Spinal Cord and Spinal Column Tumours (SCSCT) are much less common
than intracranial tumours. Despite this lower rate, it constitutes a
major part of neurosurgical emergencies. Many are benign with an excellent
prognosis as meningiomas, defined by Cushing and Eisenhardti (10) as
"one of the most gratifying of all procedures". More than a century
since the first publication of Charcot (4) about paraplegia in cancer
patients, cancerous tumours of the spinal column have always had a poor
outcome. To be able "to make" an early diagnosis and to treat SCSCT
remains a constant wish among neurosurgeons.
The purpose of this review was to study the incidence, neurological
findings, pathological aspects, treatment modalities and the surgical
outcome in these cases.
Patient Population
After exclusion of arachnoid cysts, lipomas with dysraphisms and tumours
of cauda equina, there were 150 patients with SCSCT and operated upon
between 1991 and 1997 in our two institutions (Salim Zemirli Hospital
and Ait Idir Hospital). During the same period, 1170 intracranial tumours
were operated leading to a ratio of brain to spinal cord tumours 8:1.
The patients included 78 females and 72 males with age at presentation
ranging from 2 to 74 years (mean of 32.5 years). Twenty-one (14%) of
these tumours were located in the cervical spinal canal, 104 (69.3%)
were in the dorsal, 24 (16%) within the lumbar and one case of a tumour
involving the holocord. Sixty-eight of them (45.3%) were intradural
extramedullary.
Pathological examination
Histological typing of these 150 tumours was as follows: Neurilemmomas
in 31 cases representing 20.6% of all SCSCT.
There were 19 cases of meningiomas, 12.6% of the total series. Epidermoid
and dermoid cysts in 7 patients. Lipomas in 3 cases, Teratomas 2 cases,
Lymphomas 2 cases, Haemangioblastoms in 2 cases and Hamartoma in 1 case
were present. Tumours of neuroepithelial origin were estimated at 21
cases (14%) which included 11 ependymomas, 7 astrocytom, 1 PNET (Primitive
Neuro Ectodermic nimours), 1 Ependymoblastoma and 1 Neuroblastoma. All
these tumours were located intramedullary.
Primary bone tumours were 13 and 48 cases of secondary neoplasms, which
represented respectively 8.6% and 32% of the total series. Most of them
originated from lung, breast, lymphoma, kidney, prostate and digestive
system. In children histological examination showed 3 ependymomas, 3
neurilemmomas, 2 dermoid cysts, 2 teratomas, 2 Ewing sarcomas, one epidermoid
cyst and 1 metastases of medulloblastoma. Under 3 years of age, we counted
1 ependymoma and 1 dermoid cyst.
Age and Sex Distribution
The peak incidence occurred in the third (28 cases), fourth (26 cases)
and fifth decades (30 cases). Slight predominance of females was observed
in the third, fourth and fifth decades. Four patients were less than
10 years old and 14 were less than 15 years of age (amounting to 9.3%
of the total cases). The analysis of age with the type of tumour showed
a peak incidence in the fifth decade for neurinomas and meningiomas.
Neuro epithelial tumours were exceptional after the fifth decade whereas
metastases were rare before the third decade. Female to male ratio was
1.7 to 1.4 in neurinomas and 3 to 1 in meningiomas.
Clinical features
The patient symptoms at the time of diagnosis were local or radicular
pain in 65 cases (43.3%), sensation of heaviness or weakness in the
legs in 45 cases (30%) whereas 100 patients (66.6%) complained of sphincter
disturbances .
Clinical findings were regrouped in three groups of progressive severe
neurological disability. Forty-five patients (30%) had moderate to severe
neurological syndrome. The remaining 72 patients (48%) presented hyperspastic
to flaccid paralysis with decreased sensation to pain pinprick, light
touch and proprioception below the corresponding dermatome with sphincter
deficit requiring bladder catheter. At the extreme limit, it was the
medullary syndrome of spinal cord transection.
Plain X ray films were performed pre operatively in all patients, myelography
in 101 cases, computed tomography scans in 78 cases, post myelogram
CT in 42 while magnetic resonance imaging was done only in 24 patients.
Mortality rate
In our series, there were six operative deaths (4%). Two patients died
from acute respiratory insufficiency. Two other patients died from septicaemia.
The fifth patient died from recurrence of intracranial medulloblastoma
whereas the sixth cause of death was the pre-operative bleeding in thyroid
metastases.
Recurrences
Of the 150 patients, 3 developed recurrences. In 2 patients with neurilemmomas
the tumour recurred within one and three years. In the third patient
both epi and intradural meningioma recurred within 3 years.
Follow up findings
At one-month follow up, 92 patients have shown improvement of their
neurological examination. Despite early mobilisation following surgical
treatment, we reported 54 cases of stabilisation and 3 cases of deterioration.
The outcome was influenced by the histological type of the tumour and
the level of spinal cord compression.
For the majority of patients who sought treatment of rehabilitation,
radiotherapy and chemotherapy at other institutions, after being evaluated
by us, no follow up data is available.
Although analysis of the reported results was difficult to interpret
because of the small number of patients, our study confirms previous
epidemiological reports.(5,25,26) SCSCT account for about 12.5% of our
practice in central nervous system tumours. Wenqing (26) found the same
percentage in a combined series of 2603 intraspinal tumours. The mean
age, at diagnosis, of these 150 patients was 32.5 years. Two patients
were less than 3 years of age.
Of the 150 patients, 78 were females in contrast to the Chinese series.(26)
Two different histological patterns became immediately apparent. Most
common benign tumours were neurilemmomas (20.6%) and meningiomas (12.6%).
Nittner (20) reported 111 cases of neurilemmomas in a series of 565
tumours (19.6%). In the Levy's series, this percentage was abour 25%
for each of them. 17
Meningiomas, uncommon in children and young adults (10,22) are believed
to account for about 25% in the western world (3,24) and 12.6 in China.5,26
The ratio neurilemmomas versus meningiomas was 1.6/1 in our study whereas
it was 3.8/1 in the Chinese series, 3.9/1 in Japanese literature (13)
and about 1/1 in the western world.
On the other hand, spinal epidural metastases represented 32% of the
total series, lower than those of 64.2% and 66% estimated respectively
by Lein (16) and Arseni.l The frequency of these secondary neoplasms
in the North hemisphere is in parallel with the high occurrence of carcinomatous
tumours in patients suffering from cancerous diseases.
Intramedullary spinal cord tumours are relatively rare, with perhaps
200 per year in the United States, (18) and accounting for about 2 to
4% of all central nervous system tumours. (19)
Most of them were ependymomas (8%) and astrocytomas (4.6%). Different
neurosurgeons, such as Cooper,(8) Fisher (12) and Lein (16) reported
this similar predominance of ependymomas. Epidermoid and dermoid cysts,
teratomas and lipomas were estimated in our series at 8%. They are four
times less frequent in brain.(26)
Surgery is indicated in all patients with SCSCT. Post operatively, they
must benefit from an intensive rehabilitation programme.
In cases of intramedullary tumours, radical removal can usually be achieved
(8,12) in ependymomas and low grade astrocytomas. In cases of "intensive"
astrocytomas gross total removal is not possible and post operative
treatment is recommended. Chemotherapy could be used alone or combined
with radiotherapy.
The best results were obtained in the surgery of meningiomas and neurilemmomas,
even when severe neurological dysfunction had occurred pre operatively.
In Solero's series (23) complete meningioma removal was achieved in
168 patients (97%). As for neurinomas, complete removal could be achieved
without persisting neurological deficit even if the affected nerve root
is resected.(21)
The surgical excision of the spinal and epidural metastases was usually
partial and palliative. For this reason radiotherapy and/or chemotherapy
have to be added to surgery: radiotherapy when there is a large infiltration
of the vertebra and in case of intensive pain. Chemotherapy based on
corticosteroids and chemoembolisation can produce "an effective pain
relief and a cytotoxic effect of antimitotic drugs delivered in situ".(9)
In conclusion, SCSCT are uncommon in childhood, remains rare in adults
compared with intracranial tumours. Their management outcome depends
on early diagnosis, pathological examination, resection which must be
radical. An intensive rehabilitation programme should always follow.
Acknowledgement
The authors thank Djamila Smati and Souhila Riahi for typing and preparing
the manuscript.
| 1. |
Arseni CN, Simonescu MD, Horwath L:
Tumours of the spine. A follow-up study of 350 patients with neurosurgical
considerations. Acta Psych., 16. Scand. 1959. 34: 398-410 |
| 2. |
Bouffet E, Amat D, Devaux Y, D2SUZIngeS
C: Chemotherapy for spinal cord astrocytoma. Med. Pediatr. Oncol:
1997, 29(6), 560-562 |
| 3. |
Challa WR, Markesbery WR: Meningiomas:
pathology. In Wilkins RH, Rengachary SS (eds). Neurosurg 1985, Vol.1.
McGraw Hill, New York, 613-622 |
| 4. |
Charcto JM: Lecons sur les maladies
du systeme nerveux central. 1880,2,88. Masson, Paris (Reported by
Paillas JE, Alliez B, Boudouresques G) |
| 5. |
Cheng MK: Spinal cord tumors in the
People's Republic of China: a statistical reviw - Neurosurg 1982,
10, 22-24 |
| 6. |
Ciappetta P, Salvati M, Capoccia G.,
et al: Spinal glioblastomas: Report of seven cases and review of
the literature: Neurosurg, 1991, Vol. 28, 2, 302306 |
| 7. |
Constantini S, Houten J, Miller DC,
Freed D, Ozek MM, Rorke LB, Allen JC, Epstein FJ: Intramedullary
spinal cord tumors in children under the age of 3 years. J. Neurosurg:
1996, 85(6). 1036-43 |
| 8. |
Cooper PR: Outcome after operative
treatment of intramedullary spinal cord tumors in adults: Intermediate
and long term results in 51 pateints. Neurosurg. 1989, 25,6, 855-859 |
| 9. |
Courtheoux P, Alachkar F, Casasco A.,
et al: Chemoembolisation of lumbar spine metastases. A preliminary
study. J.Neuroradiol. 1965, 12, 151163 |
| 10. |
Gushing H, Eisenhardt L: Meningiomas:
Their classification, Regional Behavior, Life History and Surgical
End Results. Springfield IL, Charles C Thomas: 1938, p.735 |
| 11. |
Fields WS: Brain tumors: morphological
aspects and classification. Brain pathology. 1993, 33, 251254 |
| 12. |
Fisher G, Brotchi J: Intramedullary
spinal cord tumors. Neurochirurgie 1994: 40, 1-108 |
| 13. |
Jones RD: Epidemiology of brain tumors
in man and their relationship with chemical agents. Food Chem Toxicol
1986: 24, 99-103 |
| 14. |
Kleihues P, Burger P, Scheithanter
BW: The new WHO classification of brain tumours. Brain Pathology.
1993, 3, 255-268 |
| 15. |
Kleihues P, Burger PC, Scheithauer
BW: Histological typing of tumors of the central nervous system.
In: WHO ed. International histological classification of tumors.
Springer-Verlaf, Berlin, 1993, 1-112 |
| 16. |
Lein HJ, Richter HP, Schaeer M: Extra
dural Spinal metastases. A retrospective study of 197 pateints.
Adv. Neurosurg. 1984, 12, 36-43 |
| 17. |
Levy WJ, Latchaw J. Hahn JF, et al:
Spinal neurofibromas: a report of 66 cases and a comparison with
meningiomas. Neurosurg. 1986, 18, 331-334 |
| 18. |
Malis Ll: Preface of intramedullary
spinal cord tumors. Neurochirurgie. Fisher G, Brotchi J. 1994. 40,
Suppl 1. |
| 19. |
McCormick PC, Stein BM: Intramedullary
tumors in adults. In: Stein BM, McCormick PC. Eds. Intradural spinal
surgery. Neurosurgery Clinics of North America. Vol. 1. Saunders
Philadelphia. 1990, 609-630 |
| 20. |
Nittner K: Spinal meningiomas, neurinomas
and neurofibromas and hourglass tumors in Vinken PJ, Bruyn BW (eds):Tumours
of the Spine and Spinal Cord, Part II: Handbook of Clinical Neurology.
Amsterdam, North Holland. 1976. Vol. 20, pp 177322 |
| 21. |
Schultheiss R, Gullota G: Resection
of relevant nerve roots in surgery of spinal neurinomas without
persisting neurological deficit. Acta Neurochir. 1993, 122 (1-2),
91-6 |
| 22. |
Sloof JL, Kernohan JW, MacCarty CS:
Primary intramedullary tumours of the Spinal Cord and Filum Terminate.
Philadelphia/London: WB Saunders. 1964, pp255 |
| 23. |
Solero CL, Fornari M, Giombini S, et
al: Spinal meningiomas: Review of 174 operated cases. Neurosurg.
1989, 25, 2, 153-160 |
| 24 |
Sundaresan N, Hugues JEO, Di Giacento
GV: Surgical approaches to primary and metastatic tumors of the
spine. In: Schmidek H, Sweet WH (eds). Operative neurosurgery. Grune
and Stratton, Orlando, Florida (USA). 1988, 2, 1525-1537 |
| 25. |
Touboul E, Khelif A, Guerin RA: Tumeurs
primitives due rachis: radiotherapie et chimiotherapie. Neurochirurgie.
1989, 35, 342-350 |
| 26. |
Wen Qing H, Shi Ju Z, Qing Sheng H,
Yuxia L, Qing Zhong X, Zi Jun I, Wen Cui Z: Statistical analysis
of central nervous system tumors in China. N. Neurosurg. 1982. 56,
555-564 |
| |
 |
|
