SI92
Tumours of the eNS - Miscellaneous
tomas of the identical twins was compared with their constitutional DNA by DNA sequencing of the complete VHL coding region. However, neither allele reduction nor allelic losses(LOH) were foundfor the VHL gene as well as for various other tumorsuppressor genes (p53, BACA1, BACA2, DCC, MCC and AT)and no VHL mutations were found in constitutional DNAof bothtwin sistersand the 2 descendants as well as in tumor DNA of all 5 removed hemangioblastomas. Accordingly, a germline mutationof presentlyunknown genetic location being unrelated to the coding sequence of VHL gene has to be considered. Mutations may occur within VHL gene regulatory elements including promoter and untranslated regions as well as within introns. Also, it is unclear whether the entire VHL coding sequence has been identified yet or to what extent other presently unidentified tumor suppressor genes may be involved in tumorigenesis of VHL disease. Accordingly, in certain VHL families the presymptomatic identification of affected individualsmay be most difficultrequiring close clinical surveillance.
10 -22-325 1 Clinicopathological study of 26 autopsy cases with intracranial malignant lymphomas KiyoshiOnda 1 , Ayuichi Tanaka 1 , Koichi Wakabayashi 2 , Toshiro Kuman ishi 3 , Hitoshi Takahashi 2 . ' Department of Neurosurgery. Brain Research Institute, NiigataUniversity, Niigata, Japan, 2 Departmentof Pathology. Brain Research Institute, Niigata University, Niigata, Japan, 3 Department of Molecular Neuropathology. Brain ResearchInstitute, Niigata University, Niigat8, Japan Introduction: Weclinicopathologically analysedautopsy caseswithintracranial malignant lymphomas for better understanding of the disease. Methods: Between 1964 and t995, 26 patients with intracranial malignant lymphomas were autopsied. Whole-body or intracranial examination was performed in 15 and 11 cases, respectively. None of them had any signs of immunodeficiency. Seventeen were male and nine were female. The age of onset varied between 11 and 71 years. Median survival was 6 months (range t to 140 months). In 17 cases, both biopsy and autopsy materials were examined. The lymphomas were classified according to the Working Formulation and immunophenotyped with L26 and UCHL1. Tumoral spreadin the CNS was examined microscopically in each case. Results: Histological examination determined subtypes of 15 diffuselarge, 7 diffusesmallcleaved, 3 diffusesmall mixedand 1 diffuselargeimmunoblastic. All were L26-positive UCHLl-negalive B cell lymphomas. Aesidual or recurrent lymphomas were found in 20 casesat autopsy. The majority of them, especially in recurrent cases, showed bilateral, supra- ad infra-tentorial tumoral spread with local meningeal invasion. Two cases had diffuse massive subarachnoid proliferation of tumor cells. On the other hand, in the remaining 6 cases, only a few lymphoma cells were detected in the autopsied brains because of radiation-based treatment. Parenchymal lesions, probablyischemic in nature, were occasionally observed in areas with tumor infiltration in non-irradrated cases. Radiation therapycaused necrosis of tumor-burdened areas and degeneration of white mailer. Twocases had systemic spreadof the tumorat autopsy. Discuss ion and Conclusions: This study showed that intracranial lymphomas spread widely in the brain even in cases of short clinical course. In recurrent cases, diffusebilateralbrain-parenchymal involvement appears common. It is suggested that ischemic lesions may happen in areas with tumor infiltration. Though only a few tumor cells were seen in several cases, brain injury causedby radiation therapy was evident.
I0-22-3261
Primary CNS lymphoma as focal tumor, treated primarily with resection-decompression technique. Report of two consecutive immunocompetent patients
GustavoA. Zomosa, Department of Neurology-Neurosurgery, Clinical Hospital University of Chile, Santiago, Chile PrimaryCentral Nervous System Lymphoma (PCNSL) was considered an unfrequent eNS primary tumor, but recently it has been reported with increased frequency not only in AIDS panems, but also in immunocompetent ones. In our department two immunocompetent patients wereadmitted with symptoms of in· creased intracranial pressure (ICP) and CAT Scansshowedtocal intracerebral tumorswith mass effectand contrastenhancement. They wereoperated on wilh radical resection-decompression technique and biopsies specimen revealed as T-eeil lymphoma in one case and B-cenin the other. Then they weretreated with quimiotherapy (metrothexate) with leucovorin rescuefollowing the De Angelis protocol. Later on, conventional radiation therapy was performed. There was a good clinicaland radiological response. Thevalueof the resectiondecompression technique hasnotbeenmentioned in the literature. We believethat specially in focal tumors like in this presentation it allows to relieve ICP and so when the patient is in a better clinical condition that perform quimiotherapyand in some selectedcases radiation therapy.
Thursday. 10 July 1997 I 0-22-32 7 1 Central neurocytoma: The role of radiation therapy and long-term outcome S.H. Paek ' , D.G. Kim I ,I.H. Kim 2 , J.G. Chi 3 • H'w. Jung I, D.H. Han' , K.S. Choi I , B.K. ChoI . I Department of Neurosurgery, 2 Departmentof Radiation Oncology. 3 Departmentof Pathology. Seoul National University Collegeof Medicine, Seoul, Korea Introduction: To clarify the beneficial effect of radiation therapy on long-term outcome, the authorsretrospectively analyzed 15cases of centralneurocytoma. Methods: Fifteen patients who were diagnosed as cenlral neurocytoma at Seoul National University between 1982 and 1995 were reviewed. The clinical records and follow-up images were reviewed and the Kamofski performance scale (KPS) was assessed to the last follow-up: the duration of follow-up was from 18 to 168 months (mean, 52). Results: Grosstotal resection (GTA) of the tumor was performed in seven patients, two of whom received fractionated radiation therapy (AT). Subtotal removal (STR) ot the tumor was performed in nine patients, five of whom received this same Iherapy. In two of five patients who did not receive AT after GTA, the tumorrecurred 8 and 21 monthsafter surgery, respectively; in contrast, recurrence was not detected in two patientswho received fractionated RT after gross total resection. In all five patients who received fractionated RT after STR, the tumor shrank (n 3) or disappeared (n 2) during the postsurgical follow-up period of between 27 and lt3 months. In three patients who did not receive ATafter STA, no changewas found. At the last follow-up, KPS of all the patients was over 90. except one who neededassistance because of delayed complication of radiation. Conclusions: It is suggested that even if the biological behavior of central neurocytoma is very benign, RT is beneficial for tumor control. The routine use of AT for a residual tumor is not mandatory, however, because radiation can cause delayed complications and the clinical course of the patients with a subtotally resected tumor is extremely benign.
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I0-22-3281 intracranial Clinical results following surgical treatment of arachnoid cysts H. Bassiouni ", L. Mayfrank 2, H. Bertalanffy 2 , W. Kueker2, M. Korinth 2, J.M. Gilsbach 2 . 1 Department of Neurosurgery. University of Essen, 45122 Essen, Germany. 2 Department of Neurosurgery, Technical University RWTH, 52074 Aachen, Germany, 3 Department of Neuroradiology. Technical University RWTH, 52074 Aachen, Germany Introduction: The indication for surgical treatment of intracranial arachnoid cysts is still a matterof debate with regard to clinical criteria and the operative method. To determine the optimum management of this pathological condition, we reevaluated our cases of the past 7 years. Methods: Between August 1989 and March 1996 we observed 33 patients (equal sex distribution, mean age 30.5, ranging from 1.5 to 67 years) with intracranial arachnoid cysts. The main presenting symptoms were headache, blurring 01 vision and epileplic fits. The cysts were located in the middle cranial fossa (23), over the cerebral convexity (3), in the cerebelloponline angle (2), the pineal (1), the suprasellar (2) and the cisternamagna region (2). Whenever possible the cysts were fenestrated to the basal cisterns, ventricles or subarachnoid spaces. An additional silicon catheter connecting the cisternal and cystic compartments was placed in t7 patients with temporal cysts. Clinical and radiological follow-up (CT or MRI) was available in 29 patients ranging from 1 to 8 years. Results: Surgery and early recovery was uneventful except for 3 closed subgaleal CSF accumulations requiring surgical repair in one case. No new or worsened neurological deficits occurred. All patients recovered completely with remission or at least substantial amelioration of preoperative complaints and signs at follow-up, There were 3 recurrencies in 2 patients with simple fenestration. No major morbidity was encountered due to insertion of a cystocistemal catheter. Conclusion: The indication for surgicaltreatment should be guided by clinical criteria in concert with a space-occupying lesion in neuroimaging. Simple fenestration of the cyst is cHeclive, but may be followed by recurrence. The insertion of a cystocisternal catheter in temporal cysts may decrease the recurrence rate with minimal additional morbidity.
I0-22-329 /lntraoperative measurement of tumor and peripheral cerebral blood flow with laser-Doppler flowmetry Chang Rongwei, Xu Baode. Chang Hongjun. Department of Neurosurgery, Jinan GeneralMilitary Hospital, Jinan, China PR (250031) Laser-Dopplerflowmetry has been used intraoperatively to measure blood flow of tumorand peripheral brain tissuein 18 patients, including 6 caseswith meningioma. 6 glioma and 6 acoustic neurinoma. The blood flow data of meningioma was 845 ± 88 (mv);glioma 680 ± 67 (mv); acoustic neurinoma 315 ± 46 (rnv),
Cerebrovascular Disorders - Aneurysms and Vasospasm
Thursday, 10 July 1997 the differences were significant (P < 0.01). The blood flow in peripheral brain tissue around meningioma was increased after tumor resection. decreased in glioma and not changed in acoustic neurioma. The significance of hemodynamic change in various tumors and their peripheral brain tissue will be discussed.
I0-22-330 I Phenytoin or sodium valproate for prophylaxis of postoperative convulsions: A randomised comparison
Wai S. Poon, Yung Chan, Richard L.C. Kay. 1 Neurosurgical Unit, Department
of Surgery, The ChineseUniversityof Hong Kong, Shatin,New Territories, Hong Kong, 2 Neurosurgical Unit Departmentof Medicine, Princeof Wales Hospital, The Chinese University of Hong Kong, Shatin, New Territories. Hong Kong 182 patients awaiting elective craniotomy and excision of brain tumours or arteriovenous malformations (AVM) were randomised to either phenytoin or sodium valproate for prophylaxis of postoperative convulsion. The anticonvulsants were commenced orally one week prior to the elective operation. The incidence of postoperative convulsion was similar in both groups [12192 (13%) vs 10190 (11.1%). P 0.7) although there was a trend towards more early seizures in the phenytoin group [7192 (7.6% vs 2190(2.2%), P 0.09J. This may be due to a significantly larger number of patients in the phenytoin group with subtherapeutic serum level [32192 (35%) vs 19190 (21%), P 0.04]. Serious side effects such as cardiorespiratory depression, bone marrow suppression, hepatitis and exfoliative dermatitis were significantly more common in the phenytoin group [10192 (11%) vs 3190 (3.3%), P = 0.048]. These results suggest that sodium valproate, in comparison with phenytoin, is as effective in the prevention of post-operative epilepsy with significantly less serious side effects.
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Thursday, 10 July 1997
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14:00-16:30
Cerebrovascular Disorders Aneurysms and Vasospasm
I0-23-331 I Outcome of 353 intracranial aneurysms patients in a developing country
J.C. Lynch, R. Andrade, C. Pereira. Department of Neurological Surgery,
Servidoresdo EstadoHospitalRio de Janeiro, Brazil Introduction: To correlate the outcome of patients operated for cranial aneurysms from intensive high technology developed neurosurgical centers with the results of low technology, third world environment. Methods: Between January 1986 and December 1995, 353 patients with intracranial aneurysms were admitted to the Servidores do Estado Hospital. We retrospectively reviewed the medical and radiologic records and compared the outcome of this group with other series derived from developed countries. Results: The overall mortality of this series was 16.2%. Considering the 316 surgical patients (all grades) the mortality was 6.9%. Of the 294 good grades surgical patients, the mortality was 4.7% and the successful results were obtained in 88.7% individuals. Conclusion: Selected patients harboring intracranial aneurysms can be satisfactory handled in less developed nations, if a careful pre-operative evaluation and a metitulous intraoperative technique are employed, even though sophisticated and expensive technology and equipments are not available.
10-23-3321 Ruptured dissecting aneurysms as the cause of SAH with unverified etiology Hirofumi Nakatomi, Kazuya Nagata, Shunsuke Kawamoto, Yoshiaki Shiokawa.
Department of Neurosurgey, ShowaGeneralHospital, Tokyo, Japan Background & Purpose: The clinical features the 'aneurysmal' subarachnoid hemorrhage (SAH) with angiographically unverified etiology were reviewed in order to clarify the incidence and natural history of dissecting aneurysms as the hemorrhagic source of SAH. Methods: We reviewed 30 patients with SAH of unverified etiology with initial CT scan showing diffuse or anteriorly distributed subarachnoid blood clot. Ten of the patients had stenotic or occlusive lesions (SOCL) on initial angiography and are reported in this study.
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Results: Among 10 patients with SOCL on the initial angiographical study, the lesions were located in the anterior circulation in six and in the posterior circulation in four. Ruptured dissecting aneurysms were confirmed from exploratory surgery or autopsy in six patients. Subsequent rupture occurred in six (60%) of the 10 patients and all the six patients suffering from subsequent rupture died. Conclusion: The incidence (6/30) of dissecting aneurysms as the cause of the SAH with unverified etiology were unexpectedly high, especially when the initial angiographical study disclosed SOCL. The moribund patients with SOCL highly rebled and untreated recurrent hemorrhages were fatal. MR imaging is a very valuable diagnostic method combined with angiography because they can directly demonstrate an intramural hematoma in subacute or chronic stages. Surgical intervention can be justified as the initial treatment because of their high rebleeding rate when the following neuroradiological findings are satisfied, 1) SOCL on the angiography. 2) compatible distribution of SAH on CT with the location of the SOCL. 3) intramural hematoma on MRI in the same region of SOCL.
I0-23-3331
Diagnosis and treatment of nontraumatic intracranial dissecting aneurysm presenting with subarachnoid hemorrhage
S. Abiko, T. Okamura. Y. Kurokawa, N. Ikeda, K. Watanabe. Department of Neurosurgery, Ube Industries, CentralHospitalUbe, Japan Introduction: The correct diagnosis of a nontraumatic intracranial dissecting aneurysm (DA) that manifests with a subarachnoid hemorrhage (SAH) is very difficult in the acute stage because of the low incidence of true diagnostic signs such as a double lumen on cerebral angiography. In addition, the surgical treatment of choice has not been determined yet. Methods and Results: we reviewed eight cases of DA presenting with SAH for the iast 4 years to investigate the diagnosis. the indications for surgery, and the importance of follow-up angiography. The patients included 5 men and 3 women, ranging in age from 42 to 67 years. Neurological grade on admission was H&K II in four patients, H&K III in 1, and H&K IV in 3. Of the 8 patients, 3 suffered rebleeding within 24 hours after the first SAH. The aneurysm was located on the VA in 6 cases. on the MCA in t, and on the ACA in 1. Angiographic findings in the acute stage were irregular fusiform dilation in 4 cases, a double lumen in 1, the pearl and string sign in 1, and normal In 2. Enlargement with a double lumen and the pearl and string sign were observed in three patients at follow-up angiography. All eight patients underwent surgical procedures. Surgical procedures included 2 proximal vertebral artery obstructions, 3 trappings. 1 trapping with OA-PICA bypass, 1 wrapping and 1 bleb clipping. Postoperatively, there were 2 deaths and 6 patients showed good recovery. The cause of death was paralytic ileus in one and premature rupture in another. Conclusion: As the rerupture rate of DA is very high in the acute stage. early surgery may be necessary to prevent rupture. When the diagnosis of DA is difficult, careful strict follow-up angiography should be performed.
I0-23-3341 Unruptured vertebrobasilar dissecting aneurysms. Symptoms and treatment Rei Kondo, Kana Kunihiro, Hirokazu Endo, Shinjiro Saito, Takamasa Kayama. Department of Neurosurgery, Yamagata University School of Medicine,
Yamagata, Japan The treatment of unruptured vertebrobasilar dissecting aneurysms is not precisely known, thus we have investigated 11 consecutive cases presenting with ischemialinfarction and/or headache. The mean follow-up period was 2.7 years and mean age was 42.4 years. Serial angiography and MRI (3D-Time of flight with Gd) revealed 8 dissections which occurred in the vertebral artery (1 extended to the basilar artery), 2 in the posterior inferior cerebellar artery, 1 in the basilar artery. In principle, we treated them conservatively in the acute stage. If the follow up angiographical findings had deteriorated, surgery was considered 2111 (18.2%) were aggravated in acute stage. One patient was treated with proximal occlusion of the parent artery immediately after the clinical deterioration. Further neurological deterioration was prevented, and the patient showed good recovery with improvement of the angiographical findings, while another patient who was treated conservatively became severely disabled. The other 9 cases (2 patients underwent surgery) recovered well and returned to work. This result suggested that clinical deterioration of the unruptured vertebrobasiJar dissecting aneurysm may be much higher than is usually expected, and the natural history of the disorder seems to be unfavorable. In case of patients who deteriorate in the acute stage, we would like to emphasize that proximal occlusion in the appropriate lime may prevent the dissections from proceeding, and further neurological deteriorations.