Chordoma: Radiologic Evaluation of 20 Cases

HOSSEIN FIROOZNIA,’ RICHARD S. PINTO,’ JOSEPH P. LIN,’ HANS H. BARUCH,2 AND JOSEPH ZAUSNER’

Experience with 20 cases of chordoma is summarized. order of frequency) include the cervical, lumbar, and
Three of four intracranial tumors presented as sellar thoracic spine [6, 10].
tumors. The clivus remained intact for 2, 3’,4, and 4 years, For intracranial chordoma, the predominant symptoms
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respectively, in three patients despite extensive destruc- at presentation are headache, diplopia, blurred vision,
tive lesions of the sellar region. Our data and those of
ptosis, visual field defect, dysphagia, paralysis of various
others indicate that approximately half the patients with
cranial nerves, and long tract signs [6]. Approximately
chordomas have erosion of the clivus at the time of initial
one-third of these patients have a retropharyngeal mass
medical evaluation. The other half mainly have sellar de-
structive lesions with or without associated clival erosion. [6, 11-14]. Occasionally, this is the only finding acciden-
Calcification occurs in 50%-70% of intracranial chordo- tally discovered. Rarely, the tumor extends intranasally
mas. A nasopharyngeal mass is present in one-third. and produces nasopharyngeal symptoms as its initial clini-
Vertebral chordomas often involve two or more vertebrae, cal manifestation [6. 1 5, 1 6].
producing destructive lesions which often have a sclerotic The clinical findings associated with vertebral and sacro-
rim. The intervertebral discs are commonly affected. A coccygeal chordoma are due to involvement of bone and
paraspinal soft tissue mass, half the time containing calci- soft tissues, compression of the cord and spinal nerves,
fication, is often present. Sacral lesions are characterized
and pressure on extraspinal structures such as the trachea
by a midline destructive lesion, occasionally associated
and esophagus or rectum and bladder [5, 1 7]. On rectal
with expansion, with or without osteosclerosis. A firm,
fixed, extrarectal, presacral mass which may contain cal-
examination, a firm mass fixed to the sacrum and clearly
cification or fragments of bone is almost always present.
extraluminal is almost always detected. In one-third of the
patients, a retrosacral mass is also present [2, 3, 7, 10].
Chordoma is a rare malignancy of notochordal origin,
most probably arising from ectopic notochordal remnants
Pathology
[1, 2]. It is a locally invasive tumor, growing slowly and
invading the adjacent bone and soft tissue structures. It Chordomas occur with predictable regularity along the
does not metastasize readily. This communication reviews path of the primitive notochord. The tumor is a grossly
our experience with 20 cases of histologically verified soft, lobulated mass which may have areas of hemorrhage,
chordoma seen from 1 964 to 1 973 at the New York cystic change, or calcification. Intracranial chordomas
University-Bellevue Medical Center. A review of the litera- rarely become very large because of the involvement of
ture is also presented. adjacent vital structures [2]. They typically arise in the
midline from the clivus and the posterior part of the sella
Clinical Findings and invade the underlying bone with ease. Vertebral and
sacrococcygeal lesions almost always arise from the
The study group consisted of 1 1 males and nine females; ectopic notochordal remnants within the bodies of the
ages ranged from 25 to 78 years. Chordomas were intra- vertebrae and often cause destruction of two or more of
cranial in four cases, vertebral in eight, and sacrococcygeal them [2]. The intervertebral discs may also be invaded.
in eight (table 1 ). Mean age at onset was 45, 54. and 60 The tumor often extends into the epidural space in the
years. respectively. The tumor grows slowly; thus there is spinal canal. It then may grow for a considerable distance
usually a delay of many months to a few years between [1, 2, 13, 18-20].
onset of symptoms and definitive diagnosis. In ur series, In our eight cases of chordomas, only one patient had
the average interval was 1 8 months.
Due to the rarity of destruction of a solitary vertebra; the rest had two or
this tumor, very few large series have been reported [3-5 1. more affected. Invasion of the discs and dura as well as
In 548 cases compiled from the world literature in 1960, extension of the tumor into the subarachnoid space was
240 (44%) were intracranial, 227 (42%) were sacro- noted at surgery in two patients. There was extensive
coccygeal, and 81 (1 4%) were vertebral in origin [6]. seeding of the spinal cord in one. This is a very rare occur-
Sacrococcygeal chordomas occur mainly between the rence, and only few well documented cases have been
fifth and seventh decades of life [2, 7, 8], being very rare reported [5, 8, 21-23].
prior to the third decade. They occur twice as frequently The overall incidence of metastasis is about 1 0%. There
in males as females. Intracranial chordomas occur in all are only 50 reported instances in the literature, 32 sacro-
age groups without a definite predilection for either sex coccygeal, 1 4 vertebral, three cranial, and one primary
and with a mean age of onset of 35 years [6, 9]. No scapular [4, 21 ]. Metastasis is usually by the hemato-
pattern age or sex exists for chordomas in other locations. genous route [7], although in most patients it is not
In the spine, the areas of involvement (in descending widely disseminated. The most frequent sites are the lungs,

Received January 6, 1976; accepted after revision June 14, 1976.

‘ DepartmentofRadiology, NewYork University Medical Center, 560 First Avenue. New York, New York 10016. Address reprint requests to J. Zausner.
2 Departments of Pathology and Radiology. City Hospital, Elmhurst Queens, New York 1 1373.

Am J Roentgenol 127:797-805, 1976 797

 798 FIROOZNIA El AL.

TABLE 1

Clinical Findings in Patients with Chordoma

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Duration of
Age at Onset Time from Onset Clinical Findings
Disease Treatment Comment
Case No. and 5ex Location to Diagnosis Symptoms and Course (Yr)
(Yr)

1 (fig. 1) . . . . 51 , M Sella turcica 1 Headache. Left 3. 4. 6 43’ Radiotherapy. Alive with

diplopia, nerve palsy; surgery disease
numbness midline naso-
of leftface pharyngeal
mass 8
months later
2 (fig. 2) . . . . 60, M Sella turcica 2 Sudden par- Bitemporal 3V Radiotherapy,
tial loss of field defect, surgery
vision of treated for
left eye pituitary
tumor,
worsening
condition
lead to
biopsy
3 57, F SeIla turcica 2Y Sudden di- Right 6 and 5 Radiotherapy, Diabetes

plopia lead- left 3, 4, 6 surgery insipidus

ing to nerve post-
paresthesia paresis inter- operatively
and weak- mittent for
nessof 2 years
right arm
4 48, F Clivus Y Vertigo. Right 3. 6 and 2 Surgery Died post-
nausea, left 6 nerve operatively
headache. palsy; ataxic with disease
diplopia gait. bilateral
hyperreflexia
5 33, F C2-C4 4 Left arm and Left hyper- 40 Surgery Surgery three
leg weak- reflexia, times for
ness, left decreasing recurrence
shoulder vibratory
pain sense in
left leg
6 24, F C2-C4 1/4 Neck pain, Atrophy left 6 Surgery
left arm and
shoulder; hand,
left upper hyporeflexia
extremity
weakness
progressing
to paralysis
7 32, M C2-C3 2 Neck pain for Nasopharyn- 7 Radiotherapy, Recurrence,
2 years, geal mass at surgery extensive
dysphagia onset, destruction
2 weeks treated with skull base
radiothera-
phy; de-
truction
middle and
posterior
fossa bi-
laterally 2
years later
 CHORDOMA 799

TABLE 1 -(continued)

Age at Onset Time from Onset Clinical Findings Duration of

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Case No and Sex Location to Diagnosis Symptoms and Course Disease Treatment Comment

8 37, F C5-C7 4 Neck pain Decreasing 3 Surgery. Recurrence at

progressing lower right radiotherapy 3 years
toleft hand,de-
shoulder creasing
and elbow, right biceps
right hand reflex, sen-
weakness sory defect
and numb- both hands
ness
9 61 . F C4-C5 ‘4 Neck pain Right hemi- 5 Surgery No recurrence
progressing paresis. left in 6 years
to left hypoalgesia
shoulderand and hyper-
elbow, left reflexia,
lower bilateral
extremity Babinski
weakness
leading to
inability
to walk
10 (fig. 3) . . . . 47, M T9-T1 1 ? Severe hip Paraplegia, 11 Surgery. Died with
and leg pain, urinary and radiotherapy metastases
bilateral fecal incon- to brain,
laminec- tinence lungs, and
tomy; sud- epidural
den para- seeding of
plegia 9 cord
years later
11 58, F L2-L3 2 Back pain for Mass at L2- 5 Surgery Paraplegic
2 years, L3, para- following
weakness of paresis recurrence
legs 4 months

12 68, M L2 Back pain Mass and 1 Surgery No recurrence

progressing tenderness in 1 year
to legs for L2-L3,
6 months, bilateral
weakness of lower
legs 2 months extremity
hyporeflexia
13 56, F Sacrum 2 Back pain for Presacral 3 Surgery Died with
2 years, pelvic mass metastasis
numbness of on rectal to brain
buttocks for exam
6 months,
urinary and
fecal incon-
tinence for 3
months
14 39, M Coccyx 4 Recurrent 15 Surgery, Surgery nine
coccygeal radiotherapy times for
pain for 4 recurrence;
years, swell- died post-
ing 6 months operatively
 800 FIROOZNIA El AL.

TABLE 1 -(continued)

Age at Onset Time from Onset Clinical Findings Duration of

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Case No and Sex Location to Diagnosis Symptoms and Course Disease Treatment Comment

15 45. M Coccyx 2 Coccygeal Presacral hard 3 Surgery No recurrence

pain 2 years mass mini- in 1 year
mally tender
1 6 (fig. 4) . . . . 68. F Sacrum 1 Y2 Back pain Presacral mass 3Y2 Surgery Died post-
progressing operatively
to legs for
1 8 months,
urinary in-
continence.
severe
constipation
17 78, M Sacrum ? Acute urinary Bladder stones, 2 Surgery, Recurrence
retention sacral lesion radiotherapy at 9 months
on x-ray

18 58. M Sacrum. ‘/ Mass on Small mass 2V Surgery No recurrence

coccyx sacrum and mild in 2V years
2 months tenderness
in sacro-
coccygeal
region

19 65. M Sacrum. 1 Backpain 8 X 8cmfirm 1 Surgery . .

coccyx progressing presacral

to legs mass
20 67. M Sacrum. S Intermittent Presacral firm 6 Surgery . .

coccyx back pain for large mass

5 years. leg
pain 2 months

liver, lymph nodes, bones, brain, skin, and peritoneum duct of Sylvius, fourth ventricle, and cisterna pontis were
[3-6, 21 ]. normal (figs. 1E and iF).
Radiologic Findings Carotid and/or vertebral angiography was performed
in all four patients. The first patient had an avascular mass
Intracranial Chordoma invading the clivus and displacing the vertebral and basilar
In our four patients there was extensive destruction of arteries posteriorly. In the remaining three patients findings
both the sella and clivus as well as retrosellar calcification indicative of a sellar mass with extension in various direc-
in one patient. The other three patients had varying de- tions was noted. The intracavernous segment of the in-
grees of erosion of the anterior and posterior clinoids, ternal carotid artery was displaced laterally in one. There
sellar floor, and dorsum sellae. There was no associated was stretching, lateral displacement, and irregularity (en-
destructive lesion of the clivus (figs. lA-i D). Pneumo- casement by tumor) of the supraclinoid segment of the
encephalography in one patient revealed a sellar mass internal carotid artery in two. The horizontal segment of
encroaching on the recesses and anterior floor of the the anterior cerebral artery was displaced superiorly in
third ventricle. The lateral ventricles were moderately three. Encasement of this segment was noted in one
enlarged. The foramen of Monroe was narrowed. The aque- patient (fig. 2).

Fig. 1 . -Chordoma arising from posterior

sella with eventual suprasellar and parasellar extension (case 1 ). A. Deossification of dorsum sellae 1 year
after clinical onset. Findings suggest increased
intracranial pressure. B. Tomogram 2 years after onset showing erosion of dorsum sellae, focal erosion
of upper clivus. and small mass in nasopharynx. C. Erosion of anterior and posterior clinoids, dorsum sellae. and upper clivus 3 years after onset.
D, Tomogram 3h years after onset showing extensive erosion of clinoids. sellar floor. dorsum sellae, upper clivus. E. Pneumoencephalogram 3 years
after onset showing anterior suprasellar extension of mass with obliteration of anterior recesses of third ventricle (arrow) and compression of frontal
horns of lateral ventricles (double arrows). F. AP projection, brow-up position of pneumoencephalogram (fig. 1 E). showing asymmetric elevation and
distortion of anterior third ventricle due to eccentric suprasellar mass.
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-
 802 FIROOZNIA El AL.

F,
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, .4 -
#.‘

I .‘

1_. (

__._._,_.‘,_J .- , -

Fig. 2.-Sellar chordoma (case 2). Selective left internal carotid angiogram 2 years after first admission and 1 year after subtotal resection of sellar
chordoma showing recurrence of lesion with prominent suprasellar and parasellar extension. Supraclinoid segment of internal carotid and horizontal
segment of middle cerebral artery are displaced upward and narrowed due to encasement by tumor Note hypertrophy of multiple small arteries
supplying intrasellar and suprasellar portions of tumor.

Comment. Chordoma was the primary radiologic diag- follow-up. In 31 patitnts reported by Schechter et al.
nosis in the first patient. In the remaining three, a pituitary [1 2]. clival erosion was recorded in 1 3 instances.
adenoma was the initial diagnosis based on the presence Differential diagnosis. The differential diagnosis should
of a sellar mass with extension to the surrounding tissues, include pituitary tumors, craniopharyngioma. epidermoid,
sellar erosion, and an intact clivus. Irradiation resulted in meningioma, aneurysm, osteochondroma, optic glioma,
partial improvement in two patients. However, symptoms nasopharyngeal malignancies. and metastasis. In patients
recurred with increasing severity, necessitating surgical with a destructive mass of the sellar region but no con-
intervention which established the histologic diagnosis of comitant erosion of the clivus, differentiation from pitui-
chordoma. Follow-up studies at 2, 32. and 4 years re- tary masses may be impossible. However, even though
vealed progressively increasing destruction of the sella. destructive changes of the sella are common, sellar
The clivus remained intact in two patients. In one patient ballooning and expansion, as seen in pituitary adenoma,
clival erosion was first noted at 2 year follow-up. are extremely rare. A recent report [27] describes a
In most patients the radiographic findings consist of chordoma confined entirely within an intact sella, said to
various degrees of erosion of the clivus, sella turcica, or be the only such case ever reported.
both, associated with a soft tissue mass [6. 1 9, 20, 23- Angiography usually establishes the diagnosis in aneu-
26]. Calcification occurs in 3O%-7O% of cases [4, 6. 7, rysms and in most meningiomas. It is helpful in clival
20, 24, 26]. It was present in two of our patients. Chor- chordoma by revealing posterior displacement of the
doma typically causes osseous erosion centrally along the basilar and/or vertebral arteries, clearly establishing their
midline of the base of the skull. However, the erosion can extraaxial origin [19, 26]. Pneumography also provides
occur predominantly on one side [6, 1 9, 20, 23]. Occa- an accurate indication of the extraaxial location of these
sionally chordoma occurs as a sellar or perisellar mass lesions and outlines the limits of their retrosellar and
without erosion of the nearby osseous structures. It may infratentorial extension [6, 1 9, 20]. Most intrasellar tumors
be seen as far anteriorly as the olfactory groove. eccen- do not extend infratentorially [201. This is a very helpful
trically in the floor of the middle cranial fossa. or in the feature to differentiate chordoma from other intrasellar
cerebellopontine angles [6, 1 8, 1 9, 23, 261. tumors. In retrosellar and clival tumors (epidural lesions),
Because of the emphasis placed on the embryologic pneumography facilitates differentiation from intradural
relationship of the notochord with the clivus, intracranial lesions such as meningiomas and from intramedullary
chordomas have come to be associated with clival destruc- tumors. According to Taveras and Wood [19], epidural
tion. Erosion of the clivus typically occurs [24], but not masses almost always allow the passage of air between
always. In fact, a review of the reported cases of intra- the brain structures and the mass, whereas intradural
cranial chordomas reveals a 50% incidence of clival ero- tumors often do not. Furthermore, the cisterna pontis
sion [1 2, 20, 23, 24, 26]. Two of our four patients have may be widened, thus favoring the diagnosis of a prepon-
not developed clival destruction after 3 and 4’/2 years of tine lesion.
 CHORDOMA 803
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Fig. 3.-Vertebral chordoma (case 10). Note large paraspinal mass containing flecks of calcification and fragments of bone associated with destruc-
tive changes of T9-T1 1 vertebrae. T9-T1O and T1O-T1 1 discs are partially destroyed Marked osteosclerosis also present Laminectomy and residual
positive contrast (myelography) are noted.

Obstruction to the flow of the cerebrospinal fluid is

Vertebral and Sacrococcygeal Chordoma
uncommon but may result from compression of the mid-
brain at the incisura of the tentorium [20]. Invasion of the The significant radiographic findings in our 16 patients
floor of the sella and extension into the sphenoid sinus is are summarized in table 2. The most striking feature is
nonspecific and occurs in pituitary tumors, craniopharyn- the presence of multiple asymmetric adjacent vertebral
gioma, chordoma, and primary and secondary nasopharyn- body destruction associated with involvement of the inter-
geal malignancies. Calcification is a common finding in vening disc spaces. This is a unique characteristic of this
intracranial chordomas but is also seen in meningioma. malignancy [2, 8. 1 9, 25]. Another interesting feature is
craniopharyngioma, epidermoid, pituitary tumors, aneu- osteosclerosis, which was seen in seven of our patients.
rysms, and osteochondroma [20, 23, 241. A prespinal It was primarily localized to the periphery of the lytic
nasopharyngeal mass (present in one-third of chordomas) lesion. We feel osteosclerosis is a helpful radiologic sign
is a very helpful finding. This is also seen in nasopharyn- in vertebral and sacral chordomas [5, 20, 25, 28]. In
geal malignancies which may invade the base of the skull pelvic tumors, barium enema and intravenous pyelography
and extend upwards. On the other hand, craniopharyn- may reveal evidence of an extrinsic mass displacing and
gioma, meningioma, pituitary tumors, clival and sellar occasionally invading the pelvic structures [20, 25]. Al-
osteochondroma, and aneurysms do not usually produce though invasion of the bowel may be severe, ulcerations
this finding. Occasionally a cartilagenous tumor of the of the lumen practically never occur.
base of the skull produces findings indistinguishable from Differential diagnosis. Differentiation of vertebral chor-
a chordoma. doma from spinal osteomyelitis, which characteristically
 804 FIROOZNIA El AL.

intervertebral discs. When an expansile lesion of the

sacrum is present, it must be differentiated from plasmo-
cytoma. aneurysmal bone cyst, giant cell tumor, and
expansile metastasis to the bone. These lesions usually do
not have an associated soft tissue mass containing calcifi-
cation and/or fragments of bone, as is often the case with
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chordoma. The most difficult tumor to differentiate from

chordoma is a centrally arising sacral chondrosarcoma
with a soft tissue mass containing calcification. To estab-
lish the histologic diagnosis the simplest biopsy method
available should be utilized, such as pharyngeal and
sphenoidal biopsy, which may have to be repeated several
times to yield a definitive diagnosis.

Prognosis and Treatment

Prognosis of cranial chordoma is poor. Average survival

time following onset of symptoms is about 4-5 years
despite surgery and/or irradiation [6]. Prognosis of sacro-
coccygeal chordoma is better, with an average survival
time of 8-1 0 years [4, 1 0]. Irradiation may produce
significant regression of the cranial and spinal tumors,
with remissions from one to several years. A combination
of irradiation and surgical intervention for decompression
of vital structures is felt to offer the best chance for pro-
Fig. 4.-Sacral chordoma (case 16). Note large destructive lesion of longation of survival [3, 4, 6]. In intracranial chordoma,
sacrum associated with multiple calcific and bony fragments.
total resection of the tumor is impossible in the majority
TABLE 2 of patients. However, surgical intervention often becomes
necessary as a means of decompression, when blindness
Radiologic Findings in Patients with
is threatened, or for performance of an external shunting
Vertebral and Sacroccygeal Chordoma
procedure. Radical surgical procedures have been per-
formed for sacrococcygeal chordoma. In this location,
Type of Study and Findings No Patients
most of the involved structures, such as sacrum, coccyx,
Plain films: and adjacent tissues, can be resected without danger to
Osteolysis 16 life. Although these radical procedures leave the patient
Involvement of two or more seriously disabled, the results are promising in terms of
vertebrae’ 7 recurrence and length of survival [2].
Osteosclerosis 7
Expansile bone lesion ......... 2
Disc involvement 6 REFERENCES
Soft tissue mass . . 10 1 . Horwitz T: Chordal ectopia and its possible relationship to
Calcification . . . . ,,., 7 chordoma. Arch Pathol 31 :354-362, 1941
Myelography: 2. Jaffe HL: Tumors and Tumorous Conditions of the Bones
Epidural defect . , , 8 and Joints. Philadelphia, Lea & Febiger, 1 958
Epidural block . . . . ...... 2 3. Dahlin DC, Macarty CS: Chordoma: a study of fifty-nine
Intradural block . . . , , . , 2 cases. Cancer5:1170-1178, 1952
Angiography: 4. Higinbotham NL, Phillips RF, Farr HW. Hustu HO: Chor-
Vascular lesion ............ 4 doma: thirty-five year study at memorial hospital. Cancer
Tumorblush , 3 20:1841-1850, 1967
Vascular displacement ....... 2 5. Poppen JL, King AB: Chordoma: experience with thirteen
Malignant vessels . . 1
, ,
cases. J Neurosurg 9: 1 39-1 63. 1952
Encasement . . . , , 1 6. Kamrin RP, Potanos JN. Pool JL: An evaluation of the
diagnosis and treatment of chordoma. J Neurol Neurosurg
Note -- 16 patients were studied with plain films, eight un
derwent myelography. and four angiography
Psychiatry 27: 1 57-1 65. 1964
. Excluding sacrum 7. Dahlin DC: Bone Tumors: General Aspects and Data on
3,987 Cases, 2d ed. Springfield, Ill., Thomas, 1967
8. Hsieh CK. Hsieh JH: Roentgenologic study of sacrococcy-
involves the vertebrae with destruction of the intervening
geal chordoma. Radiology 27:101-108, 1936
discs, may be very difficult. On the other hand, primary 9. Sassin JF, Chutorian AM: Intracranial chordoma in children.
and metastatic neoplasms of the spine can usually be ruled Arch Neurol 17:89-93, 1967
out because these lesions generally do not destroy the 10. Faust DB, Gilmore HR. Mudgett CS: Chordomata: a review
 CHORDOMA 805

of the literature with report of a sacrococcygeal case. Ann Williams & Wilkins, 1 964
Intern Med 21 :678-698, 1944 20. Wood EH, Himadi GM: Chordomas: a roentgenological
1 1 Willis AA:
. Pathology of Tumors,
4th ed. London, Butter- study of sixteen cases previously unreported. Radiology 54:
worths, 1967 706-716, 1960
1 2. Schechter MM, Liebskind AL, Azar-kia B: Intracranial chor- 21 . Fox JE, Batsakis JG. Owano LA: Unusual manifestations of
domas. Neuroradiology 8:67-82, 1974 chordoma: a report of two cases. J Bone Joint Surg 50-A:
Downloaded from www.ajronline.org by 66.96.225.125 on 02/10/21 from IP address 66.96.225.125. Copyright ARRS. For personal use only; all rights reserved

13. Stewart MJ, Morin JE: Chordoma: A review with report of 1618-1628. 1968
a new sacrococcygeal case. J Pathol 29:41 -60, 1926 22. Evans AW: Histologica/Appearances of Tumors. Edinburgh,
14. Windeyer BW: Chordoma. Proc R Soc Med 52:1088- E & S Livingstone, 1966
1100, 1959 23. Falconer MA. Bailey IC, Duchen LW: Surgical treatment of
1 5. Ormerod A: A case of chordoma presenting in the naso- chordoma and chondroma of the skull base. J Neurosurg
pharynx. J Laryngol Otol 74:245-254, 1960 29:261-275, 1968
1 6. Wright D: Nasopharyngeal and cervical chordoma: some 24. Dichiro G. Anderson WB: Clivus. Clin Radio! 16:21 1-223,
aspects of their development and treatment. J Laryngol Otol 1965
81:1355-1377, 1967 25. Utne JR. Pugh DG: The roentgenological aspects of chor-
17. Macarty CS. Waugh JM, Coventry MB, O’Sullivan DC: doma. Am J Roentgenol 74:593-608, 1955
Sacrococcygeal chordomas. Surg Gynecol Obstet 1 13: 26. Plaut HF. Blatt ES: Chordoma of the clivus: a report of four
551-554, 1961 cases. Am J Roentgenol 1 00:639-648, 1967
18. Belza J: Double midline intracranial tumors of vestigial 27. Mathews W, Wilson CB: Ectopic intrasellar chordoma. Case
origin: contiguous intrasellar chordoma and suprasellar report. J Neurosurg 39:260-263, 1974
craniopharyngioma. Case Report. J Neurosurg 25:199- 28. Pinto AS, Lin JP, Firooznia H, Lefleur AS: The osseous and
204, 1966 angiographic manifestations of vertebral chordoma. Neuro-
‘19. Taveras JM, Wood E: Diagnostic Neuroradiology. Baltimore, radiology 9:231-241, 1975
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9:S3. . [Crossref]
6. Jiu‑Fa Cui, Da‑Peng Hao, Hai‑Song Chen, Ji‑Hua Liu, Feng Hou, Wen‑Jian Xu. 2018. Computed tomography and magnetic
resonance imaging features of cervical chordoma. Oncology Letters . [Crossref]
7. Ronald Sahyouni, Khodayar Goshtasbi, Amin Mahmoodi, Jefferson W. Chen. 2018. A historical recount of chordoma. Journal
of Neurosurgery: Spine 28:4, 422-428. [Crossref]
8. Abdul-Kareem Ahmed, Hassan Y. Dawood, Omar M. Arnaout, Edward R. Laws, Timothy R. Smith. 2018. Presentation,
Treatment, and Long-Term Outcome of Intrasellar Chordoma: A Pooled Analysis of Institutional, SEER (Surveillance
Epidemiology and End Results), and Published Data. World Neurosurgery 109, e676-e683. [Crossref]
9. Ugur Selek, Erkan Topkan, Eric L. Chang. Skull Base Tumors 261-283. [Crossref]
10. Adam J. Folbe, Peter F. Svider, James K. Liu, Jean Anderson Eloy. 2017. Endoscopic Resection of Clival Malignancies.
Otolaryngologic Clinics of North America 50:2, 315. [Crossref]
11. Laurel A. Littrell, Doris E. Wenger. Imaging of Sacral Tumors and Tumor Simulators: Experience of the Mayo Clinic 25-64.
[Crossref]
12. A. Neelakantan, A.K. Rana. 2014. Benign and malignant diseases of the clivus. Clinical Radiology 69:12, 1295-1303. [Crossref]
13. Wilson C. W. Chan, Tiffany Y. K. Au, Vivian Tam, Kathryn S. E. Cheah, Danny Chan. 2014. Coming together is a beginning:
The making of an intervertebral disc. Birth Defects Research Part C: Embryo Today: Reviews 102:1, 83-100. [Crossref]
14. Bhaskar Mitra, Subhalakshmi Sengupta, Anshita Rai, Jay Mehta, Aruna Rai Quader, Subhendu Roy, Anita Borges. 2014.
Chordoma in Nasopharynx in a 70-Year-Old Female: A Rare Occurence. International Journal of Otolaryngology and Head & Neck
Surgery 03:06, 342-346. [Crossref]
15. Yu Wang, Jianru Xiao, Zhipeng Wu, Quan Huang, Wending Huang, Qing Zhu, Zaijun Lin, Liangzhe Wang. 2012. Primary
chordomas of the cervical spine: a consecutive series of 14 surgically managed cases. Journal of Neurosurgery: Spine 17:4, 292-299.
[Crossref]
16. Brian J. Jian, Orin G. Bloch, Isaac Yang, Seunggu J. Han, Derrick Aranda, Andrew T. Parsa. 2011. A comprehensive analysis of
intracranial chordoma and survival: a systematic review. British Journal of Neurosurgery 25:4, 446-453. [Crossref]
17. S. Semlali, T. Africha, L. Benaissa, M. Jidal, T. Amil, A. Hanine, A. Darbi. 2011. Masse cervicale rare. Feuillets de Radiologie
51:3, 141-145. [Crossref]
18. Hugh D. Curtin, Mari Hagiwara, Peter Som. Pathology of the Central Skull Base 947-1020. [Crossref]
19. Manish N. Shah, Paul Santiago. Congenital Abnormalities of the Thoracic and Lumbar Spine 2973-2978. [Crossref]
20. Brian J. Jian, Orin G. Bloch, Isaac Yang, Seunggu J. Han, Derick Aranda, Tarik Tihan, Andrew T. Parsa. 2010. Adjuvant radiation
therapy and chondroid chordoma subtype are associated with a lower tumor recurrence rate of cranial chordoma. Journal of Neuro-
Oncology 98:1, 101-108. [Crossref]
21. Z.Y. Yan, B.T. Yang, Z.C. Wang, J.F. Xian, M. Li. 2010. Primary Chordoma in the Nasal Cavity and Nasopharynx: CT and MR
Imaging Findings. American Journal of Neuroradiology 31:2, 246-250. [Crossref]
22. Samir K. El-Mofty. Bone Lesions 729-784. [Crossref]
 23. Prudencia N. M. Tyrrell, Victor N. Cassar-Pullicino, Radhesh K. Lalam, Bernhard J. Tins. Bone Metastases 1: Spine 461-479.
[Crossref]
24. Victor N. Cassar-Pullicino, D. C. Mangham. Notochordal Tumours 375-392. [Crossref]
25. Jorge A. Vidal, Mark D. Murphey. 2007. Primary Tumors of the Osseous Spine. Magnetic Resonance Imaging Clinics of North
America 15:2, 239-255. [Crossref]
26. Donald Resnick, Michael Kyriakos, Guerdon D. Greenway. Tumors and Tumor-Like Lesions of Bone: Imaging and Pathology
of Specific Lesions 1120-1198. [Crossref]
Downloaded from www.ajronline.org by 66.96.225.125 on 02/10/21 from IP address 66.96.225.125. Copyright ARRS. For personal use only; all rights reserved

27. Michael Kyriakos, William G. Totty, Lawrence G. Lenke. 2003. Giant Vertebral Notochordal Rest. The American Journal of
Surgical Pathology 27:3, 396-406. [Crossref]
28. Norio AOYAGI, Kuniaki KOJIMA, Hirohiko KASAI. 2003. Review of Spinal Epidural Cavernous Hemangioma. Neurologia
medico-chirurgica 43:10, 471-476. [Crossref]
29. Jean C. Tamraz, Claire Outin, Mario Forjaz Secca, Bassam Soussi. Spine and cord 435-643. [Crossref]
30. Carmen Romera, Andrés Wiehoff, Víctor P.Candela, Juan Perera. 2002. Cordoma cervical: a propósito de un caso. Radiología
44:2, 65-68. [Crossref]
31. Luis A.B. Borba, Benedicto O. Colli, Ossama Al-Mefty. 2001. Skull Base Chordomas. Neurosurgery Quarterly 11:2, 124-139.
[Crossref]
32. Jean-Denis Laredo, Abdeljalil El. Quessar, Philippe Bossard, Valérie Vuillemin-Bodaghi. 2001. Vertebral Tumors And
Pseudotumors. Radiologic Clinics of North America 39:1, 137-163. [Crossref]
33. Kazuo Miyasaka. 1999. Diagnostic Imaging of Spinal Neoplasms : Part 2 Extradural Neoplasms. Spinal Surgery 13:3, 221-234.
[Crossref]
34. K. W. Fraser, C. F. Dowd, M. J. Donovan Post. Interventional Procedures of Vertebral Tumors 151-177. [Crossref]
35. M. A. Van Buchem, V. P. M. Van Der Hulst, J. L. Bloem. Imaging of Extradural Tumors: Primary Tumors 87-134. [Crossref]
36. Servet Inci, Selçuk Palaoğlu, Behsan Önol, Aykut Erbengi. 1996. Low cervical chordoma: case report. Spinal Cord 34:6, 358-360.
[Crossref]
37. O. Karakida, J. Aoki, G. S. Seo, S. Sone, H. Tanikawa, T. Nakakouji, K. Otsuka, K. Ishii. 1996. Epidural dumbbell-shaped
chordoma mimicking a neurinoma. Pediatric Radiology 26:1, 62-64. [Crossref]
38. Jacques Favre, Jean-Pierre Deruaz, Antoine Uske, Nicolas de Tribolet. 1994. Skull base chordomas: presentation of six cases and
review of the literature. Journal of Clinical Neuroscience 1:1, 7-18. [Crossref]
39. R G SCHAMSCHULA, M Y S SOO. 1993. Clival chordomas. Australasian Radiology 37:3, 259-264. [Crossref]
40. Y.R. Yadav, V.K. Kak, V.K. Khosla, N. Khandelwal, B.D. Radotra. 1992. Cranial chordoma in the first decade. Clinical Neurology
and Neurosurgery 94:3, 241-246. [Crossref]
41. Richard C. Cabot, Robert E. Scully, Eugene J. Mark, William F. McNeely, Betty U. McNeely, C.M. Norris, Max L. Goodman.
1992. Case 21-1992. New England Journal of Medicine 326:21, 1417-1424. [Crossref]
42. Jeffrey S. Oppenheim, Bruce R. Rosenblum, Michael Sacher, David Wolfe, Sibylle Wallace, Alan S. Rothman. 1991. Intracranial
chordoma in a preadolescent case report. Surgical Neurology 35:3, 230-233. [Crossref]
43. William M. Merenich, Joel D. Swartz, Philip S. Yussen, George L. Popky, Stephen D. Silberstein. 1991. The foramen ovale MR
and CT correlation. Clinical Imaging 15:1, 20-30. [Crossref]
44. Nagui M. Antoun. The Spine 159-200. [Crossref]
45. Charles S. Resnik, Jeremy W. R. Young, Alan M. Levine, Seena C. Aisner. 1989. Case report 544. Skeletal Radiology 18:4,
303-305. [Crossref]
46. Concezio Di Rocco, Aldo Iannelli, Cesare Colosimo. Intraspinal Tumors 45-120. [Crossref]
47. E. Stennert, W. Thumfart. Chirurgie des Felsenbeins (außer Mittelohr) Tumoren und Pseudotumoren 167-341. [Crossref]
48. THEODORE C. LARSON, O. WAYNE HOUSER, EDWARD R. LAWS. 1987. Imaging of Cranial Chordomas. Mayo Clinic
Proceedings 62:10, 886-893. [Crossref]
49. Michael Brooks, Jonathan Kleefield, Gerald V. O'Reilly, Hani A. Haykal, Meredith MacLeod. 1987. Thoracic chordoma with
unusual radiographic features. Computerized Radiology 11:2, 85-90. [Crossref]
50. Richard K. Sibley, Deborah L. Day, Louis P. Dehner, Robert C. Trueworthy. 1987. Metastasizing Chordoma in Early Childhood.
Pediatric Pathology 7:3, 287-301. [Crossref]
51. C. R. Kannan. Tumors of the Pituitary 443-497. [Crossref]
 52. Franz-Martin Fink, Bernd Ausserer, Wolfgang Schröcksnadel, Anton Kaspar Pallua, Hermann Frommhold, Gregor Mikuz. 1987.
Clivus Chordoma in A 9-Year-Old Child: Case Report and Review of the Literature. Pediatric Hematology and Oncology 4:2,
91-100. [Crossref]
53. N. L. Ataya. 1986. Craniocervical chordomas in the Mersey Region. The Journal of Laryngology & Otology 100:12, 1391-1395.
[Crossref]
54. Hossein Firooznia, Cornelia Golimbu, Mahvash Rafii, Deborah L. Reede, Irvin I. Kricheff, Ann Bjorkengren. 1986. Computed
tomography of spinal chordomas. Journal of Computed Tomography 10:1, 45-50. [Crossref]
Downloaded from www.ajronline.org by 66.96.225.125 on 02/10/21 from IP address 66.96.225.125. Copyright ARRS. For personal use only; all rights reserved

55. M. A. Vaghi, M. G. Bruzzone, A. Visciani, A. Passerini. 1985. Intracranial tumors arising from the floor of the middle fossa. The
Italian Journal of Neurological Sciences 6:4, 469-475. [Crossref]
56. Richard C. Cabot, Robert E. Scully, Eugene J. Mark, Betty U. McNeely, Mark E. Molitch, E. Tessa Hedley-Whyte. 1985. Case
5-1985. New England Journal of Medicine 312:5, 297-305. [Crossref]
57. R. Gahbauer, J. Bay. External Beam Therapy of Brain Tumors 85-178. [Crossref]
58. Robert I. Grossman, Roberta M. Lynch. 1983. Neuroimaging in Neuro-Ophthalmology. Neurologic Clinics 1:4, 831-858.
[Crossref]
59. Timothy B. Mapstone, Benjamin Kaufman, Robert A. Ratcheson. 1983. Intradural chordoma without bone involvement: nuclear
magnetic resonance (NMR) appearance. Journal of Neurosurgery 59:3, 535-537. [Crossref]
60. Y. Hertzanu, R.B.J. Glass, D.B. Mendelsohn. 1983. Sacrococcygeal chordoma in young adults. Clinical Radiology 34:3, 327-329.
[Crossref]
61. Wei-Jen Shih, Richard C. Reba, Tsung-Yao Huang. 1983. Scintigraphic photopenia in sacrococcygeal chordoma. European Journal
of Nuclear Medicine 8:6, 279-280. [Crossref]
62. Morrie E. Kricun. 1983. Radiographic Evaluation of Solitary Bone Lesions. Orthopedic Clinics of North America 14:1, 39-64.
[Crossref]
63. Herman D. Suit, Michael Goitein, John Munzenrider, Lynn Verhey, Kenneth R. Davis, Andreas Koehler, Rita Linggood, Robert
G. Ojemann. 1982. Definitive radiation therapy for chordoma and chondrosarcoma of base of skull and cervical spine. Journal
of Neurosurgery 56:3, 377-385. [Crossref]
64. W. Dihlmann. 1981. Hemispherical spondylosclerosis — A polyetiologic syndrome. Skeletal Radiology 7:2, 99-106. [Crossref]
65. John S. Spratt, Anthony E. Martin, John McKeown. 1981. Sacral chordoma: A case study and review. Journal of Surgical Oncology
18:1, 101-103. [Crossref]
66. Jean François Bonneville, Jean Louis Dietemann. Retrosellar Pathology 155-158. [Crossref]
67. James E. Spoden, Robert M. Bumsted, Emory D. Warner. 1980. Case Report and Literature Review. Annals of Otology, Rhinology
& Laryngology 89:3, 279-285. [Crossref]
68. Joseph L. Zito, Kenneth R. Davis. 1980. The role of computed metrizamide myelography in evaluation of extradural extension
from vertebral chordoma. Journal of Computed Tomography 4:1, 38-42. [Crossref]
69. Robert D. Harwick, Arthur S. Miller. 1979. Craniocervical chordomas. The American Journal of Surgery 138:4, 512-516. [Crossref]
70. Dennis K. Heaston, Martin I. Gelman. 1978. Case report 74. Skeletal Radiology 3:3, 186-190. [Crossref]
71. Iain W. McCall. Radiology and Pathological Correlations of Bone Tumours of the Spine 721-741. [Crossref]
72. . Andere Knochentumoren 311-325. [Crossref]