861

Spinal

Chordoma:

Radiologic Features

in 14 Cases

.1
Francisc,a T. de BruIne1 Herman M. Kroon
The radiologic appearance of chordoma of the cervical (three patients), thoracic (four

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 conventional radiographs; three cases also had CT examinations. In thirteen patients, the tumor 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 softtissue 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
Fifty-nine tumorlike and consists

and Methods
histologically lesions of bone confirmed

of the Netherlands
on Bone Tumors

Committee
recorded

cases of chordoma were selected for study from the files on Bone Tumors, a collection of about 8000 tumors and
over a period of 34 surgeons. years. The Netherlands Committee

is the Dutch

advisory
pathologists,

board
and

on the diagnosis

and treatment

of bone tumors

of radiologists,

(54%) were located in the sacrococcygeal (24%) were found in the cervical, dorsal,
concerning the exact location of the

region,
or lumbar were

Of the 59 chordomas studied, 32 10 (1 7%) were sphenooccipital, and 14 spine. In three patients (5%), the data insufficient.

chordomas

Received October 26, 1987; accepted after revision December 4, 1987. 1 Both authors: Department of Diagnostic Radiology, University Hospital Leiden, Bldg. 1 , C2-S, Rijnsburgerweg 10, 2333 AA Leiden, the Netherlands. Address reprint requests to H. M. Kroon. AJR 150:861-863, AprIl 1988 0361 -803X/88/1 504-0861 C American Roentgen Ray Society

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 (mean, 57 years). The mean duration of complaints was 16 months (range, 3-48 months).
The most common symptom was back pain, which in three patients irradiated to the legs.

Three patients developed a neurologic deficit. Dysphagia was a feature in the patients with cervical chordoma. Plain radiographs were available in all 14 patients. In three patients, additional CT studies were available. In 1 1 patients, a surgical decompression of the spinal cord was performed. Five patients received additional radiotherapy, while three patients were treated by radiotherapy only. In seven cases (50%), the spinal tumor recurred after an average
of 2 years. Only one patient developed a metastasis to the base of the skull.

C
and osteolysis.

Fig. 1.-Serial radiographs in patient with chordoma of second lumber vertebra show both osteosclerosis 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.

-1

#{149}

:-.._

Fig. 2.-Chordoma involving second lumbar vertebra. A, Lateral tomogram shows osteosclerotic 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,
,

Of the 14 tumors, three were located in the cervical spine, four in the dorsal spine, and seven in the lumbar spine. In 12

patients,

the tumor was limited to one vertebra.

Involvement

of the intervertebral disk was found in three cases: twice in the cervical spine and once in the lumbar region. Of these three chordomas, extension to adjacent vertebral bodies was present in two. Thirteen originated in the vertebral body, and one arose in the posterior elements. In five of the 1 4 cases, the tumor was purely osteolytic on radiographs. In nine patients, osteosclerosis was a prominent feature. Eight of these nine had both osteolytic and osteosclerotic areas throughout the tumor, whereas one tumor was predominantly osteosclerotic (Figs. 1 -4). For comparison, sclerosis was found in only two of 32 sacrococcygeal and in only three of 1 0 sphenooccipital chordomas. An accompanying soft-tissue mass was visible on conventional radiographs and/or on CT in nine patients.

only about 1 5% are located in the vertebral column [1 2]. Theremaining 85% involvethe sacrococcygealareaor sphenooccipital area [1 2]. In our series of 59 patients with chordoma, however, 24% (1 4 cases) were located in other parts of the spine. Seven were in the lumbar spine, four were in the thoracic spine, and three were in the cervical spine. This
,

distribution

is in agreement

with

the

distribution

reported

in

the literature [3]. Previous reports indicate that dominantly destructive, involving

spinal chordomas are preone or more vertebrae and

often associated
occasionally

with a paraspinal
[1
,

soft-tissue

mass, which is

Discussion

Chordoma is a rare malignant nantly at either end of the neural

tumor occurring predomiaxis. It accounts for only 1

2, 4, 5]. The intervertebral disks are usually spared [3]. In our series, 1 2 (86%) of the 1 4 tumors were limited to one vertebra; only two extended to adjacent vertebrae. Most lesions (90%) originated in the body of the vertebra; only one was located in the posterior elements of the vertebra. A paraspinal soft-tissue mass was a prominent 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 osteomyelitis difficult. Bone destruction was the main radiographic feature noted in eight cases of chordoma of the spine reported by Utne and
calcified

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.

Fig. 4.-Chordoma of the 12th thoracic vettebra. Frontal (A) and lateral (B) radiographs show marked osteosclerosis with involvement of right pediCle.

Pugh [4]. Osteosclerosis attributed to compression of bone or osteoarthritis was described in four of their cases. Pinto et al. [2] mentioned sclerosis in five of eight cases. Firooznia et al. [3J observed a destructive lesion with a sclerotic rim in seven of 1 6 patients (the series, however, consisted of both spinal and sacral chordomas). The authors of several case

long-standing chordoma.

back

pain

should

raise

the

possibility

of

ACKNOWLEDGMENTS The authors

graphic work

gratefully
and Ineke

acknowledge
Lek for her

Gemt

Kracht for his photoassistance.

secretarial

reports mention osteosclerosis as a feature of chordoma [58]. Nine (64%) of our 14 patients had osteosclerosis. Eight
patients showed a mixed osteolytic-osteosclerotic appear-

REFERENCES
1 . Firooznia Computed H, Golimbu C, Rafli M, Reede DL, Kricheft tomography of spinal chordomas. J II, Bjorkengren A. Comput Tomogr

ance (Figs. 1-3), and one patient had sclerosis as a main feature (Fig. 4). In contrast to earlier reports, the sclerosis in
our patients was not confined to the periphery of the tumor

1986;10(1):45-50
2. Pinto RS, Un JP, Firooznia H, Lefleur RS. The osseous and angiographic features of vertebral chordomas. Neuroradiology 1975;9:231-241 3. Firooznia H, Pinto RS, Un JP, Baruch HH, Zausner J. Chordoma: radiologic evaluation of 20 cases. AiR 1976;127:797-805 4. Utne JR, Pugh DG. The roentgenologic aspects of chordoma. Am J Roentgenol Radium Thor NucI Med 1955:74 :593-608 5. Meyer JE. Lepke RA, Undfors KK. et ai. Chordomas: their CT appearance in the cervical thoracic and lumbar spine. Radiology 1984: 1 53(3): 693-696 6. Abdelwahab IF, OLeary PF, Steiner GC, Zwass A. Case report 357: chordoma of the fourth lumbar vertebra metastasizing to the thoracic spine and ribs. Skeletal Radiol 1986;1 5(3): 242-246 7. Murali R, Rovit RH, Benjamin M%/. Chordoma of the cervical spine. Neurosurgery 1981;9(3):253-256 8. Schwarz SS, Fisher WS Ill, Pulliam MW, Wanstein ZR. Thoracic chordoma in a patient with paraparesis and ivory vertebral body. Neurosurgery 1985:16(1): 100-102

and could not be attributed

to compression

or osteoarthritis

only. Osteosclerosis occurred much more frequently in the 1 4 patients with spinal chordoma than in the 42 patients with
sacrococcygeal and sphenooccipital chordomas in our series.

On the basis of these findings,

we believe that chordoma

should be included in the differential diagnosis of sclerotic or partially sclerotic solitary vertebral lesions, in addition to much more common causes such as metastasis, lymphoma, Paget disease, and chronic spinal osteomyelitis. The combination of a sclerotic and/or osteolytic lesion in a vertebral body with a

large,

paraspinal,

soft-tissue

mass

in an older patient

with