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British Journal of Neurosurge y (1 993) 7, 557-559 SHORT REPORT Spinal subarachnoid haemorrhage from an “ancient” schwannoma of the cervical spine BRIDGET MILLS, PAUL V. MARKS & JOHANNA M. NIXON* Departments of Neurosurge y and *Pathology, Auckland Hospital, Park Road, Auckland 1, New Zealand Abstract A case of spinal subarachnoid haemorrhage secondary to degenerative changes in a cervical schwannoma is presented. The problems associated with diagnosis as well as the possible mechanisms resulting in haemorrhage from spinal tumours are reviewed. Key words: Spinal subarachnoid haemorrhage, “ancienr schwannoma, spinal tumour. Introduction Spinal subarachnoid haemorrhage (SAH) is rare and most frequently seen after bleeding from an arteriovenous malformation.’ Haem- orrhage into the spinal subarachnoid space can, on rare occasions, arise from an intradural turnour.’ We report a case of spinal SAH arising from an “ancient” or degenerate schwannoma which arose in the lower cervical region. Case report A 53-year-old man presented to his local hos- pital with a 3-day history of right-sided subscapular pain of sudden onset, which he described as being the worst pain that he had ever experienced. The pain radiated down his right arm and into the back of his head. Over the next 12 h his condition deteriorated so that he was unable to walk and he became incon- tinent of urine. He was then transferred to the Regional Neurosurgical Unit at Auckland Hospital. Examination revealed marked neck stiffness which was associated with a spastic tetra- paresis. A sensory level was present at C7 and his bladder was palpable at the level of the umbilicus. The CSF was heavily blood stained and had an elevated protein content (greater than 5 gA). A diagnosis of spinal SAH was made and a C T myelogram performed. This showed an almost complete block of contrast at the C7-T1 level due to an intradural extra- medullary lesion causing displacement of the cord posteriorly. Laminectomy was carried out between C6 and T1 and the dura was noted to be tense and discoloured. Upon opening the dura, the cord was found to be bowed over a bluish mass anterior to it. The lesion was dissected from the cord and was found to contain fresh blood as well as organized haematoma. Total removal was achieved and the cord returned to its normal position. Twenty-four hours later, there was a dramatic increase in the power of his legs and 5 days after surgery he was able to walk. His 557 Br J Neurosurg Downloaded from informahealthcare.com by University of North Carolina on 11/20/14 For personal use only.

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Page 1: Spinal subarachnoid haemorrhage from an “ancient” schwannoma of the cervical spine

British Journal of Neurosurge y (1 993) 7, 557-559

SHORT REPORT

Spinal subarachnoid haemorrhage from an “ancient” schwannoma of the cervical spine

BRIDGET MILLS, PAUL V. MARKS & JOHANNA M. NIXON*

Departments of Neurosurge y and *Pathology, Auckland Hospital, Park Road, Auckland 1, New Zealand

Abstract A case of spinal subarachnoid haemorrhage secondary to degenerative changes in a cervical schwannoma is presented. The problems associated with diagnosis as well as the possible mechanisms resulting in haemorrhage from spinal tumours are reviewed.

Key words: Spinal subarachnoid haemorrhage, “ancienr ” schwannoma, spinal tumour.

Introduction

Spinal subarachnoid haemorrhage (SAH) is rare and most frequently seen after bleeding from an arteriovenous malformation.’ Haem- orrhage into the spinal subarachnoid space can, on rare occasions, arise from an intradural turnour.’ We report a case of spinal SAH arising from an “ancient” or degenerate schwannoma which arose in the lower cervical region.

Case report

A 53-year-old man presented to his local hos- pital with a 3-day history of right-sided subscapular pain of sudden onset, which he described as being the worst pain that he had ever experienced. The pain radiated down his right arm and into the back of his head. Over the next 12 h his condition deteriorated so that he was unable to walk and he became incon- tinent of urine. He was then transferred to the Regional Neurosurgical Unit at Auckland Hospital.

Examination revealed marked neck stiffness which was associated with a spastic tetra- paresis. A sensory level was present at C7 and his bladder was palpable at the level of the umbilicus.

The CSF was heavily blood stained and had an elevated protein content (greater than 5 gA). A diagnosis of spinal SAH was made and a C T myelogram performed. This showed an almost complete block of contrast at the C7-T1 level due to an intradural extra- medullary lesion causing displacement of the cord posteriorly.

Laminectomy was carried out between C6 and T 1 and the dura was noted to be tense and discoloured. Upon opening the dura, the cord was found to be bowed over a bluish mass anterior to it. The lesion was dissected from the cord and was found to contain fresh blood as well as organized haematoma. Total removal was achieved and the cord returned to its normal position.

Twenty-four hours later, there was a dramatic increase in the power of his legs and 5 days after surgery he was able to walk. His

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Page 2: Spinal subarachnoid haemorrhage from an “ancient” schwannoma of the cervical spine

558 B. Mills, P. V. Marks &J. M. Nixon

FIG. 1. Dilated blood vessels and haernorrhage with intervening cellular tumour tissue (H&E, x 108).

continence returned and he was discharged home after 8 days. When reviewed 6 months later, he had made a full recovery and neuro- logical examination was entirely normal.

Histological examination of the surgical specimen showed it to consist predominantly of haemorrhage and dilated thin-walled vascu- lar channels (Fig. 1). These were separated by spindle-cell tumour exhibiting intense S- 100 positivity and degenerative nuclear atypia without mitoses, typical of an “ancient” schwannoma (Fig. 2).

Discussion

The first clear clinical description of sponta- neous spinal SAH was given by Michon in 1928.3 He likened it to being stabbed in the spine (le coup de poignard rachidien).

Spinal SAH accounts for less than 1% of all cases of subarachnoid haemorrhage.’ Most cases are associated with bleeding from a spinal arteriovenous malformation,’ but it is well recognized that spinal neoplasms, trauma, connective tissue disorders and haemorrhagic diatheses may result in spinal SAH.”‘

The onset of symptoms is typically sudden with vertebral pain referred to the site of bleeding. If the haemorrhage arises in the cer- vical region, cranial radiation of pain may make differentiation of spinal SAH from in- tracranial causes extremely difficult on clinical grounds. Thus, the failure to identify an in- tracranial source of bleeding should alert the

clinician in such circumstances to consider the possibility of spinal SAH, especially if radicu- lar pain precedes the onset of headache and there is no loss of consciousness. If this diag- nosis is suspected, CT myelography with subsequent angiography, or MRI may demon- strate the underlying path010gy.~3~

The term “ancient” schwannoma has been applied to tumours showing pronounced degenerative changes,‘ frequently without highly differentiated Antoni A areas. These changes include cyst formation, haemorrhage and calcification, and may be so marked as to obliterate much of the underlying tumour tissue. Nuclear atypia is often present and may raise concern of malignancy, but in the absence of mitotic activity is attributed to cellular degeneration. Bizarre vascular forma- tions including cavernous and telangiectatic channels with hyalinized walls and peri- vascular deposits of haemosiderin, may be prominent.’,’

The mechanism of haemorrhage in such tumours is not fully understood, but two theories have been advanced. First, it is postu- lated that the ectatic hyalinized vessels may undergo spontaneous thrombosis. This is followed by distal tumour necrosis compli- cated by haemorrhage. Alternatively, vascular tumours may be obliterated by endothelial proliferation. Recanalization by meningeal vessels may occur with resultant haemorrhage into the necrotic t u m o ~ r . ~

The second theory invokes a mechanical

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Page 3: Spinal subarachnoid haemorrhage from an “ancient” schwannoma of the cervical spine

Spinal subarachnoid haemorrhage 559

FIG. 2. Enlarged and hyperchromatic nuclei without mitotic activity (H&E, x 108).

cause.',") It postulates that haemorrhage into the subarachnoid space occurs when traction is exerted on vascular attachments to nerve roots, because the line of action of the force lies along the spinal axis. Such tractional force occurs in areas of greater mobility and might explain why the onset of symptoms in 50% of cases appears related to exertion. This theory is thought to account for the observation that the majority of spinal tumours producing SAH are situated in the regon of the conus medullaris and cauda equina.

A review of 50 cases of spinal SAH secondary to tumour showed that only two cases were attributable to lesions arising in the cervical region. ' I

Address for correspondence: Mr P. Marks, Leeds General Infirmary, Great George Street, Leeds LSI 3EX, UK.

References 1 Wisoff HS. Spontaneous intraspinal haemorrhage.

In: Wilkins RH, Rengachary SS, eds. Neurosurgery

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