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861

Spinal Chordoma: Radiologic Features


in 14 Cases

.1
Francisc,a T. de BruIne1 The radiologic appearance of chordoma of the cervical (three patients), thoracic (four
Herman M. Kroon patients), and lumbar spine (seven patients) was studied. Eleven patients were over 50
years old and presented with long-standing back pain. All were examined with conven-
tional radiographs; three cases also had CT examinations. In thirteen patients, the tumor
American Journal of Roentgenology 1988.150:861-863.

originated in the vertebral body and, in one patient, in the posterior element of a vertebra.
In nine (64%) of the 14 cases, osteosclerosis was a prominent feature. In the remaining
five cases (36%), the bone lesion was purely osteolytic Involvement of the intervertebral
disk was found in three patients; in two of these the tumor extended to an adjacent
vertebra. In nine patients, a soft-tissue mass was a distinctive additional feature.
A sclerotic and/or osteolytic lesion in a vertebral body with a large, paraspinal soft-
tissue mass in an older patient with long-standing back pain should raise the possibility
of a chordoma.

Chordoma is a rare malignant tumor of the axial skeleton arising from remnants
of the primitive notochord. The tumor tends to occur at either end of the axial
skeleton, the most common sites being the sacrococcygeal and sphenooccipital
regions, where 85% of the lesions are found [1 2]. Involvement of the cervical, ,

thoracic, and lumbar spine is uncommon, occurring in only 15% of cases [1 2]. ,

The tumor grows slowly and is locally invasive. Recurrence after surgical resection
is likely. Distant metastases are uncommon.
We studied retrospectively 1 4 patients in whom the tumor was located in the
cervical, dorsal, or lumbar spine. The radiologic findings in these patients are the
subject of this report.

Subjects and Methods


Fifty-nine histologically confirmed cases of chordoma were selected for study from the files
of the Netherlands’ Committee on Bone Tumors, a collection of about 8000 tumors and
tumorlike lesions of bone recorded over a period of 34 years. The Netherlands’ Committee
on Bone Tumors is the Dutch advisory board on the diagnosis and treatment of bone tumors
and consists of radiologists, pathologists, and surgeons. Of the 59 chordomas studied, 32
(54%) were located in the sacrococcygeal region, 10 (1 7%) were sphenooccipital, and 14
(24%) were found in the cervical, dorsal, or lumbar spine. In three patients (5%), the data
concerning the exact location of the chordomas were insufficient.
The medical records and radiographic studies of the 14 patients with spinal chordoma
were studied. There were nine men and five women, ranging in age from 16 to 80 years
Received October 26, 1987; accepted after re- (mean, 57 years). The mean duration of complaints was 16 months (range, 3-48 months).
vision December 4, 1987.
The most common symptom was back pain, which in three patients irradiated to the legs.
1 Both authors: Department of Diagnostic Ra-
Three patients developed a neurologic deficit. Dysphagia was a feature in the patients with
diology, University Hospital Leiden, Bldg. 1 , C2-S,
cervical chordoma. Plain radiographs were available in all 14 patients. In three patients,
Rijnsburgerweg 10, 2333 AA Leiden, the Nether-
lands. Address reprint requests to H. M. Kroon. additional CT studies were available. In 1 1 patients, a surgical decompression of the spinal

AJR 150:861-863, AprIl 1988


cord was performed. Five patients received additional radiotherapy, while three patients were

0361 -803X/88/1 504-0861


treated by radiotherapy only. In seven cases (50%), the spinal tumor recurred after an average
C American Roentgen Ray Society of 2 years. Only one patient developed a metastasis to the base of the skull.
A B C
Fig. 1.-Serial radiographs in patient with chordoma of second lumber vertebra show both osteosclerosis and osteolysis.
A, Initial radiograph.
B, Radiograph made 3 months later shows progression of tumor and compression of vertebral body.
C, Radiograph made 3 years later shows further destruction and collapse.
American Journal of Roentgenology 1988.150:861-863.

‘. ‘-1 . #{149} P
Fig. 2.-Chordoma involving second lumbar
vertebra.
A, Lateral tomogram shows osteosclerotic
. . . p
and osteolytic changes.
B, CT of second lumber vertebra shows a
:-.._ huge soft-tissue mass extending from vertebral
body.

Results 2% of primary malignant bone tumors. Of all reported cases,


only about 1 5% are located in the vertebral column [1 2]. ,
Of the 14 tumors, three were located in the cervical spine,
Theremaining 85% involvethe sacrococcygealareaor spheno-
four in the dorsal spine, and seven in the lumbar spine. In 12
occipital area [1 2]. In our series of 59 patients with chor-
,
patients, the tumor was limited to one vertebra. Involvement
doma, however, 24% (1 4 cases) were located in other parts
of the intervertebral disk was found in three cases: twice in
of the spine. Seven were in the lumbar spine, four were in the
the cervical spine and once in the lumbar region. Of these
thoracic spine, and three were in the cervical spine. This
three chordomas, extension to adjacent vertebral bodies was
distribution is in agreement with the distribution reported in
present in two. Thirteen originated in the vertebral body, and
the literature [3].
one arose in the posterior elements. In five of the 1 4 cases,
Previous reports indicate that spinal chordomas are pre-
the tumor was purely osteolytic on radiographs. In nine pa-
dominantly destructive, involving one or more vertebrae and
tients, osteosclerosis was a prominent feature. Eight of these
often associated with a paraspinal soft-tissue mass, which is
nine had both osteolytic and osteosclerotic areas throughout
occasionally 2, 4, 5]. The intervertebral
calcified [1 , disks are
the tumor, whereas one tumor was predominantly osteoscle-
usually spared [3]. In our series, 1 2 (86%) of the 1 4 tumors
rotic (Figs. 1 -4). For comparison, sclerosis was found in only
were limited to one vertebra; only two extended to adjacent
two of 32 sacrococcygeal and in only three of 1 0 sphenooc-
vertebrae. Most lesions (90%) originated in the body of the
cipital chordomas. An accompanying soft-tissue mass was
vertebra; only one was located in the posterior elements of the
visible on conventional radiographs and/or on CT in nine
vertebra. A paraspinal soft-tissue mass was a prominent
patients.
feature in 64% of the patients. Intervertebral disk involvement
was found in two of the three cervical chordomas and in one
of the seven lumbar tumors, making differentiation from spinal
Discussion
osteomyelitis difficult.
Chordoma is a rare malignant tumor occurring predomi- Bone destruction was the main radiographic feature noted
nantly at either end of the neural axis. It accounts for only 1 - in eight cases of chordoma of the spine reported by Utne and
AJR:150, April 1988 SPINAL CHORDOMA 863

Fig. 3.-Chordoma involving fourth lumbar


vertebra. Frontal (A) and lateral (B) radiographs
show combined osteolysis and osteosclerosis.
American Journal of Roentgenology 1988.150:861-863.

Fig. 4.-Chordoma of the 12th thoracic vet-


tebra. Frontal (A) and lateral (B) radiographs
show marked osteosclerosis with involvement of
right pediCle.
A B

Pugh [4]. Osteosclerosis attributed to compression of bone long-standing back pain should raise the possibility of a
or osteoarthritis was described in four of their cases. Pinto et chordoma.
al. [2] mentioned sclerosis in five of eight cases. Firooznia et ACKNOWLEDGMENTS
al. [3J observed a destructive lesion with a sclerotic rim in
The authors gratefully acknowledge Gemt Kracht for his photo-
seven of 1 6 patients (the series, however, consisted of both
graphic work and Ineke Lek for her secretarial assistance.
spinal and sacral chordomas). The authors of several case
reports mention osteosclerosis as a feature of chordoma [5- REFERENCES
8]. Nine (64%) of our 14 patients had osteosclerosis. Eight 1 . Firooznia H, Golimbu C, Rafli M, Reede DL, Kricheft II, Bjorkengren A.
patients showed a mixed osteolytic-osteosclerotic appear- Computed tomography of spinal chordomas. J Comput Tomogr
ance (Figs. 1-3), and one patient had sclerosis as a main 1986;10(1):45-50
2. Pinto RS, Un JP, Firooznia H, Lefleur RS. The osseous and angiographic
feature (Fig. 4). In contrast to earlier reports, the sclerosis in
features of vertebral chordomas. Neuroradiology 1975;9:231-241
our patients was not confined to the periphery of the tumor 3. Firooznia H, Pinto RS, Un JP, Baruch HH, Zausner J. Chordoma: radiologic
and could not be attributed to compression or osteoarthritis evaluation of 20 cases. AiR 1976;127:797-805
only. Osteosclerosis occurred much more frequently in the 4. Utne JR, Pugh DG. The roentgenologic aspects of chordoma. Am J
1 4 patients with spinal chordoma than in the 42 patients with Roentgenol Radium Thor NucI Med 1955:74 :593-608
5. Meyer JE. Lepke RA, Undfors KK. et ai. Chordomas: their CT appearance
sacrococcygeal and sphenooccipital chordomas in our series.
in the cervical thoracic and lumbar spine. Radiology 1984: 1 53(3): 693-696
On the basis of these findings, we believe that chordoma 6. Abdelwahab IF, O’Leary PF, Steiner GC, Zwass A. Case report 357:
should be included in the differential diagnosis of sclerotic or chordoma of the fourth lumbar vertebra metastasizing to the thoracic spine
partially sclerotic solitary vertebral lesions, in addition to much and ribs. Skeletal Radiol 1986;1 5(3): 242-246
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more common causes such as metastasis, lymphoma, Paget
rosurgery 1981;9(3):253-256
disease, and chronic spinal osteomyelitis. The combination of 8. Schwarz SS, Fisher WS Ill, Pulliam MW, Wanstein ZR. Thoracic chordoma
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