Surg Neurol 1985;24:227-30
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Intracerebellar Hemorrhage Due to Cerebellar Hemangioblastoma Akira Matsumura, Eiki Kobayashi,
M.D.,
Yutaka Maki, M.D.,
Katsuharu
Munekata,
M.D.,
and
M.D.
Department of Neurosurgery, Institute of Clinical Medicine, University of Tsukuba, and Tsukuba Memorial Hospital, Ibaraki, Japan
Matsumura A, Maki Y, Munekata K, Kobayashi E. Intracerebellar hemorrhage due to cerebellar hemangioblastoma. Surg Neurol 1985;24:227-30. A rare case of a bleeding from a cerebellar hemangioblastoma is described. The tumor was diagnosed preoperatively with a computed tomography scan and a vertebral angiogram. The characteristic computed tomography scan findings and probable mechanism of bleeding are discussed. KEY WORDS: Brain neoplasm; Cerebellum; Hemangioblastoma; Computed tomography; Cerebral hemorrhage; Spontaneous cerebellar hemorrhage
Intracranial h e m o r r h a g e from a brain t u m o r is known to occur [ 1 , 3 , 5 - 7 , 1 3 , 1 6 , 1 8 - 2 1 ] and shows a sudden clinical deterioration such as an apoplexy. H o w e v e r , intracerebellar hemorrhage from a hemangioblastoma is not c o m m o n . In a survey o f many cases of hemangioblastoma, we found only one case o f intracranial supratentorial hemorrhage caused by this t u m o r [ 1]. W e report a rare case o f a hemangioblastoma with the sudden onset of a cerebellar hemorrhage which was diagnosed preoperatively. Case Report A 54-year-old man was admitted to our hospital with a sudden onset o f severe nuchal pain, vomiting, and progressive loss o f consciousness on July 9, 1982. H e had no past history o f hypertension or other chronic medical problems. On admission he was semicomatose, his blood pressure was 180/100 m m H g , pulse rate 110 beats/min, and respiratory rate 18/min, and the patient showed mild left hemiparesis with a conjugate deviation of eye to the
right. H e had an anisocoria (left greater than right) but his light reflexes remained reactive. A computed tomography (Hitachi CT-W3) examination without contrast e n h a n c e m e n t showed a high-density mass lesion (4 x 4 x 3 cm in size) in the right cerebellar hemisphere (Figure 1A). T h e fourth ventricle was collapsed and not visible. Just beneath the lateral side of this high-density mass, a small patchy isodensity mass was identified. The contrast-enhanced computed tomography scan demonstrated a mass with marked enhancement and irregular margins (Figure 1B). We suspected organic lesions such as arteriovenous malformations, aneurysm, or brain tumors, and we made a retrograde brachial angiogram. The anteroposterior view demonstrated a small tumor stain in the lateral branch o f the right superior cerebellar artery and an avascular area below this tumor stain (Figure 2A). T h e lateral view showed the same t u m o r stain with a faint margin to the front, but no arteriovenous shunting was demonstrated (Figure 2B). From these findings the lesion was suspected to be a cerebellar hemangioblastoma associated with an intraparenchymal hemorrhage extending downwards. An emergent suboccipital craniotomy was p e r f o r m e d and total evacuation of the h e m a t o m a and total removal of the reddish mural nodule were carried out. T h e patient's postoperative course was uneventful. O n August 19, 1982, the patient was discharged on his feet, and he resumed his normal daily activities. At the time of a recent postoperative visit in N o v e m b e r 1984, he was enjoying healthy life as retired businessman although he could work full time. T h e histologic examination using hematoxylin and eosin stain revealed large numbers o f small capillary vessels separated by stroma cells (Figure 3). T h e pathologic diagnosis of a capillary hemangioblastoma was confirmed. Discussion
Yutaka Maki, M.D., Department of Neurosurgery, Institute of Clinical Medicine, University of Tsukuba, Tennodai, Sakura-mura, Niihari-gun, Ibaraki-ken 305, Japan. Address reprint requests to:
© 1985 by ElsevierSciencePublishingCo., Inc.
Hemangioblastoma is a relatively u n c o m m o n benign tum o r will or without cyst formation. T h e clinical course 0090-3019/85/$3.30
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B
Figure 1. (A) Computed tomography scan without contrast enhancement showing a massive cerebellar hemorrhage and an isodense tumor mass shadow (arrow). (B) Contrast-enhanced computed tomography scan demonstrating enhanced tumor mass (arrow). is usually slowly progressive. Although microscopic hemorrhages from hemangioblastoma have been reported [15], the sudden onset of a massive hemorrhage Figure 2. Retrograde brachial angiography demonstrating a tumor stain in the right cerebellar hemisphere. (A) Anteroposterior view. (B) Lateral
from a hemangioblastoma has been reported very rarely. A review of the literature revealed only one case report of intracranial supratentorial hemorrhage reported by Adegbite et al [ 1]. The other cases reported by Djindian et al [2] and Kormos et al [9] were subarachnoid hemorrhages from spinal hemangioblastomas. In other large series of tumoral bleeding, hemorrhage from a hemangioblastoma was not reported [19,20] except a case reported by Fujita and Matsumoto [3], in which the clinical features were not described. Massive intracerebral hemorrhages from the brain tumors occur more frequently with malignant tumors than with benign tumors [19]. The mechanism of a hemorrhage from a brain tumor has been proposed as follows: rupture of the thin fragile huge vessel [6,11,12,21], hemorrhagic infarct due to stenosis or occlusion of vessels by endothelial proliferation or tumor emboli [5,17], rupture of a vessel due to the direct invasion of the tumor cells [ 10,13 ], secondary vascular degeneration or necrosis due to radiation therapy or chemotherapy [3,7,10], or sudden structural changes of a vessel by the imbalance of intracranial pressure after ventriculoperitoneal shunt or external drainage [16,18]. In our case no predisposing factors such as radiotherapy, chemotherapy, or a surgical procedure existed. The tumor was highly vascular, but because it was a benign tumor, we could not see any vascular occlusion or direct tumor invasion of a vessel. Thin-walled capillary vessels were present and these might have been the cause of the
view.
A
B
Hemorrhage from Hemangioblastoma
tumoral bleeding but no definite bleeding source was found. C o m p u t e d tomography scan is the most valuable examination for the first step o f the differential diagnosis in cases o f secondary cerebellar hemorrhages; thus, in our case, the enhanced mural nodule was the clue leading us to the correct diagnosis. For the differential diagnosis o f hemangioblastomas such tumors as meningioma, astrocytoma, metastatic tumor, medulloblastoma, and arteriovenous malformations should be mentioned. Ganti et al [4] stated that the mural nodules of hemangioblastoma are usually seen near the pia, particularly, the tentorial surface, or the occipital surface o f the cerebellum and compared to cerebellar astrocytomas, hemangioblastomas are not calcified and tend to have a relatively small nodule associated with a large cyst. Because it is often difficult to differentiate these tumors by c o m p u t e d t o m o g r a p h y scan alone, other examinations such as vertebral angiography should be p e r f o r m e d for m o r e reliable preoperative diagnosis [8,14]. In cases of spontaneous cerebellar hemorrhage, one must be aware of a rare cause of h e m o r r h a g e as reported here. This patient did not have any evidence of hypertension and the c o m p u t e d tomography scan was essential in making the correct diagnosis. P r o m p t diagnosis
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Figure 3. Photomicrograph of the tumor demonstrating large numbers of small capillary vessels separated by stroma cells (Hematoxylin and eosin, x 200).
requires not only a plain computed tomography scan but also a contrast-enhanced computed tomography scan and angiography. T h e immediate lifesaving surgical evacuation of the h e m a t o m a and the total removal of the tumor can only be made after a careful preoperative diagnosis. References 1. Adegbite AB, Rozdilsky B, Varughese G. Supratentorial capillary hemangioblastoma presenting with fatal spontaneous intracerebral hemorrhage. Neurosurgery 1983;12:327-30. 2. Djindian M, Djindian R, Houdart R, Hurth M. Subarachnoid hemorrhage due to intraspinal tumors. Surg Neurol 1978;9:223-9. 3. Fujita K, Matsumoto S. Intracerebral hemorrhage in brain tumors. Neurol Surg 1980;8:929-34. 4. Ganti SR, Silver AJ, Hilal SK, Mawad ME, Sane P. Computed tomography of cerebellar hemangioblastoma. J Comput Tomogr 1982;6:912-9. 5. Glas B, Abott KH. Subarachnoid hemorrhage consequent to intracranial tumors. Arch Neurol Psychiatry 1955;73:369-79. 6. Globus JH, Sapirstein M. Massive hemorrhage into brain tumor. Its significance and probable relationship to rapidly fatal termination and antecedent trauma. JAMA 1942;120:348-52.
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7. Hatanaka M, Sobata E, Nakaoka T, Saito K. Intracerebral hemorrhage due to treated brain tumor. Neurol Surg 1982;10:1105-9. 8. Kitaoka K, Ito T, Tashiro K. Vertebral angiography of cerebellar hemangioblastoma. Neurol Surg 1981;9:37-49. 9. Kormos RL, Tucker WS, Blabao JM, Gladston RM, Bass AG. Subarachnoid hemorrhage due to spinal hemangioblastoma: case report. Neurosurgery 1980;6:657-60. 10. Kothbauer P, Jellinger K, Flament H. Primary brain tumors presenting as spontaneous intracerebral hemorrhage. Acta Neurochir (Wien) 1979;49:35-45. 11. Mandybur TI. Intracranial hemorrhage caused by metastatic tumors. Neurosurgery 1977;27:650-5. 12. Modesti LM, Binet EF, Collins GH. Meningiomas causing spontaneous intracranial hematomas. J Neurosurg 1976;45:437-41. 13. Nakagawa Y, Tashiro K, Isu T, Tsuru M. Occlusion of cerebral artery due to metastasis of chroioepithelioma. J Neurosurg 1979;51:247-50. 14. Nakao T, Kikuchi H, Matsumoto A. The value of computed tomography in the differential diagnosis of cerebellar hemangioblastomas. Neurol Surg 1981;9:277-83.
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15. Rubinstein LJ. Tumors of the central nervous system In: Atlas of Tumors. Pathology, 2nd series, fasicle 6. Washington, D.C: Armed Forces Institute of Nervous Pathology, 1970:235-40. 16. Vaquero J, Cabezudo JM, Desola RG, Nombela L. Intratumoral hemorrhage in posterior fossa tumoral after ventricular drainage. Report of two cases. J Neurosurg 1981;54:406-8 17. Vick NA. Brain tumor microvasculature In: Brain metastasis. The Hague-Boston-London: Martinus Nijihoff Publishers, 1980: 151-3. 18. Waga S, Shimizu T, Shimosaka S, Tochio H. lntratumoral hemorrhage after a ventriculoperitoneal shunting procedure. Neurosurgery 1981;9:249-52. 19. Wakai S, Yamakawa K, Manaka S, Takakura K. Spontaneous intracranial hemorrhage caused by brain tumor: its incidence and clinical significance. J Neurosurg 1982;10:437-44. 20. Zimmerman RA, Bilaniuk LT. Computed tomography of acute intratumoral hemorrhage. Radiology 1980;135:355-9. 21. Ziilch KJ. Neuropathology of intracranial hemorrhage. Prog Brain Res 1968;30:151-65.