FALLBERICHTE - CASE REPORTS Neurochirurgia 22 (1979) 189-193 © Georg Thieme Verlag Stuttgart

Meningioma of the Foramen Magnum Presenting as Subarachnoid Haemorrhage and Cerebellar Haematoma J. Hamer

Summary An unusual case of a posterior fossa meningioma which caused subarachnoid haemorrhage (SAH) and cerebellar haematoma is presented. The possible causes of tumoral bleeding and the surprising clinical course are discussed in the context of the few similar cases reported in the literature. The importance of cerebral CT scanning in SAH without angiographic demonstration of an aneurysm or angioma is emphasized. Key-Words: Subarachnoid haemorrhage - Intracranial haematoma - Meningioma

Zusammenfassung Meningeom des Foramen magnum mit Subarachnoidalblutung und intrazerebellärem Hämatom Es wird über einen ungewöhnlichen Fall eines Meningioms der hinteren Schädelgrube berichtet, das zu einer Subarachnoidalblutung und zu einem intracerebellären Hämatom geführt hatte. Die möglichen Ursachen der Tumorblutung und der überraschende klinische Verlauf werden im Zusammenhang mit den wenigen ähnlichen bislang publizierten Fällen diskutiert. Der Wert der axialen Schädelkomputertomographie bei Subarachnoidalblutung ohne angiographischen Aneurysmaoder Angiomnachweis wird hervorgehoben.

Introduction It is well known that brain tumours may occasionally lead to intracranial haemorrhage. Intracerebral bleeding has been found particularly in melanomas and metastatic brain neoplasms (24), but has also been observed

in gliomas (14, 20), plexus papillomas (1, 6, 15), angioblastomas (11) and pituitary adenomas (9, 12, 22). Meningiomas, however, seem to be a very unusual cause of subarachnoid haemorrhage and cerebral haematoma. No more than 27 cases of meningiomas presenting as spontaneous intracranial haemorrhage have been reported up to now (2,3,4, 8, 10,17,19,23, 25, 26, 27, 28, 29). Among these rare cases were only two patients with meningiomas of the posterior fossa (cerebellopontine angle) which became clinically manifest by subarachnoid haemorrhage (28). To the author's knowledge, the present case is the first report on an infratentorial meningioma which produced both subarachnoid bleeding and intraparenchymal cerebellar haematoma. Moreover, the preoperative clinical course differed in some respects from previously published cases.

Case report The 36-year-old patient fell ill suddenly in May 1977 with severe frontal headache, vomiting and stiff neck. He was admitted to a local municipal neurology department in a conscious state. There were no pathological clinical findings, apart from meningism. Lumbar puncture revealed bloody cerebrospinal fluid which was xanthochromic after centrifuging. Bilateral carotid angiography was performed (the corresponding angiograms were later presented to the neurosurgeon), but no pathological process was found. The patient recovered quickly and was discharged

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Department of Neurosurgery, University of Heidelberg, Heidelberg, Federal Republic of Germany

/• Hamer

Fig. 1: Technetium brain scan showing a lesion (arrow) in the posterior fossa.

Fig. 2: CT scanning demonstating a hyperdense area in the lower left cerebellar hemisphere.

without complaints. However, in the following weeks and months he frequently felt nausea, and a mild ataxia slowly developed. Finally, ten days before the second neurological admission, he complained again of headache, vomiting, diplopia, vertigo and rapidly progressive disturbance of gait. Brain scanning with technetium pertechnetate (see Fig. 1) showed a lesion in the posterior fossa on the left side and the patient was then transferred to the Department of Neurosurgery of the University of Heidelberg.

sixth cranial nerve, a horizontal nystagmus to the left, dysmetria and dysdiadochokinesia on the left side and marked ataxia. Cerebral CT scanning (see Fig. 2) revealed a large hyperdense area in the left cerebellar hemisphere. This finding was interpreted as an intraparenchymal haematoma. This tentative diagnosis seemed to be supported by vertebral angiography (see Fig. 3) which showed a small vascular area suggestive of an angioma.

Operation Examination The patient was conscious, his blood pressure was with 140/90 mm Hg, within normal limits. However he had been under medical treatment for the last two years because of arterial hypertension. Neurological examination revealed a mild paresis of the

Suboccipital craniotomy and the exposure of the posterior fossa were performed with the patient in the sitting position. After opening the dura an extensive meningioma at the foramen magnum was found. The tumour had lifted upwards the basal parts of both cerebellar hemispheres and had grown into

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Fig. 3: Vertebral angiography. Arrows indicate at the lower cerebellopontine angle a vascular zone which suggested a small angioma or angioblastoma.

both cerebellopontine angles. Laminectomy down to C 3 had to be carried out in order to reach the caudal pole of the meningioma. In the lower part of the left cerebellar hemisphere, a cone-shaped tumour nodule had invaded the cerebellar tissue. A large haemorrhagic cavity with organized clots was opened after having excised this part of the meningioma. After complete removal of the haematoma, no angioma was detected in the wall of the cavity. Moreover, the two vertebra] arteries and their branches which were exposed during microsurgical resection of the tumour showed no sign of any aneurysm. Radical extirpation of the meningioma including the posterior cerebellar and cervical dura was accomplished. The dural defect was closed with fascia lata. The postoperative course was uneventful. The patient was discharged two weeks after operation. Diplopia and ataxia had almost completely disappeared. Histologically, the excised tumour was an endotheliomatous meningioma.

Discussion In their classical study on 313 meningiomas Gushing and Eisenbar dt (5) did not mention the association of this tumour and intracranial haemorrhage. Also the neuropathological literature (18, 30) is apparently unfamiliar with this problem, apart from the two cases reported by Russell and Rubinstein (23) in their text-book. The clinically documented cases show that bleeding meningiomas are almost exclusively located supratentorially and they are mainly parasagittal. This may be easily explained by the fact that meningiomas are much more frequent in the supratentorial space than in the posterior fossa. In most of the 27 cases, the neoplastically induced bleeding led to intracerebral haemorrhage (4, 8, 10, 17, 19, 23, 25), occasionally also to subdural haematoma (3, 17, 27). Five cases have been reported where besides subarachnoid haemorrhage a marked bleeding into the meningioma was found at operation (8, 17, 23, 28). Massive intracranial haemorrhage has also been observed in two

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Meningioma of the Foramen Magnum Presenting as Subarachnoid Haemorrhage

/. Hamer

recurrent meningiomas (4, 19). In one of these patients the initially benign meningioma later showed histologically a transition to malignancy. Goran et al. (8) among their five cases also had one patient with an anaplastic meningioma which had led to massive intracranial haemorrhage. It seems reasonable to ask whether there is a causal relationship between the histological type of the meningioma and the incidence of cerebral bleeding. Do markedly vascular meningiomas, and the angioblastic type in particular, favour subarachnoid haemorrhage and cerebral haematoma? Among the 27 cases in the relevant literature, the author found only two angioblastic meningiomas (17, 19). In four cases, the histology was characterized as angiomatous, and the rest represented - as far as detailed histology was reported - fibroblastic or endotheliomatous meningiomas of which one was diagnosed angiographically as a vascular lesion (4). In this connexion one should also emphasize that Pitkethly et al, (21) in their study on 81 angioblastic meningiomas did not list a single case of tumoral bleeding. Thus, the assumption of a specific histological tumour type predisposing to intracranial haemorrhage seems to be rather unlikely. Which factors finally cause subarachnoid haemorrhage in meningiomas remain unknown. Extrinsic contributary factors may be trauma and arterial hypertension. But only in two patients out of 27, was trauma the likely cause of neoplastic haemorrhage (3, 28). In one case report (17), it was explicitly stated that the patient was hypertensive prior to the neurological disease. Possibly, arterial hypertension may also have played a role in the present case. However, most of the authors assume that SAH and subsequent cerebral or subdural haematoma originate from spontaneous perforation or rupture of tumour vessels, although, in most cases the source of the bleeding could never be identified with certainty, either during operation or - in pertinent instances -

at autopsy. Another cause may be a haemorrhagic infarction due to compression of the brain and of cortical veins. Moreover, one may speculate in the present case whether the marked finger-like growth of the meningioma into the cerebellar hemisphere eroded small cortical vessels and thus led to subarachnoid and cerebral bleeding. In the author's case, the spatial intracranial compensation of both mass lesions which is reflected in the benign preoperative clinical course and in the initially slight neurological symptoms, is very surprising and contrasts with the suddenly severe and progressive neurological deterioration in almost all of the other published cases. Only Modesti et al. (17) observed another case of a bleeding endotheliomatous meningioma with remarkable cerebral compensation, where recurrent SAH occurred over ten years. Undoubtedly, among the cases with meningiomas presenting as intracranial haemorrhage the clinical course of these two patients is exceptional. In the present case, cerebral CT scan was of decisive diagnostic value. This clinical observation also indicates that CT scanning should be mandatory in all patients who suffered SAH and where angiography did not reveal an aneurysm or an angioma, irrespective of whether the patient recovered quickly from the intracranial bleeding or not. Nevertheless, meningioma as well as neurinoma (7, 16) must be regarded as a very rare cause of subarachnoid haemorrhage as already stated in the introduction. This is also borne out by the American Cooperative Study of intracranial aneurysms and subarachnoid haemorrhage: among 2092 patients with SAH, but without angiographic demonstration of an aneurysm or angioma, Locksley et al. (13) found a bleeding neoplasm in only 1-3 % of the cases. There were 12 metastases, 4 neoplasms suggesting a metastatic origin, and 12 primary cerebral tumours with only one parasagittal meningioma.

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1 Abbott, K. H., Z. H. Rollas, J. N. Meagher: Choroid plexus papilloma causing spontaneous subarachnoid hemorrhage. Report of case and review of literature, J. Neurosurg. 14 (1957) 566-570 2 Askenasy, H. M., A. Behmoavan: Subarachnoid hemorrhage in meningiomas of the lateral ventricle. Neurology (Minneap.) 10 (1969) 484-489 3 Bingas, B.t M. Meese: Subdurales Hämatom seltener Ätiologie (Fallmitteilung). Nervenarzt 37 (1966) 175-177 4 Budny, J. L., F. C. Glasauer, K. Sil: Rapid recurrence of meningioma causing intracerebral hemorrhage. Surg. Neurol. 8 (1977) 323-325 5 Cashing, H., L. Eisenhardt: Meningiomas. Their classification, regional behavior, life history and surgical end results. Springfield, 111. Charles C. Thomas 1938, 785 pp. 6 Ernsting, ].: Choroid plexus papilloma causing spontaneous subarachnoid hemorrhage. J. Neurol. Neurosurg. Psychiat. 18 (1955) 134-136 7 Gleeson, R. K., j . F. Butzer, O. D. Grin: Acoustic neurinoma presenting as subarachnoid hemorrhage. J. Neurosurg. 49 (1978) 602-604 8 Goran, A., V. ). Ciminello, R. G. Fisher, N. Y. Ponghkeepi: Hemorrhage into meningiomas. Arch. Neurol. 13 (1969) 65-69 9 Grumme, Th.: Hypophysen-Adenom mit apoplektiformer Blutung. Zbl. Neurochir. 32 (1971) 187-192 10 Gruskiewicz, J., Y. Doran, B. Gellei, E. Peyser: Massive intracerebral bleeding due to supratentorial meningioma. Neurochirurgia 12 (1969) 107-111 11 Hamer, J.: Angioblastoma of the posterior fossa in childhood. Mod. Probl. Paediat. Vol. 18 (1977) 27-29 12 Kirschbaum, J. D., B. M. Chapman: Subarachnoid hemorrhage secondary to a tumor of the hypophysis with acromegaly. Ann. Intern. Med. 29 (1948) 536-540 13 Locksley, H. B., A. L. Sahs, R. Sandler: Report on the Cooperative Study of Intracranial Aneurysms and Subarachnoid Hemorrhage. Section III. Subarachnoid hemorrhage unrelated to intracranial aneurysms and a-v-maliormations. A study of associated diseases and prognosis. J. Neurosurg. 24 (1966) 1034-1056 14 Manganiello, L. O. ].: Massive spontaneous hemorrhage in gliomas. A report of seven verified cases. J. Nerv. Dis. 110 (1949) 277-298 15 Maison, D. D., F, D. C. Crofton: Papilloma of the choroid plexus in childhood. J. Neurosurg. 17 (1960) 1002-1027 im Chirurgischen Zentrum der Unii

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16 McCoyd, K., K. D. Barron, R. J. Cassidy: Acoustic neurinoma presenting as subarachnoid hemorrhage. Case report. J. Neurosurg. 41 (1974) 391-393 17 Modesti, L. M., E. F. Binet, G. H. Collins: Meningiomas causing spontaneous intracranial hematomas. J. Neurosurg. 45 (1976) 437-441 18 Mutlu, N., R. G. Berry, B. J. Alpers: Massive cerebral hemorrhage. Clinical and pathological correlations. Arch, Neurol. 8 (1963) 644-661 19 Nakao, S., S. Sato, S. Ban: Massive intracerebral hemorrhage caused by angioblastic meningioma. Surg. Neurol. 7 (1977) 245-248 20 Oldberg, E.: Hemorrhage into gliomas. A review of 832 consecutive verified cases of glioma. Arch. Neurol. Psychiat. 30 (1933) 1061-1073 21 Pitkethly, D. T., J. M. Hardman, L. G. Kempe, K. M. Earle: Angioblastic meningiomas. Clinicopathological study of 81 cases. J. Neurosurg. 32 (1970) 539-544 22 Rovit, R. L., J. M. Fein: Pituitary apoplexy. A review and reappraisal. J. Neurosurg. 37 (1972) 280-288 23 Russell, D. D., L. J. Rubinstein: Pathology of tumours of the nervous system. London, Edward Arnold Ltd. (1959) 429 pp. 24 Scott, M.: Spontaneous intracerebral haematoma caused by cerebral neoplasms. Report of eight verified cases. J. Neurosurg. 42 (1975) 338-342 25 Skultety, F, M.: Meningioma simulating ruptured aneurysm. Case report. J. Neurosurg. 28 (1968) 380-382 26 Tönnis, W., W. Schiefer, W. Walter: Zur Differentialdiagnose intrakranieller Blutungen (unter Ausschluß der neuroradiologischen Methoden). Dtsch. Z. Nervenheilk. 176 (1957) 666-692 27 Walsh, J. W., K. R. Winston, T. Smith: Meningioma with subdural hematoma. Surg. Neurol. 8 (1977) 293295 28 Yasargil, M. G., S. C. So: Cerebellopontine angle meningioma presenting as subarachnoid haemorrhage. Surg. Neurol. 6 (1976) 3-6 29 Zimmerman, H. M.: Cited from Goran et al. (8), personal communication Aug. 28 (1963) 30 Ziilch, K. J.: Biologie und Pathologie der Hirngeschwülste. Handbuch der Neurochirurgie. Dritter Band. Pathologische Anatomie der raumbeengenden intrakraniellen Prozesse. Herausgegeben von H. Olivecrona und W. TÖnnis. Springer, Berlin-Göttingen-Heidelberg 1956, pp. 399-455 Professor Dr. med. Jürgen Hamer, Neurochirurgische Abteilung t Heidelberg, Im Neuenheimer Feld 110, D-6900 Heidelberg 1

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Meningioma of the foramen magnum presenting as subarachnoid haemorrhage and cerebellar haematoma.

FALLBERICHTE - CASE REPORTS Neurochirurgia 22 (1979) 189-193 © Georg Thieme Verlag Stuttgart Meningioma of the Foramen Magnum Presenting as Subarachn...
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