ACTA
NEUROCHIRURGICA
9 by Springer-Verlag 1981

Acta Neurochimrgica 58, 121--126 (1981)

1 Ludwig-Boltzmann-Institute of Clinical Neurology and Department of Neurology, Lainz-Hospital, Vienna, and ~ Department of Neurosurgery, Hospital
Rudolfstiftung, Vienna, Austria

Giant-Cell Glioblastoma of the Thoracic Cord
By

W. Grisold 1, G. Pernetzky 2, and K. Jellinger 1
With 3 Figures

Summary
A case of giant-cell glioblastoma occurring in the upper thoracic cord of
a male aged 76 without evidence of intracranial lesion is reported. Partial
tumour resection with decompression and radiotherapy did not improve the
patient's neurological deficit.

Keywords: Intramedullary tumour; giant-cell glioblastoma.
M a l i g n a n t a s t r o c y t o m a s (grades I I I and IV)10, ~ arising in the
spinal cord are rare 9, ~7-19. I n 1971 F o r t u n a and G i u f f r e 6 collected
22 cases f r o m the literature and a d d e d four personal observations.
A m o n g the available case reports, o n l y t w o 16, 17 were classified as
monstro-cellular glioblastoma. A f u r t h e r example o f p r i m a r y anaplastic glioma o f the thoracic cord w i t h the features o f giant-cell
glioblastoma is described in the present report.

Case Report
Case History. This 76-year-old white male had been well until 9 months
prior to hospital admission, when he noted slowly progressive weakness of the
legs with hypaesthesia below the sternum. Neurological examination showed
paraparesis of the legs and a sensory level at T 8. A myelogram revealed an
incomplete block at T3 (Fig. 1), and subsequent laminectomy from T2 to 4
showed the spinal cord segments T 2-T 4 swollen by a bluish, ill-delimited intramedullary tumour, which was partly resected by microsurgery.

0001-6268/81/0058/0121/$ 01.20

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Pathological Examination (N 275-80). All tumour specimens, altogether approximately 3ram in diameter, were embedded in paraffin, and stained by
routine methods. Histologically, the highly cellular and pleomorphic turnout
was mainly composed of large protoplasmic astrocytes with large numbers of giant
and multinucleated cells (Fig. 2). Their eosinophilic cytoplasm showed coarse
thick processes. The nuclei were huge with variable chromatin distribution,
and there were occasional cytoplasmic invaginations or "intranuclear inclusions"

Fig. I. Myelography showing incomplete block at T 3
(Fig. 3), while the number of mitotic figures was small. There were some small
areas of necrosis and obvious vascular proliferation. No intact spinal cord
tissue was seen. The diagnosis of giant-cell or monstrocellular glioblastoma
grade IV was made and confirmed by the Brain Turnout Reference Centre of
the German Association of Neuropathology.
Clinical Course. Postoperatively the condition of the patient was not changed.
He received irradiation by betatron (4950 cGy) over four weeks directed locally
at the tumour site. Despite the decompression and radiotherapy the patient's
symptoms progressed over the subsequent two months to the point where there
was almost complete motor and sensory deficit below the T 8 spinal cord level.
No control myelogram was performed, and spinal CT scan was not rewarding.
No systemic chemotherapy was given. At the time of writing there is slight
improvement in the patient's motor and sensory deficit, and there is no evidence
of intracerebral lesion.

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123

Discussion

While glioblastoma multiforme accounts for more than 50 per
cent of all primary intracranial gliomas 9, is, 10, it accounts for only
0.1 to 5.80/0 of all spinal cord tumours 9.11, 11, 18, 20, with an average
of 1 to 2 per cent. In 1971 Fortuna and Giuffr6 6 estimated the total
number of intramedullary glioblastomas at about 60, but the subject

Fig. 2. Histology of intramedullary biopsy specimen showing monstrocellular
glioblastoma with multinucleated giant cells and occasional mitoses. C. V. • 140
Fig. 3. Detail of tumour showing bizarre multinucleated giant celI with cytoplasmic invaginations. H. and E. • 540

is difficult to demarcate precisely because of the diversity of the
nomenclature used for high grade gliomas. Some additional instances
of intramedullary glioblastoma have been reported 13, 16. In a recent
autopsy series of 288 glioblastomas among 1,491 CNS tumours there
were 8 spinal medullary instances 19. Intramedullary glioblastoma
occurs at any age and not rarely affects younger subjects 6; however, among 900 spinal tumours in infancy and childhood there were
only 4 glioblastomas, or 0.50/0 11. They may affect all levels of the

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W. Grisold et al.:

cord with slight preference for the cervical and thoracic segments 6, 18
Their common clinical features are: a short history with occurrence
of pronounced neurological symptoms frequently progressing to complete motor and sensory deficit, often markedly increased CSF protein, negative plain X-rays of the spine but positive myelography,
and a poor prognosis, irrespective of the extent of surgical removal
and attempts at radiation and chemotherapy ~, 16
The histological appearance of intramedullary glioblastoma corresponds to that of the glioblastoma described in the cerebral
hemispheres, but the monstrocellular variant appears to be rare in the
spinal cord. Recent ultrastructural and immunocytochemical studies
have supported the astrocytic origin of this type of tumour a, 7, which
should be called a giant-cell glioblastoma. The irregular and most
grotesque multinucleated giant cells, which were shown to have a
slow rate of proliferation 8, may occur spontaneously or as a result
of some degeneration showing less GFAP (glial fibrillary acidic
protein) than well differentiated glial cells, suggesting an inverse
relationship between GFAP content and the degree of malignancy a
The morphogenesis of intramedullary glioblastoma is unknown. While
dissemination from primary intracranial glioblastoma 23 can be excluded in most reported cases of intramedullary glioblastoma, it has
also been argued that it may represent a spinal manifestation of
multifocal gliomas ~. The overall incidence of multifocal glioblastoma
ranges from 2.5 to 6 per cent 9, 18, and their occurrence in both the
cerebral and cerebellar hemispheres is well established 9, is, 21. Multiple
gliomas have been observed in neurofibromatosis 12. Some intramedullary glioblastomas, mainly in children, were associated with
other intraspinal tumours 6 or with intramedullary cavities, syringomyelia, and other malformations of the spine and cord 2, ~, 22. While
invasion of the meninges and dissemination into the cerebral CSF
pathways is rarely seen in isomorphic gliomas of the spinal cord ~, 14,
15, 20, 24, intramedullary glioblastoma, like its cerebral counterpart,
often spreads into the CSF pathways with occasional intracranial
seeding 1, 4, 6, is, 20 In our patient, there was no evidence of intracranial lesion at the time of writing. While recent results of multimodality treatment of malignant cerebral gliomas give some encouraging results, the prognosis of intramedullary glioblastoma is
extremely poor, and apparently very little experience of the effects
of irradiation and chemotherapy of these neoplasms is available.
Acknowledgement
Revision of the histological slides by the Brain Turnout Reference Center
of the German Association of Neuropathology (Prof. Dr. P. Kleihues, Freiburg/B.,
Federal Republic of Germany) is acknowledged.

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125

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Authors' address: Prof. Dr. K. Jellinger, Ludwig Boltzmann-Institut fiir Klinische Neurobiologie, Lainz Hospital, Wolkersbergenstrasse 1, A-1130 Wien, Austria.