. The morbidity of the tumor, the
tumor’s malignant potential, and the patient's
overall prognosis must be compared to the
morbidity and potential mortality of radical
resection of a tumor near the spinal cord, the
aorta, or the heart. The degree of associated
blood loss and the overall health of the patient
also must be taken into consideration when
considering a resection. If the patient is known
to have metastatic or systemic tumor involvement,
this may be a contraindication to radical
resection of a paraspinous tumor, which may render
the patient paralyzed.
Weinstein, Boriani, and Biagnini
have developed a descriptive staging system for
spine tumors based on the principles of Enneking
staging system for primary bone tumors of the
extremities. In their staging system for the
spine, the transverse extension of the vertebral
tumor is described with reference to 12 radiating
zones numbered 1-12 in a clockwise order, and to 5
concentric layers A to E, from the paravertebral
extraosseous compartments to the dural
involvement. The longitudinal extent of the tumor
is recorded according to the levels involved.
Based on an understanding of the biologic behavior
of the tumor, the oncologic staging aids the
surgeon to decide what surgical margin provides
the best chance for complete tumor resection and
possible cure. This system is complex and
sometimes difficult to apply clinically.
Enneking classification of
benign lesions applies to benign spine tumors.
Lesions can be latent (stage 1), active (stage 2),
or aggressive (stage 3). Stage 1 lesions are
usually asymptomatic and are discovered
incidentally. Stage 2 lesions usually present with
symptoms; most commonly, pain is in the area of
the lesion. Stage 3 lesions are locally aggressive
and can actually metastasize.
Enostosis
Also termed a bone island,
enostosis is a mass of calcified medullary defects
of lamellar compact bone with haversian systems
found within the cancellous portion of the bone.
Enostosis occurs most frequently in the thoracic
and lumbar spine, usually between T1 and T7 and
between L1 and L2. Enostosis is one of the most
common lesions to involve the spine. Enostoses are
usually stage 1 lesions and are discovered
incidentally. Most remain stable, but some may
slowly increase in size. Resnik et al determined
the incidence of enostosis to be approximately 14%
in cadavers.
Radiographically, enostoses are
circular or oblong osteoblastic lesions with a
spiculated margin, which gives it the appearance
of thorny periphery. An abrupt transition from
normal to the sclerotic bone is exhibited on the
x-ray. Bone scan findings are usually normal, and
MRI demonstrates low signal intensity with normal
surrounding intensity. Enostosis sometimes can be
confused with osteoblastic metastatic disease.
Enostosis can be differentiated by lack of
activity on bone scan, by the normal appearance of
adjacent bone, by its thorny margins, and by lack
of a primary tumor for metastasis. If the
enostosis exhibits an increase in diameter of
greater than 25% in 6 months, a biopsy should be
performed.
Osteoid osteoma
Osteoid osteomas usually present
in children aged 10-20 years with a male
predominance. They involve the axial skeleton only
10% of the time. In the spine, 59% of osteoid
osteomas are found in the lumbar region, 27% in
the cervical region, 12% in the thoracic region,
and 2% in the sacral region.
Osteoid osteomas are usually
stage 2 lesions and are actively symptomatic.
Osteoid osteomas can result in painful scoliosis,
radicular pain, gait disturbances secondary to
pain and splinting, and muscular atrophy. Symptoms
usually are relieved or ameliorated by
nonsteroidal anti-inflammatory drugs (NSAIDs) or
salicylates. In the spine, osteoid osteomas occur
75% of the time in the posterior elements, either
the pedicles, facets, or laminae. Osteoid osteomas
occur 7% of the time in the vertebral body and 18%
of the time in the transverse and spinous
processes.
On plain x-ray, osteoid osteomas
appear as a round or oval radiolucent nidus, with
a surrounding rim of sclerotic bone. An area of
central calcification may be present, but this
classic appearance may be obscured by complex
spinal architecture. On bone scan, marked
increased uptake by the nidus is demonstrated, and
a double intensity pattern may exist. CT scan is
the criterion standard for radiographic diagnosis.
The nidus is well-defined area of low attenuation
with or without central calcification surrounded
by an area of sclerosis.
The nidus is usually smaller
than 1.5-2.0 cm, composed of microscopic
well-organized trabecular bone with vascular
fibrous connective tissue stroma surrounded by
reactive cortical bone.
Treatment is accomplished by
resection of the nidus by open surgical approach
or by percutaneous CT scan-guided resection.
Recently, percutaneous radiofrequency ablation of
the nidus has been performed with acceptable
results.
Osteoblastoma
Histologically similar in
appearance to the osteoid osteoma, the
osteoblastoma is behaviorally very different.
Demographically, it occurs in young patients in
the second or third decade of life. A 2:1
male-to-female predominance exists. The lesion is
distributed equally in the cervical, thoracic, and
lumbar segments of the spine. The posterior
elements are involved in 55% of cases, but the
tumor can extend to the vertebral body in 42% of
cases.
Patients typically complain of
dull localized pain and paresthesias, paraparesis,
and, if the tumor is large enough and encroaching
on the spinal cord, paralysis.
Osteoblastomas are expansile
lesions with multiple small calcifications and a
peripheral scalloped and sclerotic rim. In more
aggressive lesions, osseous expansion, bone
destruction, infiltration of the surrounding
tissue, and intermixed matrix calcification are
present. Fifty percent of osteoblastomas are
radiolucent, and 20% are osteoblastic. Marked
radionucleotide uptake is exhibited on bone scan.
CT scan demonstrates areas of mineralization,
expansile bone remodeling, and sclerosis or a thin
osseous shell at its margins. MRI is nonspecific
but is the criterion standard to assess the effect
of the tumor on the cord and surrounding tissues.
Osteoblastomas are typically
larger than 2.0 cm in diameter with histologic
features of interconnecting trabecular bone and
fibrovascular stroma similar to, but not as well
organized as, osteoid osteoma. They can have an
aneurysmal bone cyst component in 10-15% of cases.
Wide local resection is the
treatment of choice whenever possible. This
sometimes is limited by the proximity of vital
vessels or neural tissue in the spine. A 10-20%
recurrence rate exists for conventional
osteoblastomas. Aggressive osteoblastomas have a
recurrence rate of approximately 50% if wide
margins are not attained. These tumors are not
radiosensitive.
Aneurysmal bone cysts
Aneurysmal bone cysts (ABCs)
typically affect young patients, with 80%
occurring in people younger than 20 years. The
spine is involved 12-30% of the time. The thoracic
spine is affected most commonly, followed by the
lumbar and cervical spines. Sacral involvement is
rare.
ABCs of the spine usually
present as expansile areas of bone remodeling in
the posterior elements. Extension into the
vertebral body can occur 75% of the time. The
lesion may have a thin outer periosteal rim of
bone, and septations within the mass may be
apparent. The mass may extend into adjacent
vertebrae, discs, ribs, and paravertebral soft
tissues. The bone scan exhibits peripheral
increased uptake with a central "cold area"
creating a donut sign. If angiography is
performed, the mass is found to be hypervascular
75% of the time. CT scans and MRIs are used to
confirm the cystic nature of the lesion as well as
the tumor extension into surrounding tissues and
the tumor’s relationship to the spinal canal.
Single or multiple fluid/fluid levels sometimes
can be visualized on MRI. MRIs with gadolinium
demonstrate enhancement of the periosteal rim and
septations and not the cystic spaces.
ABCs are characteristically
multiloculated blood-filled spaces that are not
lined by endothelium. They are not vascular
channels. Primary ABCs are believed to result from
micro-trauma to the bone with local circulatory
disturbance. Other underlying neoplasms such as
giant cell tumors (GCTs), osteoblastomas,
chondroblastomas, or osteosarcomas produce
secondary ABCs. These other neoplasms produce
venous obstruction and possible arteriovenous
malformations and set the stage for ABC formation.
Most ABCs are considered primary (65-95%).
Because of the locally
aggressive behavior of spinal ABCs, their
treatment can be problematic. The severe morbidity
that can be associated with complete resection is
caused generally by danger to surrounding vascular
or neural elements. ABCs can have a recurrence
rate of 20-30% or higher, depending on the degree
of resection. Preoperative embolization therapy
and radiation may help shrink the tumor’s size and
decrease the amount of intraoperative blood loss
associated with resection.
Osteochondroma
Osteochondromas make up 4% of
all solitary spine tumors. They also are commonly
referred to as exostosis. Spinal lesions are
encountered in 7-9% of patients with multiple
hereditary exostoses (MHE). Osteochondromas occur
in patients aged 20-30 years. Patients with MHE
tend to develop the osteochondroma at a younger
age; they also tend to experience neurologic
deficits and myelopathy more frequently (77% of
the time) than the patient with solitary
osteochondroma (34%). A male predominance exists.
Osteochondromas are more common in the cervical
spine, especially at C2. The posterior elements
usually are involved. The lesions are believed to
arise secondary to trapping of the physeal
cartilage outside the growth plate during skeletal
development.
Making the diagnosis of
osteochondroma in the spine on plain radiography
can be difficult unless the lesion is large and
protruding posteriorly from a spinous process. In
fact, 15% of patients with osteochondromas of the
spine have normal appearing x-rays. CT scan is the
study of choice to detect the exostosis and
determine its relationship to the surrounding soft
tissue and spinal canal. T1-weighted MRI scans
reveal a central area of high signal intensity,
which represents yellow marrow. This area has
intermediate intensity on T2-weighted images. The
cortex of the exostosis has low signal intensity.
The hyaline cartilage cap of the exostosis is best
evaluated with MRI and appears with low signal
intensity on T1 and high intensity on T2. The
cartilage cap should be less than 2 cm in adults.
Lesions with cartilage caps greater than 2 cm
should be suspected of malignant transformation to
chondrosarcoma.
Qualitatively, the bone
composing an osteochondroma is normal. Abnormal
bone growth occurs at and as a result of the
cartilage cap. A continuity of the lesion with the
marrow and cortex of the underlying bone is
present. The exostosis may be sessile or
pedunculated.
Complete surgical resection is
usually curative. Clinical symptoms improve in 89%
of patients following removal of the exostosis.
Incomplete resection can lead to recurrence of the
lesion.
Giant cell tumor
GCTs of the spine comprise only
7% of all GCTs in the body. The spine is the
fourth most common site for the occurrence of GCTs
in the body. Most GCTs in the spine occur in the
sacrum followed by the thoracic, cervical, and
lumbar regions, respectively. GCTs are more common
in women and occur in the third to fifth decades
of life. GCTs can increase dramatically in size
during pregnancy secondary to hormonal influences.
The symptoms of spinal GCT include pain with
radicular pattern. With neurologic impingement,
weakness and sensory deficits also can be
manifest.
Spinal GCTs are usually
radiolucent and expansile lesions. They do not
exhibit active matrix production. When present in
the sacrum, these lesions are large with
destruction of the sacral foraminal lines on plain
x-rays. GCTs usually can involve both sides of the
midline and can extend past the sacroiliac joints
bilaterally. When present in sites proximal to the
sacrum, they usually are found in the vertebral
body. The classic findings of GCT on technetium
bone scan include diffuse radionucleotide uptake
with areas of central photopenia and increased
peripheral uptake. Angiography illustrates that
most GCTs are hypervascular lesions. CT scans
demonstrate soft tissue attenuation with
well-defined margins and a thin rim of sclerotic
bone. MRI exhibits characteristic heterogeneous
signal intensity with low-to-intermediate
intensity on both T1- and T2-weighted images.
Most GCTs are benign; malignant
GCTs occur in only 5% of cases. Malignant GCTs
usually are related to previous irradiation in the
vicinity of the tumor. Although most GCTs are
benign, the lesions are locally aggressive, and
their size and location may not allow complete
resection. Those that cannot be excised en bloc
should be curetted. Radiation is reserved for
surgically inaccessible tumors. Selective arterial
embolization also can be used in the management of
these tumors. The recurrence rates can be as high
as 40-60%.
Chondrosarcoma
Chondrosarcoma is the second
most common nonlymphoproliferative tumor of the
spine. Chondrosarcomas comprise 7-12% of all spine
tumors, and the spine is the primary site in 3-12%
of all chondrosarcomas. Men are affected 2-4 times
more frequently than women. The mean age of
presentation is 45 years. The thoracic spine is
the most common site, but chondrosarcomas can
occur at all levels of the spine. The most common
symptoms are pain, a palpable mass, and neurologic
complaints in 45% of patients.
Plain radiographs of
chondrosarcomas typically demonstrate bone
destruction. The lesions may be apparent in the
vertebral body 15% of the time, in the posterior
elements 40% of the time, or in both 45% of the
time. In 70% of patients, the characteristic
chondroid matrix in the form of rings and arcs are
apparent on x-ray. Cortical destruction with soft
tissue extension is best observed on CT scan or
MRI. Chondrosarcomas that arise from malignant
transformation of osteochondromas are observed as
a thickening of the cartilaginous cap. Involvement
of the adjacent vertebral levels by extension
through the disc is observed in 35% of all
lesions. On CT scan or MRI, mineralization is
usually apparent in the soft tissue component of
the lesion. The radionucleotide uptake by the
lesion is intense and has a heterogeneous
appearance on bone scan.
Chondrosarcomas are relatively
low-grade lesions (grade I or II). Most lesions
are primary chondrosarcomas rather than secondary
chondrosarcomas that arise from the malignant
degeneration of osteochondromas as previously
noted. Chondrosarcomas have relatively sparse
cartilaginous stroma with a surrounding
pseudocapsule. Examination under higher
magnification reveals atypical nuclei with several
mitotic figures per high-powered field.
Surgical resection by vertebral
corpectomy and strut bone grafting sometimes may
be necessary for complete excision. Cure is
possible when complete resection can be achieved;
this is possible 25% of the time. If wide marginal
resection cannot be achieved, the tumor recurrence
results in death in 74% of cases. The mean
survival for all patients with chondrosarcomas is
5.9 years according to Shives et al. Adjunctive
treatment with radiation is controversial for
these tumors. Chemotherapy is used sometimes to
help decrease the size of the mass with high-grade
chondrosarcomas and dedifferentiated
chondrosarcomas. Metastases of chondrosarcoma
depend on the grade of the primary chondrosarcoma.
The lungs are the most frequent sites of
metastasis.
Ewing sarcoma
Ewing sarcoma is the most common
nonlymphoproliferative primary malignant tumor of
the spine in children. Lesions of the spine
comprise 3-10% of all primary sites of Ewing
sarcoma. Metastatic foci of Ewing sarcoma
involving the spine are more common than primary
lesions of the spine. Patients with Ewing sarcoma
usually present when aged 10-20 years. The most
common site of occurrence in the spine is the
sacrococcygeal region followed by the lumbar and
thoracic segments. Ewing sarcoma rarely occurs in
the cervical spine. Lesions are centered primarily
in the vertebral body but they can extend into the
posterior elements.
Plain x-rays reveal permeative
bone lysis, osseous expansion, or sclerosis.
Diffuse sclerosis is observed in 69% of spinal
lesions and is associated with osteonecrosis. CT
scans and MRIs demonstrate osseous involvement as
well as surrounding soft tissue involvement.
However, MRI is nonspecific.
Tissue from a Ewing sarcoma is
composed of sheets of small, round, blue cells
divided by septa, scant cytoplasm, and abundant
collagen. Areas of osteonecrosis are found in
spinal lesions. These correspond to the sclerotic
areas observed on plain x-rays as discussed above.
Genetically, patients with Ewing sarcoma are found
to have an 11;12 chromosomal translocation.
Before the advent of
chemotherapy, the survival rate for patients with
Ewing sarcoma was dismal due to the inability to
completely resect these lesions, especially in the
axial skeleton. Radiation and chemotherapy are the
current mainstays of treatment of Ewing sarcoma in
the spine, achieving almost 100% local control
with an 86% long-term survival rate in patients
with spinal Ewing nonsacral sarcomas. Sacral
tumors have a 62% local control rate and only 25%
long-term survival rate because of the tendency
for delayed clinical presentation and larger tumor
size. The most important prognostic indicator for
survival of Ewing sarcoma is the tumor’s response
to chemotherapy.
Osteosarcoma
Osteosarcomas of the spine are
rare, making up only 0.6-3.2% of all osteosarcomas
and only 5% of all primary malignant tumors of the
spine. They typically present in patients in the
fourth decade of life and have a male
predominance. Osteosarcomas are found at all
levels of the spine but are most common in the
lumbosacral segments. Eccentric involvement of the
vertebral body with extension into the posterior
elements is common. Patients often present with
pain and a palpable mass. Neurologic symptoms,
ranging from sensory deficits to paresis, are
found in 70-80% of patients. Serum alkaline
phosphatase may be elevated.
Plain x-rays of spinal
osteosarcomas reveal a densely mineralized matrix,
giving rise to the term ivory vertebrae. A loss of
vertebral height often occurs, with sparing of the
adjacent disc. Purely lytic lesions also have been
described. CT scans and MRIs are useful to
evaluate the extent of bony and soft tissue
involvement. If a large amount of mineralized
matrix is present, the lesion may appear with low
signal intensity on all MRI sequences.
Most osteosarcomas are blastic
lesions. They can be osteoblastic, chondroblastic,
or fibroblastic. Osteosarcomas can arise primarily
or secondarily from an exposure to radiation.
Secondary osteosarcomas can have a latency of up
to 20 years. Spinal osteosarcomas also have been
found in patients with Paget disease.
Surgical resection is the rule;
however, resection of spine lesions is often
incomplete due to the size and location of the
tumor at the time of presentation. Adjuvant
chemotherapy and radiation therapy often are
employed with varying degrees of utility. Spinal
osteosarcomas have a dismal prognosis, with deaths
usually occurring within the first year of
diagnosis. Only a few patients have been reported
to survive longer than 2 years.
Chordoma
Luschka first described chordoma
morphologically in 1856 in Virchow’s lab. The
discovery of the notochordal nature of the tumor
and the coining of the term chordoma is credited
to Ribbert in 1894.
Chordomas are uncommon tumors
comprising 2-4% of all primary malignant bone
tumors with a prevalence of 0.51 per million.
However, they are excluding lymphoproliferative
tumors and metastases, the most common primary
malignant tumor of the spine in the adult. As
Ribbert described, chordomas arise for the
notochord remnant. The notochord normally evolves
into the nucleus pulposus of the intervertebral
discs. Nonneoplastic notochord vestiges also are
found at the midline of the sphenooccipital
synchondrosis and in the sacrococcygeal regions.
The locations in which chordomas occur parallel
these vestigial distributions.
Regarding chordoma prevalence,
30-35% occur in the sphenooccipital region, 50% in
the sacrococcygeal region (especially S4-S5), and
15% occur in the other spinal segments.
Interestingly, chordomas have
not been reported to arise from the intervertebral
discs. Chordomas occur most commonly in patients
aged 30-70 years, with a peak incidence in the
fifth to sixth decades of life. Sphenooccipital
lesions have equal sex distributions but
sacrococcygeal lesions have a 3:1 male-to-female
ratio.
Presentation of chordomas is
often subtle, with a gradual onset of pain,
numbness, motor weakness, and constipation or
incontinence. Constipation is a uniform finding in
most patients with sacrococcygeal lesions.
Chordomas are typically slow growing lesions and
are often very large at the time of presentation.
On plain x-ray, chordomas appear
as a destructive lesion of a vertebral body in the
midline, with a large associated soft tissue mass.
In sacrococcygeal lesions, osseous expansion is
frequent and may extend across the sacroiliac
joints. Mineralization within the tumor may be
observed on the plain x-rays of 50-70% of
sacrococcygeal lesions. The mineralization is
amorphous and predominates in the periphery of the
lesion. Lesions in spinal segments above the
sacrum are less expansile and demonstrate evidence
of calcification in only 30% of cases. They may
have areas of sclerosis in 43-62% of cases. The
intervertebral discs above or below a chordoma may
be involved and narrowed in a manner that
simulates infection. The lesion can make its way
through the intervertebral disc to infiltrate an
adjacent level. This occurs in approximately
11-14% of cases.
CT scan demonstrates both the
osseous and soft tissue components of the tumor.
Coronal and sagittal reconstructions of the CT
scan are helpful in assessing neural foraminal and
sacroiliac joint involvement. MRI scans are an
important adjunct in the workup of chordomas. The
lesions appear with low-to-intermediate signal
intensity on T1 images with very high signal
intensity on T2 images, reflecting the high water
content of chordomas. Enhancement occurs following
intravenous contrast on both CT scan and MRI.
Chordomas are lobulated
neoplasms, which usually are contained within a
pseudocapsule. Histology of these lesions reveals
long cords of physaliphorous cells. Physaliphorous
cells are clear cells containing intracytoplasmic
vacuoles with abundant intracellular and
extracellular mucin. Sarcomatous chondroid,
osteoid, or fibroid elements may be found within
the chordoma.
Surgical resection is the rule.
Adjuvant postoperative radiation therapy, proton
beam therapy, and brachytherapy all have been used
with varying results. The prognosis depends on
whether the tumor can be resected completely. The
location of the lesion and the size at
presentation often necessitate incomplete
resection. Persons with sacrococcygeal tumors
often have improved survival because the
surrounding structures are relatively more
expendable and allow a more complete resection.
Persons with sacrococcygeal lesions typically have
8-10 years survival as opposed to 4-5 years
survival for persons with chordomas in other
spinal sites. Death usually is related to local
recurrence and invasion rather than metastatic
disease. Chordomas can metastasize. The most
common sites of metastases are the liver, lungs,
regional lymph nodes, peritoneum, skin, and heart.
Multiple myeloma
Multiple myeloma is a systemic
disease that affects middle-aged people and is
characterized by areas of local bone destruction.
Multiple myeloma is the most common primary
malignancy of bone and the spine. The underlying
cell line is the malignant plasma cell, which
produces abnormal quantities of immunoglobulins.
The presentation of patients
with myeloma is similar to that of other spine
tumor patients. Patients complain of pain that may
be worse at night. The laboratory workup for these
patients should include a complete blood count
with differential looking for anemia and
thrombocytopenia, an elevation of the erythrocyte
sedimentation rate, and a decrease in the serum
albumin with increased total serum protein. The
abnormal production of immunoglobulins can be
detected on serum or urine electrophoresis and can
be used to confirm the diagnosis. Radiographically,
skeletal survey is used to screen for lesions that
can occur throughout the skeleton. Bone scans have
a high false-negative rate and are not optimal
studies for the evaluation of myeloma. Once a
lesion is detected in the spine, CT scan, MRI, or
both should be performed to assess the destruction
of the vertebrae and the effect of this
destruction on the surrounding neurologic and
paraspinous tissues.
Multiple myelomas are generally
sensitive to radiation and chemotherapies. Surgery
for stabilization is indicated in myelomas of the
spine when destruction of the vertebral body
exists to such an extent that collapse and
possible kyphosis with canal compromise could
result. Prophylactic posterior stabilization can
be carried out with segmental instrumentation in
cases prior to fracture. Anterior strut grafting
or cage reconstruction may be necessary once
fracture and collapse have occurred. Adjuvant
radiation therapy may be used postoperatively once
healing of the surgical site has been obtained.
Solitary plasmacytoma
Akin to multiple myeloma as a
descendent of plasma cell malignancies, the
plasmacytoma is a solitary lesion that usually
affects the vertebral body. Plasmacytomas
generally affect younger patients than multiple
myeloma and are associated with a better
prognosis. Plasmacytomas eventually can evolve
into multiple myeloma; thus, patients should be
monitored for more than 20 years following the
original diagnosis of plasmacytoma. The diagnosis
is made by biopsy of the lesion, and treatment
includes radiation and bracing except in persons
with pathologic or impending pathologic fractures.
In these individuals, surgical resection and
stabilization should be carried out with
postoperative adjuvant radiation therapy once 6-8
weeks of postsurgical healing has occurred.
Patients have greater than 60% 5-year survival
rates.