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
                                                                                                                                                    7. Murali R, Rovit RH, Benjamin       M%/. Chordoma     of the cervical spine. Neu-
                                                      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
                                                      a sclerotic and/or osteolytic lesion in a vertebral body with a                                  in a patient     with paraparesis   and ivory vertebral      body. Neurosurgery
                                                      large,     paraspinal,       soft-tissue      mass      in an older patient        with          1985:16(1):    100-102