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Intracranial chordomas
Clin. Radiol. (1974)25, 309-316 INTRACRANIAL CHORDOMAS J. D A N Z I G E R , K. L E W E R A L L E N and S. BLOCH
From the Departments of Radiology and Neurosurgery, Princess Nursing Home, Hillbrow, Johannesburg, Republic of South Africa. This retrospective study of nine cases of intracranial chordomas suggests that the growth pattern of this tumour is either forwards or downwards depending upon the site of origin on the clivus. It is possible to correlate the symptom patterns with the radiographic appearances, and it is now thought possible to predict the evolution of an early case from the site of origin of the lesion from notochordal remnants on the clivus.
A study of patients suffering from intracranial chordomas has shown that it is often possible to correlate the symptom patterns with the radiological appearances, and to predict the direction in which further spread will occur and therefore subsequent symptom patterns.
MATERIAL Nine cases of intracranial chordomas have been seen in the above institution over a 20-year period. In all cases, the diagnosis was proved by histological examination. The histogram (Fig. 1) shows the distribution of the patients by age and sex. RESULTS The exact origin of the tumour from notochordal remnants within the clivus was difficult to identify, because the patients presented with advanced clinical pictures and radiological studies were not done in the early course of the disease. When attempts were made to correlate the clinical findings and radiological appearances, we observed that cranial chordomas could behave in one of two ways :A. Forward extending group. B. Downward extending group. In the forward extending group, it is thought that the tumour arose higher up on the clivus (probably from the basi-sphenoid) and from there it extended anteriorly into the sphenoid, the pituitary fossa (Fig. 2) and subsequently into the optic foramina, superior orbital fissures and orbits. By extradural spread, lateral extension into the middle cranial fossa also occurred (Fig. 3). With involvement of these bones, their corticated margins were lost and areas of rarefaction became evident, expansion of bone occurred and normal
anatomical cavities became obliterated (Wood and Himadi, 1950). These features are summarised in Table 1 (Cases 1-5). In this group the pituitary fossa was always abnormal. It was usually enlarged, with erosion and thinning of the posterior clinoid processes. Undercutting of the anterior clinoid processes was seen in all cases in this group (Fig. 4)~ In the downward extending group, the site of origin was considered to be in the lower part of the clivus, probably from the basiocciput (Fig. 5). Extension from here was downward, producing destruction of basiocciput with involvement of the apical ligament and adjacent cervical vertebrae. The earliest visible site of extension to the cervical spine was the upper margin of the dens (Fig. 6), and with further tumour growth, scalloping along the anterior margins of several cervical vertebrae was observed. At surgery it was noted that involvement of multiple vertebrae was due to growth of tumour along or under the anterior longitudinal ligament. This occurred in three out of four cases in this group. (Table 1, Cases 6, 8 and 9). In one case (No. 9) the neural arch of C2 showed an area of destruction (Fig. 7). Concurrent extension at the level of the clivus took place either into the prepontine cisterns with displacement of the brainstem posteriorly (Fig. 8), or anteriorly into the nasopharynx with development of a nasopharyngeal soft-tissue mass (Fig. 9). The pituitary fossa, sphenoidal sinuses, orbits and superior orbital fissures and upper part of clivus were spared in this group. Nasopharyngeal soft-tissue masses were seen in cases in both groups. CLINICAL PRESENTATION The behaviour of chordomas is arbitarily divided into four stages: 1. Initial silent phase (Intra-osseous phase). Slow 309
310
CLINICAL RADIOLOGY
DM ¢-
~,3 ,
-
[---]
F
m
¢N--
o
~2
rq0 ID _o
E
Z
,'q
10
30
20
40
50
60
Age distribution Fro. 1 HISTOGRAM
FIG. 2 Lateral tomogram of the skull demonstrating destruction of the upper clivus as well as the posterior clinoid processes, dorsum sellae and floor of the pituitary fossa. The sphenoid sinus is also encroached upon. Note that the lower half of the clivus is spared.
FIG. 3 Autopsy specimen showing the extension of the multilobulated t u m o u r into the right middle cranial fossa.
I N T R A C R A N I A L CHORDOMAS
FIG. 4 Lateral tomogram of pituitary fossa demonstrating undercutting of the anterior clinoid processes. The posterior clinoid processes, dorsttm sellae and floor are destroyed.
311
FIG. 5 Lateral tomogram of clivus showing destruction of the lower third. Note erosion of the postero-superior margin of the odontoid peg (1'). The pituitary fossa and sphenoid sinus are normal.
FIG. 6 (A &B) Lateral tomogram of odontoid peg demonstrating destruction of its superior margin ( t ) .
312
C L I N I C A L RADIOLOGY
FIG. 7 Lateral view of cervical spine demonstrating an area of destruction in the neural arch of C2 (1'). Note the destroyed lower clivus and odontoid peg. A large nasopharyngeal mass is present.
FIG. 8 Vertebra] angiogram, arterial phase lateral projection, demonstrating posterior displacement of basilar artery by a mass extending into the posterior fossa. Note the normal pituitary fossa, sphenoid sinus and upper clivus.
growth of the tumour within the clivus is usually silent and may be protracted. Symptoms usually occur only when the tumour breaks out of the clivus and then extends to cuff and constrict nerves and arteries. However, headache may occur due to expansion of the mass within the clivus. 2. Active phase: In this phase the patients present with clinical symptoms and signs which indicate encroachment on vital structures. In the forward extending group this phase was usually associated with complaints referable to the eyes whereas in the downward extending group the complaints were usually referable to compression of posterior fossa contents (Lewer Allen and Kerr, 1968). In both groups the symptoms and signs of raised intracranial pressure were uncommon, and if it occurred it was a late manifestation. This is in contradistinction to another reported series (Utne and Pugh, 1955) where problems associated with raised intracranial pressure were observed at an early stage. 3. Latent active phase: This phase usually occurs after surgery and varied from a few months to 2-3 years, during which the patients enjoyed a reasonable period of health with minimal symptoms, although the tumour was recurring.
FIG. 9 Lateral skull radiograph showing mass within nasopharynx. The lower part of the clivus is destroyed.
INTRACRANIAL
CHORDOMAS
313
TABLE 1 RADIOLOGICAL FINDINGS IN NINE PATIENTS WITH CLIVAL CHORDOMAS
P l a i n F i l m s (Destruction) Angiography Clivus Air Eneephalography (Displacements)
1
4"'--
4.
4.
4-
4.
4.
4.
4.
4.
4.
4-+
+
+
N o t done.
42
4, ' - -
4.
+
+
4.
4.
4.
4.
4.
4.
4.
--
÷
N o t done.
3
+.--
+
+
+
+
+
4-
+
+
+
+
+
+
N o t done.
4
@
+
+
+
+
.
+
--
--
N/D
N o t done.
5
÷
+
+
+
+
--
A q u e d u c t , 4th & posterior part o f 3rd ventricle displaced backwards.
--
N o t done.
6
--
÷
+
Aqueduct & 4th ventricle displaced backwards,
7 • --
• +
÷
Aqueduct &4th ventricle displaced backwards. (also oil Myodil ventriculogram.
+
N o t done.
' --
8
--
+
9
--
+
(+) = Abnormal
.
.
.
.
.
.
.
÷
--
--
(--) = Normal
( N / D ) = N o t done
+
+
-~-
+
+
÷ + with C.
(C) = Calcification
A q u e d u c t & 4th ventricle displaced backwards,
Basilar artery displaced backwards. --
Basilar artery displaced backwards.
Normal. --
Basilar artery displaced backwards.
9 years.
Surgery. Large mass in nasopharynx (with calcification seen radiographically). 5 years. 3 years. 3 months.
Coughing blood-stained sputum. Progressive neck stiffness. Acute pain in neck on movement.
Lower clivus
10 years. Surgery and Radiotherapy. Left conductive deafness. Mass in nasopharynx. 3months. 3months.
Nasal obstruction. Deaf in left ear.
Lower clivus
2 years. Radiotherapy. Trigeminal nerve impairment on right. Accessory and hypoglossal cranial nerve paresis on right. Mass in nasopharynx.
2 months. 2 months. 2 months.
Transient double vision. Difficulty with swallowing. Pain in neck.
Lower clivus
7 years. Surgery and Radiotherapy.
Right abducens paresis. Right hypoglossal nerve paresis. Limited neck movement. Mass in nasopharynx.
8 months. 1 month. 1 month.
Difficulty with speaking. Transient double vision. Neck stiffness and pain in neck.
Lower clivus
1 year.
2 years. Radiotherapy.
No abnormality. Early papilloedema. Nystagmus. Right trochlear and abducens paresis. Sensory impairment in all divisions of trigeminal nerve on the left. Left hypoglossal paresis. Left cerebellar signs.
1 year. l year. I year. 1 year.
Blackouts. Numbness of left side of face. Speech slurred. Choked easily when eating.
Upper clivus
6 months.
5
Headache.
Upper clivus
Biopsy of sphenoid sinus.
Died within 24 hours. No treatment.
Early papilloedema. Bilateral proptosis. Right oculomotor and abducens paresis. Impaired sensation in the 1st & 2nd div. of the trigeminal nerve on the right. Mass in nasopharynx.
4months. 4months. 4months.
Headache on left side of head. Double vision. Periods of mental confusion.
Upper clivus
10 years.
2 months. 14 months. Few weeks. Few weeks.
Headache behind left eye. Failing vision. Discharge from back of nose. Hearing loss on left.
Upper clivus
Surgery and Radiotherapy.
Right optic atrophy. Right proptosis. Right anosmia. Right oculomotor paresis. Mass in nasopharynx. Left optic atrophy. 1st div. of left trigeminal nerve impaired. Right hemiparesis.
Duration of disease 4 years.
Treatment Surgery and Radiotherapy.
Physical examination
6 months.
Duration
Impaired vision.
Complah~t
Upper clivus
s#e
In#ial
4
Case No.
TABLE 3 SUMMARY OF CASE HISTORIES IN NINE PATIENTS WITH CLIVAL CHORDOMAS ON FIRST EXAMINATION.
315
I N T R A C R A N I A L CHORDOMAS TABLE3 CORRELATIONBETWEENPHYSICALEXAMINATION& RADIOGRAPHICAPPEARANCESON FroST EXAMINATION Case No.
Physical examination
Radiograp.hic appearances
1
Right optic atrophy. Right proptosis. Anosmia (Right). Right oculomotor paresis,
Destruction of pituitary fossa, medial wall of right orbit, optic foramen and superior orbital fissure. Extension into anterior cranial fossa.
2
Left optic atrophy. Left trigeminal nerve involved - (lst div.) Right hellaiparesis,
Destruction of pituitary fossa, left superior orbital fissure, optic foramen, medial wall of orbit and inner half of sphenoidal ridge.
3
Early papilloedema. Bilateral proptosis. Right oculomotor and abducens paresis, Impaired sensation in 1st and 2nd div. of trigeminal nerve. (Right)
Destruction of pituitary fossa, medial orbital walls bilaterally, right superior orbital fissure and optic foramen.
4
No signs.
Destruction of pituitary Fossa.
5
Right trochlear and abducens paresis. Sensory impairment in all divisions of trigeminal nerve, (Left). Nystagmus and left cerebellar signs, Left hypoglossal paresis,
Destruction of pituitary fossa, sphenoid sinus an d posterior displacement of 4th ventricle and aqueduct associated with destruction of upper clivus.
6
Right abducens paresis. Right hypoglossal nerve paresis. Limited neck movement,
Destruction of lower clivus with mass extending into posterior fossa. Destruction of odontoid peg in its upper part.
7
Accessory and hypoglossal cranial nerve paresis on right. Mass in nasopharynx,
De struction of lower clivus with posterior dis placement of 4th ventricle and aqueduct.
8
Left conductive deafness.
Destruction of lower clivus. Large mass in nasopharynx.
9
No signs. Mass in nasopharynx.
Destruction of lower clivus with involvement of odontoid peg.
. Stage of" recurrence with clinical deterioration: As with most progressive intracranial neoplasms, recurrence of symptoms and signs inevitably occurred and all patients eventually succumbed to the relentless progression of tumour growth despite surgery and/or radiation therapy.
CORRELATION BETWEEN SYMPTOM PATTERN AND RADIOGRAPHIC APPEARANCES Initially and before surgical intervention there is good correlation between the symptom pattern, physical examination and the radiographic appearances (Tables 2 and 3). In the forward extending group, in all patients presenting with visual impairment, appropriately situated bony abnormalities were seen on plain films of the skull.
Sphenoid sinus opacification was seen in all patients in this group, but no symptom complex could be specifically attributed to this except for headache, especially retro-orbital, which was seen in three patients. Most patients in this group demonstrated nasopharyngeal soft tissue masses on skull radiographs but this seemed to be clinically asymptomatic. When anosmia was present, radiography demonstrated extension of the tumour mass into the anterior cranial fossa. In the downward extending group the commonest presenting symptom was due to involvement of lower cranial nerves, and in particular of the 12th cranial nerve, presenting as dysphagia and dysarthria. Here, destruction of the lower part of the clivus was always evident and was commonly associated with calcification. When dysphagia and cerebellar signs were present, specialised radiographic procedures always demonstrated extension into the posterior fossa.
316
CLINICAL RADIOLOGY
Only one patient in this series presented with conductive deafness and this was due to obstruction of a eustachian tube by the nasopharyngeal mass. Two patients complained of pain in the neck, and in both scalloping of the anterior margin and later destruction of the bodies of upper cervical vertebrae were demonstrated. This correlation often disappears following surgery, as after extensive removal of tumour gross radiographic abnormalities will still be present while symptoms and signs may be minimal. DISCUSSION The radiographic appearances in this group of clivus chordomas have suggested a classification into a forward and downward extending group. Thus from this series it can be suggested that:1. Because the passage of growth has been forward into the sphenoid sinus, pituitary fossa, orbits and related structures and the lower clivus is spared, the tumour has arisen high up on the clivus from notochordal remnants. 2. Because the tumour growth has extended into the posterior fossa or upper cervical spine and the upper clivus is spared, it has arisen from noto-chordal remnants low in the clivus. It is thought from the above information that in a patient presenting early and in which the site or origin of the chordoma is demonstrated radio-
graphically, the radiologist and clinician should be able to predict the direction of spread and consequent clinical and radiographic evolution. This is clearly of importance in the long term follow-up of these patients in that the clinician will be alerted to the development of further clinical signs. PROGNOSIS The prognosis is variable but poor. Recurrences occur despite a full course of radiotherapy and surgery (Poppen and King, 1952). Survival of ten years was seen in one case, although the average was between two and three years. Aekaowledgements.--We would like to thank the Department of Neurosurgery of the Johannesburg General Hospital and the University of the Witwatersrand for allowing us to publish these cases, and to Mrs. J. Sudlow for typing the manuscript.
REFERENCES LEWER-ALLEN, K. & KERR, W. A. (1968). Chordomas of the clivus and the cervical spine. South African Medical Journal, 42, 1165-1174. POPPEN, J. P. & KING, A. B. (1952). Chordoma - experience with thirteen cases. Journal of Neurosurgery, 9, 139-163. UTNE, J. R. & PUCH, D. G. (1955). The roentgenologic aspects of chordomas. American Journal of Roentgenology, 74, 593-608. WOOD, E. H. Jr. • HIMADI, G. M. (1950). Chordomas. Radiology, 54, 706-716.
From the Departments of Radiology and Neurosurgery, Princess Nursing Home, Hillbrow, Johannesburg, Republic of South Africa. This retrospective study of nine cases of intracranial chordomas suggests that the growth pattern of this tumour is either forwards or downwards depending upon the site of origin on the clivus. It is possible to correlate the symptom patterns with the radiographic appearances, and it is now thought possible to predict the evolution of an early case from the site of origin of the lesion from notochordal remnants on the clivus.
A study of patients suffering from intracranial chordomas has shown that it is often possible to correlate the symptom patterns with the radiological appearances, and to predict the direction in which further spread will occur and therefore subsequent symptom patterns.
MATERIAL Nine cases of intracranial chordomas have been seen in the above institution over a 20-year period. In all cases, the diagnosis was proved by histological examination. The histogram (Fig. 1) shows the distribution of the patients by age and sex. RESULTS The exact origin of the tumour from notochordal remnants within the clivus was difficult to identify, because the patients presented with advanced clinical pictures and radiological studies were not done in the early course of the disease. When attempts were made to correlate the clinical findings and radiological appearances, we observed that cranial chordomas could behave in one of two ways :A. Forward extending group. B. Downward extending group. In the forward extending group, it is thought that the tumour arose higher up on the clivus (probably from the basi-sphenoid) and from there it extended anteriorly into the sphenoid, the pituitary fossa (Fig. 2) and subsequently into the optic foramina, superior orbital fissures and orbits. By extradural spread, lateral extension into the middle cranial fossa also occurred (Fig. 3). With involvement of these bones, their corticated margins were lost and areas of rarefaction became evident, expansion of bone occurred and normal
anatomical cavities became obliterated (Wood and Himadi, 1950). These features are summarised in Table 1 (Cases 1-5). In this group the pituitary fossa was always abnormal. It was usually enlarged, with erosion and thinning of the posterior clinoid processes. Undercutting of the anterior clinoid processes was seen in all cases in this group (Fig. 4)~ In the downward extending group, the site of origin was considered to be in the lower part of the clivus, probably from the basiocciput (Fig. 5). Extension from here was downward, producing destruction of basiocciput with involvement of the apical ligament and adjacent cervical vertebrae. The earliest visible site of extension to the cervical spine was the upper margin of the dens (Fig. 6), and with further tumour growth, scalloping along the anterior margins of several cervical vertebrae was observed. At surgery it was noted that involvement of multiple vertebrae was due to growth of tumour along or under the anterior longitudinal ligament. This occurred in three out of four cases in this group. (Table 1, Cases 6, 8 and 9). In one case (No. 9) the neural arch of C2 showed an area of destruction (Fig. 7). Concurrent extension at the level of the clivus took place either into the prepontine cisterns with displacement of the brainstem posteriorly (Fig. 8), or anteriorly into the nasopharynx with development of a nasopharyngeal soft-tissue mass (Fig. 9). The pituitary fossa, sphenoidal sinuses, orbits and superior orbital fissures and upper part of clivus were spared in this group. Nasopharyngeal soft-tissue masses were seen in cases in both groups. CLINICAL PRESENTATION The behaviour of chordomas is arbitarily divided into four stages: 1. Initial silent phase (Intra-osseous phase). Slow 309
310
CLINICAL RADIOLOGY
DM ¢-
~,3 ,
-
[---]
F
m
¢N--
o
~2
rq0 ID _o
E
Z
,'q
10
30
20
40
50
60
Age distribution Fro. 1 HISTOGRAM
FIG. 2 Lateral tomogram of the skull demonstrating destruction of the upper clivus as well as the posterior clinoid processes, dorsum sellae and floor of the pituitary fossa. The sphenoid sinus is also encroached upon. Note that the lower half of the clivus is spared.
FIG. 3 Autopsy specimen showing the extension of the multilobulated t u m o u r into the right middle cranial fossa.
I N T R A C R A N I A L CHORDOMAS
FIG. 4 Lateral tomogram of pituitary fossa demonstrating undercutting of the anterior clinoid processes. The posterior clinoid processes, dorsttm sellae and floor are destroyed.
311
FIG. 5 Lateral tomogram of clivus showing destruction of the lower third. Note erosion of the postero-superior margin of the odontoid peg (1'). The pituitary fossa and sphenoid sinus are normal.
FIG. 6 (A &B) Lateral tomogram of odontoid peg demonstrating destruction of its superior margin ( t ) .
312
C L I N I C A L RADIOLOGY
FIG. 7 Lateral view of cervical spine demonstrating an area of destruction in the neural arch of C2 (1'). Note the destroyed lower clivus and odontoid peg. A large nasopharyngeal mass is present.
FIG. 8 Vertebra] angiogram, arterial phase lateral projection, demonstrating posterior displacement of basilar artery by a mass extending into the posterior fossa. Note the normal pituitary fossa, sphenoid sinus and upper clivus.
growth of the tumour within the clivus is usually silent and may be protracted. Symptoms usually occur only when the tumour breaks out of the clivus and then extends to cuff and constrict nerves and arteries. However, headache may occur due to expansion of the mass within the clivus. 2. Active phase: In this phase the patients present with clinical symptoms and signs which indicate encroachment on vital structures. In the forward extending group this phase was usually associated with complaints referable to the eyes whereas in the downward extending group the complaints were usually referable to compression of posterior fossa contents (Lewer Allen and Kerr, 1968). In both groups the symptoms and signs of raised intracranial pressure were uncommon, and if it occurred it was a late manifestation. This is in contradistinction to another reported series (Utne and Pugh, 1955) where problems associated with raised intracranial pressure were observed at an early stage. 3. Latent active phase: This phase usually occurs after surgery and varied from a few months to 2-3 years, during which the patients enjoyed a reasonable period of health with minimal symptoms, although the tumour was recurring.
FIG. 9 Lateral skull radiograph showing mass within nasopharynx. The lower part of the clivus is destroyed.
INTRACRANIAL
CHORDOMAS
313
TABLE 1 RADIOLOGICAL FINDINGS IN NINE PATIENTS WITH CLIVAL CHORDOMAS
P l a i n F i l m s (Destruction) Angiography Clivus Air Eneephalography (Displacements)
1
4"'--
4.
4.
4-
4.
4.
4.
4.
4.
4.
4-+
+
+
N o t done.
42
4, ' - -
4.
+
+
4.
4.
4.
4.
4.
4.
4.
--
÷
N o t done.
3
+.--
+
+
+
+
+
4-
+
+
+
+
+
+
N o t done.
4
@
+
+
+
+
.
+
--
--
N/D
N o t done.
5
÷
+
+
+
+
--
A q u e d u c t , 4th & posterior part o f 3rd ventricle displaced backwards.
--
N o t done.
6
--
÷
+
Aqueduct & 4th ventricle displaced backwards,
7 • --
• +
÷
Aqueduct &4th ventricle displaced backwards. (also oil Myodil ventriculogram.
+
N o t done.
' --
8
--
+
9
--
+
(+) = Abnormal
.
.
.
.
.
.
.
÷
--
--
(--) = Normal
( N / D ) = N o t done
+
+
-~-
+
+
÷ + with C.
(C) = Calcification
A q u e d u c t & 4th ventricle displaced backwards,
Basilar artery displaced backwards. --
Basilar artery displaced backwards.
Normal. --
Basilar artery displaced backwards.
9 years.
Surgery. Large mass in nasopharynx (with calcification seen radiographically). 5 years. 3 years. 3 months.
Coughing blood-stained sputum. Progressive neck stiffness. Acute pain in neck on movement.
Lower clivus
10 years. Surgery and Radiotherapy. Left conductive deafness. Mass in nasopharynx. 3months. 3months.
Nasal obstruction. Deaf in left ear.
Lower clivus
2 years. Radiotherapy. Trigeminal nerve impairment on right. Accessory and hypoglossal cranial nerve paresis on right. Mass in nasopharynx.
2 months. 2 months. 2 months.
Transient double vision. Difficulty with swallowing. Pain in neck.
Lower clivus
7 years. Surgery and Radiotherapy.
Right abducens paresis. Right hypoglossal nerve paresis. Limited neck movement. Mass in nasopharynx.
8 months. 1 month. 1 month.
Difficulty with speaking. Transient double vision. Neck stiffness and pain in neck.
Lower clivus
1 year.
2 years. Radiotherapy.
No abnormality. Early papilloedema. Nystagmus. Right trochlear and abducens paresis. Sensory impairment in all divisions of trigeminal nerve on the left. Left hypoglossal paresis. Left cerebellar signs.
1 year. l year. I year. 1 year.
Blackouts. Numbness of left side of face. Speech slurred. Choked easily when eating.
Upper clivus
6 months.
5
Headache.
Upper clivus
Biopsy of sphenoid sinus.
Died within 24 hours. No treatment.
Early papilloedema. Bilateral proptosis. Right oculomotor and abducens paresis. Impaired sensation in the 1st & 2nd div. of the trigeminal nerve on the right. Mass in nasopharynx.
4months. 4months. 4months.
Headache on left side of head. Double vision. Periods of mental confusion.
Upper clivus
10 years.
2 months. 14 months. Few weeks. Few weeks.
Headache behind left eye. Failing vision. Discharge from back of nose. Hearing loss on left.
Upper clivus
Surgery and Radiotherapy.
Right optic atrophy. Right proptosis. Right anosmia. Right oculomotor paresis. Mass in nasopharynx. Left optic atrophy. 1st div. of left trigeminal nerve impaired. Right hemiparesis.
Duration of disease 4 years.
Treatment Surgery and Radiotherapy.
Physical examination
6 months.
Duration
Impaired vision.
Complah~t
Upper clivus
s#e
In#ial
4
Case No.
TABLE 3 SUMMARY OF CASE HISTORIES IN NINE PATIENTS WITH CLIVAL CHORDOMAS ON FIRST EXAMINATION.
315
I N T R A C R A N I A L CHORDOMAS TABLE3 CORRELATIONBETWEENPHYSICALEXAMINATION& RADIOGRAPHICAPPEARANCESON FroST EXAMINATION Case No.
Physical examination
Radiograp.hic appearances
1
Right optic atrophy. Right proptosis. Anosmia (Right). Right oculomotor paresis,
Destruction of pituitary fossa, medial wall of right orbit, optic foramen and superior orbital fissure. Extension into anterior cranial fossa.
2
Left optic atrophy. Left trigeminal nerve involved - (lst div.) Right hellaiparesis,
Destruction of pituitary fossa, left superior orbital fissure, optic foramen, medial wall of orbit and inner half of sphenoidal ridge.
3
Early papilloedema. Bilateral proptosis. Right oculomotor and abducens paresis, Impaired sensation in 1st and 2nd div. of trigeminal nerve. (Right)
Destruction of pituitary fossa, medial orbital walls bilaterally, right superior orbital fissure and optic foramen.
4
No signs.
Destruction of pituitary Fossa.
5
Right trochlear and abducens paresis. Sensory impairment in all divisions of trigeminal nerve, (Left). Nystagmus and left cerebellar signs, Left hypoglossal paresis,
Destruction of pituitary fossa, sphenoid sinus an d posterior displacement of 4th ventricle and aqueduct associated with destruction of upper clivus.
6
Right abducens paresis. Right hypoglossal nerve paresis. Limited neck movement,
Destruction of lower clivus with mass extending into posterior fossa. Destruction of odontoid peg in its upper part.
7
Accessory and hypoglossal cranial nerve paresis on right. Mass in nasopharynx,
De struction of lower clivus with posterior dis placement of 4th ventricle and aqueduct.
8
Left conductive deafness.
Destruction of lower clivus. Large mass in nasopharynx.
9
No signs. Mass in nasopharynx.
Destruction of lower clivus with involvement of odontoid peg.
. Stage of" recurrence with clinical deterioration: As with most progressive intracranial neoplasms, recurrence of symptoms and signs inevitably occurred and all patients eventually succumbed to the relentless progression of tumour growth despite surgery and/or radiation therapy.
CORRELATION BETWEEN SYMPTOM PATTERN AND RADIOGRAPHIC APPEARANCES Initially and before surgical intervention there is good correlation between the symptom pattern, physical examination and the radiographic appearances (Tables 2 and 3). In the forward extending group, in all patients presenting with visual impairment, appropriately situated bony abnormalities were seen on plain films of the skull.
Sphenoid sinus opacification was seen in all patients in this group, but no symptom complex could be specifically attributed to this except for headache, especially retro-orbital, which was seen in three patients. Most patients in this group demonstrated nasopharyngeal soft tissue masses on skull radiographs but this seemed to be clinically asymptomatic. When anosmia was present, radiography demonstrated extension of the tumour mass into the anterior cranial fossa. In the downward extending group the commonest presenting symptom was due to involvement of lower cranial nerves, and in particular of the 12th cranial nerve, presenting as dysphagia and dysarthria. Here, destruction of the lower part of the clivus was always evident and was commonly associated with calcification. When dysphagia and cerebellar signs were present, specialised radiographic procedures always demonstrated extension into the posterior fossa.
316
CLINICAL RADIOLOGY
Only one patient in this series presented with conductive deafness and this was due to obstruction of a eustachian tube by the nasopharyngeal mass. Two patients complained of pain in the neck, and in both scalloping of the anterior margin and later destruction of the bodies of upper cervical vertebrae were demonstrated. This correlation often disappears following surgery, as after extensive removal of tumour gross radiographic abnormalities will still be present while symptoms and signs may be minimal. DISCUSSION The radiographic appearances in this group of clivus chordomas have suggested a classification into a forward and downward extending group. Thus from this series it can be suggested that:1. Because the passage of growth has been forward into the sphenoid sinus, pituitary fossa, orbits and related structures and the lower clivus is spared, the tumour has arisen high up on the clivus from notochordal remnants. 2. Because the tumour growth has extended into the posterior fossa or upper cervical spine and the upper clivus is spared, it has arisen from noto-chordal remnants low in the clivus. It is thought from the above information that in a patient presenting early and in which the site or origin of the chordoma is demonstrated radio-
graphically, the radiologist and clinician should be able to predict the direction of spread and consequent clinical and radiographic evolution. This is clearly of importance in the long term follow-up of these patients in that the clinician will be alerted to the development of further clinical signs. PROGNOSIS The prognosis is variable but poor. Recurrences occur despite a full course of radiotherapy and surgery (Poppen and King, 1952). Survival of ten years was seen in one case, although the average was between two and three years. Aekaowledgements.--We would like to thank the Department of Neurosurgery of the Johannesburg General Hospital and the University of the Witwatersrand for allowing us to publish these cases, and to Mrs. J. Sudlow for typing the manuscript.
REFERENCES LEWER-ALLEN, K. & KERR, W. A. (1968). Chordomas of the clivus and the cervical spine. South African Medical Journal, 42, 1165-1174. POPPEN, J. P. & KING, A. B. (1952). Chordoma - experience with thirteen cases. Journal of Neurosurgery, 9, 139-163. UTNE, J. R. & PUCH, D. G. (1955). The roentgenologic aspects of chordomas. American Journal of Roentgenology, 74, 593-608. WOOD, E. H. Jr. • HIMADI, G. M. (1950). Chordomas. Radiology, 54, 706-716.