ClinicalNeurology and Neurosurgery,94 (1992) 241-246 0 1992 Elsevier Science Publishers B.V. All rights reserved 0303-8467/92/$05.00
CLINEU
241
00194
Case report
Cranial chordoma in the first decade Y.R. Yadav”, V.K. Kak”, V.K. Khosla”, N. Khandelwalb and B.D. Radotrac Departments of “Neurosurgery, bRadiodiagnosis and ‘Neuropathology, Postgraduate Institute of Medical Education and Research, Chandigarh - 160 012, India (Received (Revised,
(Accepted
Key words:
15 January,
received
1991)
12 February,
12 February,
1992)
1992)
Chordoma; Skull tumour; Clivus; Radiation therapy; Pediatric brain tumour; Base of skull; Management; Prognosis
Summary
Cranial chordomas are extremely rare in childhood with only 25 cases having been reported in the first decade of life. A 6-year-old female child with cranial chordoma is reported. Literature on the subject is reviewed, with special reference to the management, histopathological features and prognosis in childhood chordomas as compared to the adult variety.
Introduction
Chordomas, derived from the remnants of the notochord, can occur anywhere along the vertebral column or the base of skull and account for O.l-0.2% of all primary brain tumours. The sacrum (50%) is involved more often as compared to the clivus (35%) and other vertebrae (15%) [1,2]. Though benign, the location, locally aggressive nature, high rate of recurrence and the tendency to metastasize make treatment of chordomas problematic [3-71. Chordomas commonly present in the fourth and fifth decades of life. Intracranial childhood chordomas (CC) are extremely rare with only 25 cases reported in the first decade of life [3,5-251. However, no attempt has so far been made to find out as to how they differ from adult chordomas and their histopathological correlation. We report here a 6-year-old female child with a cranial Correspondence to: Dr. V.K. Kak, Postgraduate garh-160
Institute
012, India.
of Medical
Department
Education
of Neurosurgery.
and Research,
Chandi-
chordoma along with a review of the literature on CC highlighting their clinical features, management, histopathology and the prognosis.
Case report
A 6-year-old female child presented with a history of intermittent drooping of the eyelids for 10 months, headache and vomiting for 8 months, diminution of vision in both eyes for 1 l/2 months and proptosis of the right eye of 15 days duration. Examination revealed signs of raised intracranial pressure with no perception of light in the left eye and finger counting at 6 m in the right eye. She had bilateral proptosis, bilateral total ophthalmoplegia and bilateral trigeminal sensory deficit involving all the three divisions. Skull x-ray showed destruction of the sella and the middle cranial fossa with calcification. CT scan showed bilateral sellar and parasellar enhancing mass, more on the left side encroaching the left orbit and ethmoid sinuses (Fig. 1). A left temporal craniotomy and partial excision of the tumour was done. The postopera-
242
Fig. 2. Photomicrograph of chordoma showing cords of polyhedral cells with intercellular myxoid background (H&E, Fig. 1. Contrast-enhanced CT scan showing bilateral sellar and parasellar enhancing mass, more on the left side, encroaching on the left orbit and ethmoid sinuses.
x 140).
though tive course was uneventful. Histological examination revealed a chordoma with no significant nuclear hyperchromatism or pleomorphism (Fig. 2). She was discharged from the hospital on the 8th day with the advise to undergo radiation therapy, but she deteriorated on the 21 st postoperative day and died the following day at her home.
Discussion
highly characteristic
imaging
features
are being
reported. Histologically most of these tumours are benign, but locally invasive. However, a few are truly malignant and show distant metastases. A variant of chordoma, designated as “chondroid chordoma”. has been proposed when sufficient cartilaginous areas are noted, but the existence of this variant has recently been refuted based on immunohistochemical studies [27]. There have been several immunohistochemical studies on chordomas. It is generally assumed that chordoma is positive for epithelial marker proteins. such as EMA, CK and tissue polypeptide antigen [25,28-301. Clusters of mito-
chondrosis.
chondria surrounded by rough endoplasmic reticulum and desmosomes have been shown in chordoma cells on electron microscopy (25,31,32]. CT scan findings include an enhancing soft tissue mass with erosion of the clivus and sella turcica. Tumour calcification or bone sequestration within the tumour is seen
Intracranial chordomas are common in the fourth and fifth decades [19]. Presentation in the first decade is extremely rare and only 25 cases have been reported in the
in 30~70% of cases [2,19,33,34]. Solitary or multiple low attenuation areas in the tumour are representative of its myxoid and gelatinous elements [34]. MRI, especially in
world literature (Table 1) [3,5525]. The sex incidence varies [26] in adult chordomas whereas only 8 of the 25 reported childhood chordomas were females. However,
the sagittal plane, defines the relationship of the tumour to the clivus and the extent and location of its extra-axial extension [22]. The T,-weighted images usually show a
details of two reported cases were not known. Chordoma generally grows slowly and the average duration of symptoms is about 20 months [7]. In childhood chordomas, the duration of symptoms varied from 3 to 13 months. Clinical presentation with raised intracranial pressure and multiple cranial nerves involvement is common to both adult and children [3,7,8,19]. The diagnosis of chordoma is essentially histological
higher signal intensity than T,-weighted imaged [35]. Septations of intermediate signal intensity, corresponding to pathologic fibrous septae, have been demonstrated on axial MRI [23,36]. Surgery and radiotherapy are the available modes of
Cranial chordomas localise along the axis of the embryonic notochord in craniocaudal direction [8]. They may be along the upper clivus (basisphenoid), lower clivus (basiocciput) or below the spheno-occipital syn-
treatment
for chordomas.
Although
gross total removal
would be the goal of any surgical procedure. gical excision is limited in cranial chordomas
radical surdue to their
243 TABLE 1 REPORTED S. No.
1. 2. 3. 4. 5. 6. I. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24.
CASES OF CRANIAL
Author -
(year) Adson et al. (1935) Schneegans and Mandingas (1938) Sassin and Chutorian (1967) Arana Iniguez et al. (1968) Bartel and Heilbronn (1970) Haffelfinger et al. (1973) Becker et al. (1975) Plese et al. (1978) Nolte (1979) Chambers and Schwinn (1979) Brooks et al. (1981) Cummings et al. (1983) Nakamura et al. (1983) Nakamura et al. (1983) Wold and Laws (1983) Wold and Laws (1983) Wold and Laws (1983) Raffel et al. (1985) Handa et al. (1987) Schroeder et al. (1987) Sibley et al. (1987) Sen et al. (1989) Matsumoto et al. (1989) Nagib et al. (1990)
CHORDOMAS
IN THE FIRST DECADE
Age (years)
Sex
Location
Treatment
Outcome
8
M
Clivus
Surgery/RT
Death - 3 weeks
‘is
M
Clivus
RT
Death - 18 months
2%
M
Clivus
RT
Death - 3 months
8
F
Basioccipital
Surgery
Alive - 26 months
1%
M
Basioccipital
Surgery
Alive - 6 months
8
?
Intracranial
Surgery
7
‘I4
M
Clivus
Surgery/RT
Alive - 4 years
6%
F
Basisphenoid
Surgery
Death - postoperative
3
M
Clivus
SurgerylRT
Death - 9 months
5
M
Cranial
Surgery
Death - 7 years
1‘I2
M
Clivus
RTKhemotherapy
Death - 7 months
2
M
Clivus
Surgery/RT
Alive - 10 years
4
F
Clivus
Surgery
Death - 1 day
1%
M
Clivus
Surgery/RT
Death - 9 months
8
M
Basioccipital
SurgeryiRT
Death - 1 month
8
M
Basioccipital
Surgery/RT
Alive - 1 month
8
F
Clivus
Surgery/RT
Alive - 7 years
9
M
Clivus
Surgery
Death - 13 months
9
M
Clivus
SurgeryiRT
Alive - 12 months
4%
F
Clivus
Biopsy
?
2
F
Clivus+C.l/2
‘)
Death - 3 months
3
M
Clivus
SurgerylRT
Death - 6 months
‘I’
M
Clivus
5
F
Basioccipital
Autopsy diagnosis Surgery/RT
Alive - 3 years
244 TABLE 1 (continued) REPORTED
CASES OF CRANIAL
S. No.
Author
25.
Kaneko et al. (1991) Present case (1992)
26.
(year)
location
and extension.
enabled
the performance
base operations
CHORDOMAS
IN THE FIRST DECADE
Age (years)
Sex
Location
Treatment
Outcome
4
F
Clivus
Surgery
Death - 9 months
6
F
Basisphenoid
Surgery
Death - 22 days
Microsurgical
techniques
of middle and posterior
with acceptable
morbidity
only a moderate
have
[16,40,41].
cranial
quired
[22,37-391.
dose of radiation
Rich et al. reported
and most recur locally that tumour
6500&7000 cGy [42]. Radiation
control
re-
doses in excess of
The basal subfrontal approach [37] is excellent for tumours invading the ethnoid and sphenoid sinuses and the clivus. The subtemporal and preauricular infratemporal
8000 cGy offered an 80% chance of tumour control, but had obvious limitations due to neurotoxicity. Encouraging results have been reported with the use of proton
approach to middle and posterior cranial base provides an extensive lateral exposure with control of the petrous internal carotid artery [38]. A combination of the
beam and helium ion therapy [43-451. A better prognosis in children than adults has been reported in a small retrospective analysis [ 181.
subfrontal transbasal and infratemporal approaches has been recommended for more extensive tumours [22]. A combined transoral labiomandibular approach has been used by Nagib et al. for basioccipital chordomas in chil-
The prediction of biologic behaviour of childhood chordomas is difficult. Although intracranial chordomas are benign neoplasms. the possibility of rapid growth and a tendency to metastasize should be considered in the management of childhood chordomas. The reason for this aggressive behaviour is not clear. Smaller tu-
dren [24]. The effect of radiation
therapy
on chordomas
varies
mours
greatly. With conventional radiation modalities such as x-rays or cobalt-60, these tumours can usually be given
and males
prognosis
have been reported
[43]. The
histologic
subtype
to have a better of chondroid
TABLE 2 Studies with histological
No. of
Adult intracranial
First decade intracranial
chordoma
chordoma
patients
group
Heffelfinger et al. ( 1973) [ 121: (A) Chondroid 19 (B) Typical and others.’ 36
No. of patients
Duration of survival
Mortality rate
Recurrence
Metastases
No. of patients
Duration of survival
Mortality rate
Recurrence
Metas-
(%)
(years)
(%)
(%)
(%)
(%)
(weeks)
(%)
(%)
(%)
34.5 65.5
15.8 4.1
_ _
_
I”
_
_
_ _
_
_ _.
16.2 65.0
Id
36
_
tases
(P
CLINEU
241
00194
Case report
Cranial chordoma in the first decade Y.R. Yadav”, V.K. Kak”, V.K. Khosla”, N. Khandelwalb and B.D. Radotrac Departments of “Neurosurgery, bRadiodiagnosis and ‘Neuropathology, Postgraduate Institute of Medical Education and Research, Chandigarh - 160 012, India (Received (Revised,
(Accepted
Key words:
15 January,
received
1991)
12 February,
12 February,
1992)
1992)
Chordoma; Skull tumour; Clivus; Radiation therapy; Pediatric brain tumour; Base of skull; Management; Prognosis
Summary
Cranial chordomas are extremely rare in childhood with only 25 cases having been reported in the first decade of life. A 6-year-old female child with cranial chordoma is reported. Literature on the subject is reviewed, with special reference to the management, histopathological features and prognosis in childhood chordomas as compared to the adult variety.
Introduction
Chordomas, derived from the remnants of the notochord, can occur anywhere along the vertebral column or the base of skull and account for O.l-0.2% of all primary brain tumours. The sacrum (50%) is involved more often as compared to the clivus (35%) and other vertebrae (15%) [1,2]. Though benign, the location, locally aggressive nature, high rate of recurrence and the tendency to metastasize make treatment of chordomas problematic [3-71. Chordomas commonly present in the fourth and fifth decades of life. Intracranial childhood chordomas (CC) are extremely rare with only 25 cases reported in the first decade of life [3,5-251. However, no attempt has so far been made to find out as to how they differ from adult chordomas and their histopathological correlation. We report here a 6-year-old female child with a cranial Correspondence to: Dr. V.K. Kak, Postgraduate garh-160
Institute
012, India.
of Medical
Department
Education
of Neurosurgery.
and Research,
Chandi-
chordoma along with a review of the literature on CC highlighting their clinical features, management, histopathology and the prognosis.
Case report
A 6-year-old female child presented with a history of intermittent drooping of the eyelids for 10 months, headache and vomiting for 8 months, diminution of vision in both eyes for 1 l/2 months and proptosis of the right eye of 15 days duration. Examination revealed signs of raised intracranial pressure with no perception of light in the left eye and finger counting at 6 m in the right eye. She had bilateral proptosis, bilateral total ophthalmoplegia and bilateral trigeminal sensory deficit involving all the three divisions. Skull x-ray showed destruction of the sella and the middle cranial fossa with calcification. CT scan showed bilateral sellar and parasellar enhancing mass, more on the left side encroaching the left orbit and ethmoid sinuses (Fig. 1). A left temporal craniotomy and partial excision of the tumour was done. The postopera-
242
Fig. 2. Photomicrograph of chordoma showing cords of polyhedral cells with intercellular myxoid background (H&E, Fig. 1. Contrast-enhanced CT scan showing bilateral sellar and parasellar enhancing mass, more on the left side, encroaching on the left orbit and ethmoid sinuses.
x 140).
though tive course was uneventful. Histological examination revealed a chordoma with no significant nuclear hyperchromatism or pleomorphism (Fig. 2). She was discharged from the hospital on the 8th day with the advise to undergo radiation therapy, but she deteriorated on the 21 st postoperative day and died the following day at her home.
Discussion
highly characteristic
imaging
features
are being
reported. Histologically most of these tumours are benign, but locally invasive. However, a few are truly malignant and show distant metastases. A variant of chordoma, designated as “chondroid chordoma”. has been proposed when sufficient cartilaginous areas are noted, but the existence of this variant has recently been refuted based on immunohistochemical studies [27]. There have been several immunohistochemical studies on chordomas. It is generally assumed that chordoma is positive for epithelial marker proteins. such as EMA, CK and tissue polypeptide antigen [25,28-301. Clusters of mito-
chondrosis.
chondria surrounded by rough endoplasmic reticulum and desmosomes have been shown in chordoma cells on electron microscopy (25,31,32]. CT scan findings include an enhancing soft tissue mass with erosion of the clivus and sella turcica. Tumour calcification or bone sequestration within the tumour is seen
Intracranial chordomas are common in the fourth and fifth decades [19]. Presentation in the first decade is extremely rare and only 25 cases have been reported in the
in 30~70% of cases [2,19,33,34]. Solitary or multiple low attenuation areas in the tumour are representative of its myxoid and gelatinous elements [34]. MRI, especially in
world literature (Table 1) [3,5525]. The sex incidence varies [26] in adult chordomas whereas only 8 of the 25 reported childhood chordomas were females. However,
the sagittal plane, defines the relationship of the tumour to the clivus and the extent and location of its extra-axial extension [22]. The T,-weighted images usually show a
details of two reported cases were not known. Chordoma generally grows slowly and the average duration of symptoms is about 20 months [7]. In childhood chordomas, the duration of symptoms varied from 3 to 13 months. Clinical presentation with raised intracranial pressure and multiple cranial nerves involvement is common to both adult and children [3,7,8,19]. The diagnosis of chordoma is essentially histological
higher signal intensity than T,-weighted imaged [35]. Septations of intermediate signal intensity, corresponding to pathologic fibrous septae, have been demonstrated on axial MRI [23,36]. Surgery and radiotherapy are the available modes of
Cranial chordomas localise along the axis of the embryonic notochord in craniocaudal direction [8]. They may be along the upper clivus (basisphenoid), lower clivus (basiocciput) or below the spheno-occipital syn-
treatment
for chordomas.
Although
gross total removal
would be the goal of any surgical procedure. gical excision is limited in cranial chordomas
radical surdue to their
243 TABLE 1 REPORTED S. No.
1. 2. 3. 4. 5. 6. I. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24.
CASES OF CRANIAL
Author -
(year) Adson et al. (1935) Schneegans and Mandingas (1938) Sassin and Chutorian (1967) Arana Iniguez et al. (1968) Bartel and Heilbronn (1970) Haffelfinger et al. (1973) Becker et al. (1975) Plese et al. (1978) Nolte (1979) Chambers and Schwinn (1979) Brooks et al. (1981) Cummings et al. (1983) Nakamura et al. (1983) Nakamura et al. (1983) Wold and Laws (1983) Wold and Laws (1983) Wold and Laws (1983) Raffel et al. (1985) Handa et al. (1987) Schroeder et al. (1987) Sibley et al. (1987) Sen et al. (1989) Matsumoto et al. (1989) Nagib et al. (1990)
CHORDOMAS
IN THE FIRST DECADE
Age (years)
Sex
Location
Treatment
Outcome
8
M
Clivus
Surgery/RT
Death - 3 weeks
‘is
M
Clivus
RT
Death - 18 months
2%
M
Clivus
RT
Death - 3 months
8
F
Basioccipital
Surgery
Alive - 26 months
1%
M
Basioccipital
Surgery
Alive - 6 months
8
?
Intracranial
Surgery
7
‘I4
M
Clivus
Surgery/RT
Alive - 4 years
6%
F
Basisphenoid
Surgery
Death - postoperative
3
M
Clivus
SurgerylRT
Death - 9 months
5
M
Cranial
Surgery
Death - 7 years
1‘I2
M
Clivus
RTKhemotherapy
Death - 7 months
2
M
Clivus
Surgery/RT
Alive - 10 years
4
F
Clivus
Surgery
Death - 1 day
1%
M
Clivus
Surgery/RT
Death - 9 months
8
M
Basioccipital
SurgeryiRT
Death - 1 month
8
M
Basioccipital
Surgery/RT
Alive - 1 month
8
F
Clivus
Surgery/RT
Alive - 7 years
9
M
Clivus
Surgery
Death - 13 months
9
M
Clivus
SurgeryiRT
Alive - 12 months
4%
F
Clivus
Biopsy
?
2
F
Clivus+C.l/2
‘)
Death - 3 months
3
M
Clivus
SurgerylRT
Death - 6 months
‘I’
M
Clivus
5
F
Basioccipital
Autopsy diagnosis Surgery/RT
Alive - 3 years
244 TABLE 1 (continued) REPORTED
CASES OF CRANIAL
S. No.
Author
25.
Kaneko et al. (1991) Present case (1992)
26.
(year)
location
and extension.
enabled
the performance
base operations
CHORDOMAS
IN THE FIRST DECADE
Age (years)
Sex
Location
Treatment
Outcome
4
F
Clivus
Surgery
Death - 9 months
6
F
Basisphenoid
Surgery
Death - 22 days
Microsurgical
techniques
of middle and posterior
with acceptable
morbidity
only a moderate
have
[16,40,41].
cranial
quired
[22,37-391.
dose of radiation
Rich et al. reported
and most recur locally that tumour
6500&7000 cGy [42]. Radiation
control
re-
doses in excess of
The basal subfrontal approach [37] is excellent for tumours invading the ethnoid and sphenoid sinuses and the clivus. The subtemporal and preauricular infratemporal
8000 cGy offered an 80% chance of tumour control, but had obvious limitations due to neurotoxicity. Encouraging results have been reported with the use of proton
approach to middle and posterior cranial base provides an extensive lateral exposure with control of the petrous internal carotid artery [38]. A combination of the
beam and helium ion therapy [43-451. A better prognosis in children than adults has been reported in a small retrospective analysis [ 181.
subfrontal transbasal and infratemporal approaches has been recommended for more extensive tumours [22]. A combined transoral labiomandibular approach has been used by Nagib et al. for basioccipital chordomas in chil-
The prediction of biologic behaviour of childhood chordomas is difficult. Although intracranial chordomas are benign neoplasms. the possibility of rapid growth and a tendency to metastasize should be considered in the management of childhood chordomas. The reason for this aggressive behaviour is not clear. Smaller tu-
dren [24]. The effect of radiation
therapy
on chordomas
varies
mours
greatly. With conventional radiation modalities such as x-rays or cobalt-60, these tumours can usually be given
and males
prognosis
have been reported
[43]. The
histologic
subtype
to have a better of chondroid
TABLE 2 Studies with histological
No. of
Adult intracranial
First decade intracranial
chordoma
chordoma
patients
group
Heffelfinger et al. ( 1973) [ 121: (A) Chondroid 19 (B) Typical and others.’ 36
No. of patients
Duration of survival
Mortality rate
Recurrence
Metastases
No. of patients
Duration of survival
Mortality rate
Recurrence
Metas-
(%)
(years)
(%)
(%)
(%)
(%)
(weeks)
(%)
(%)
(%)
34.5 65.5
15.8 4.1
_ _
_
I”
_
_
_ _
_
_ _.
16.2 65.0
Id
36
_
tases
(P
