Clinical features and surgical outcome of patients with indolent brain tumors and epilepsy

INTRODUCTION: In this study the authors review the outcomes of 22 patients with medically refractory epilepsy and slow growth brain tumors. OBJECTIVES: Evaluate the clinical, electrophysiological, operative, and histopathological features. PATIENTS AND RESULTS: The majority of the tumors were located in the temporal lobe (n = 20) and involved the cortical gray matter. The most frequent tumors were gangliogliomas (n = 9), astrocytomas grade I and II (n = 6), dysembryoplastic neuroepithelial tumors (n = 5) and ganglioneuroma (n = 2). The biological behavior of the tumors was strikingly indolent, as indicated by a long preoperative history of chronic seizures (mean, 14 years). Mean follow-up time after resection was 27 months, and according to EngelÆs classification, 85 percent were seizure-free, 10 percent showed a reduction of seizure frequency of at least 90 percent, and 5 percent had reduction in seizure frequency at least 75 percent. CONCLUSION: The data indicate that neoplasms associated with pharmacoresistent epilepsy constitute a distinct clinicopathological group of tumors that arise in young patients, involve the cortex, and exhibit indolent biological behavior for many years. Complete surgical removal of these tumors, including the epileptogenic area, can achieve excellent seizure control.

INTRODUÇÃO: Neste estudo os autores avaliaram retrospectivamente 22 pacientes tratados cirurgicamente com diagnóstico de epilepsia refratária e tumor cerebral de crescimento lento. OBJETIVOS: Avaliar os aspectos clínicos, eletrofisiológicos, cirúrgicos e histopatológicos. PACIENTES E RESULTADOS: A maioria dos tumores estava localizada no lobo temporal (n = 20) com envolvimento da substância cinzenta. Ganglioglioma foi o tumor mais frequente (n = 9), seguido do astrocitoma grau I e II OMS (n = 6), tumor neuroepitelial disembrioplástico (DNET) (n = 5) e ganglioneuroma (n = 2). O comportamento biológico dos tumores foi estritamente indolente como indicado pela longa história pré-operatória de (média, 14 anos). O tempo de acompanhamento pós-operatório médio foi de 27 meses e de acordo com a Classificacão de Engel, 85 por cento ficaram sem crises (Classe I), 10 por cento obtiveram redução maior de 90 por cento das crises (Classe II), e 5 por cento tiveram redução menor que 75 por cento (Classe III). CONCLUSÃO: Os dados indicam que neoplasias associadas à epilepsia crônica refratária constituem um grupo de tumores com características clinico-patológicas distintas que se iniciam em pacientes jovens, envolvem o córtex e apresentam comportamento biológico indolente. A ressecção cirúrgica completa destes tumores, incluindo a zona epileptogênica, levou ao controle total das crises na maior parte dos casos estudados.

Neurosurgical management of cerebellar cavernous malformations.

OBJECT: The aim of this study was to analyze cerebellar cavernous malformations (CMs) with respect to epidemiological, clinical, radiological, and therapeutic aspects. METHODS: Between 1984 and 2004, 100 patients were surgically treated for intracranial CMs at the Division of Neurosurgery of Federal University of Sao Paulo. The authors reviewed the records of 10 patients whose lesions were located in the cerebellum. There were four male and six female patients (ratio 1:1.5) whose ages ranged from 14 to 45 years (mean age 33 years). Clinical presentation was sudden or acute in all cases, and neuroimaging examinations performed in all patients demonstrated signs of bleeding. The mean size of the malformations was 4.6 cm, and in all but one patient the lesions were totally removed without complications. After a mean follow-up period of 70 months, all patients were considered to be in good or excellent clinical condition. CONCLUSIONS: Cerebellar CMs should be analyzed separately from other posterior fossa CMs. These lesions can reach large sizes and cause massive hemorrhages, resulting in acute or sudden presentation. Surgery is a safe and effective option that provides a curative treatment when a complete removal is achieved.

MR cerebral blood volume maps correlated with vascular endothelial growth factor expression and tumor grade in nonenhancing gliomas.

BACKGROUND AND PURPOSE: Relative cerebral blood volume (rCBV) measurements derived from perfusion-weighted imaging (PWI) may be useful to evaluate angiogenesis and preoperatively estimate the grade of a glioma. We hypothesized that rCBV is correlated with vascular endothelial growth factor (VEGF) expression as marker of the angiogenic stimulus in presumed supratentorial low-grade gliomas (LGGs). METHODS: From February 2001 to February 2004, we examined 20 adults (16 men, four women; mean age 36 years; range, 23-60 years) with suspected (nonenhancing) supratentorial LGG on conventional MR imaging. Preoperative MR imaging used a dynamic first-pass gadolinium-enhanced, spin-echo echo-planar PWI. In heterogeneous tumors, we performed stereotactic biopsy in the high-perfusion areas before surgical resection. Semiquantitative grading of VEGF immunoreactivity was applied. RESULTS: Nine patients had diffuse astrocytomas (World Health Organization grade II), and 11 had other LGG and anaplastic gliomas. In patients with heterogeneous tumors on PWI, the high-rCBV focus had areas of oligodendroglioma or anaplastic astrocytoma on stereotactic biopsy, whereas the surgical specimens were predominantly astrocytomas. Anaplastic gliomas had high rCBV ratios and positive VEGF immunoreactivity. Diffuse astrocytomas had negative VEGF expression and mean rCBV values significantly lower than those of the other two groups. Three diffuse astrocytomas had positive VEGF immunoreactivity and high rCBV values. CONCLUSION: Our results confirmed the correlation among rCBV measurements, VEGF expression, and histopathologic grade in nonenhancing gliomas. PWI may add useful data to the preoperative assessment of nonenhancing gliomas. Its contribution in predicting tumor behavior and patient prognosis remains to be determined.

Stereotactic biopsy guidance in adults with supratentorial nonenhancing gliomas: role of perfusion-weighted magnetic resonance imaging.

OBJECT: The. diagnosis of low-grade glioma (LGG) cannot be based exclusively on conventional magnetic resonance (MR) imaging studies, and target selection for stereotactic biopsy is a crucial issue given the high risk of sampling errors. The authors hypothesized that perfusion-weighted imaging could provide information on the microcirculation in presumed supratentorial LGGs. METHODS: All adult patients with suspected (nonenhancing) supratentorial LGGs on conventional MR imaging between February 2001 and February 2004 were included in this study. Preoperative MR imaging was performed using a dynamic first-pass gadopentate dimeglumine-enhanced spin echo-echo planar perfusion-weighted sequence, and the tumors' relative cerebral blood volume (rCBV) measurements were expressed in relation to the values observed in contralateral white matter. In patients with heterogeneous tumors a stereotactic biopsy was performed in the higher perfusion areas before resection. Among 21 patients (16 men and five women with a mean age of 36 years, range 23-60 years), 10 had diffuse astrocytomas (World Health Organization Grade II) and 11 had other LGGs and anaplastic gliomas. On perfusion-weighted images demonstrating heterogeneous tumors, areas of higher rCBV focus were found to be oligodendrogliomas or anaplastic astrocytomas on stereotactic biopsy; during tumor resection, however, specimens were characterized predominantly as astrocytomas. Diffuse astrocytomas were associated with significantly lower mean rCBV values compared with those in the other two lesion groups (p < 0.01). The rCBV ratio cutoff value that permitted better discrimination between diffuse astrocytomas and the other lesion groups was 1.2 (80% sensitivity and 100% specificity). CONCLUSIONS: Perfusion-weighted imaging is a feasible method of reducing the sampling error in the histopathological diagnosis of a presumed LGG, particularly by improving the selection of targets for stereotactic biopsy.

[Arteriovenous malformation-glioma association: study of four cases]. / Associação de malforma o vascular e gliomas: estudo de quatro casos.

We reviewed the clinical presentation, imaging and histopathologic findings in 4 patients with the diagnosis of arteriovenous malformation associated with glioma that were operated on from 1991 to 2000 in our institution. Four patients (2 males; age between 15 and 52 years) presented with progressive headache with clinical evidence of intracranial hypertension (in 3) and partial seizures (in 1). CT scan showed a brain tumor without any detectable pathologic vessels. Histologic examination revealed astrocytic tumors associated with arteriovenous malformation. No patient presented the vascular component intermixed with the tumor. The arteriovenous-glioma association is rare and must be identified by a clear demarcation between the malformation and the tumor.

Associaçäo de malformaçäo vascular e gliomas: estudo de quatro casos / Arteriovenous malformation-glioma association: study of four cases

Entre os pacientes operados no Hospital São Paulo e acompanhados pelo setor de neuro-oncologia no período de 1991 a 2000, avaliamos a apresentaçäo clínica, aspectos de imagem e características histopatológicas de 4 pacientes (2 homens; idade entre 15 e 52 anos) cujo diagnóstico histológico foi malformaçäo vascular associada a glioma. O quadro inicial foi cefaléia progressiva com características de hipertensäo intracraniana (em 3) e crises parciais motoras (em 1). O diagnóstico tomográfico inicial foi processo expansivo, sem que houvesse suspeita de malformaçäo vascular pelo aspecto da imagem em nenhum caso. O exame histológico mostrou neoplasias de linhagem astrocítica associadas a malformações vasculares. Em nenhum paciente o componente vascular esteve localizado na intimidade da neoplasia. A associaçäo de malformaçäo vascular e gliomas é rara e deve ser caracterizada por nítida separaçäo entre a malformaçäo e a neoplasia, independente da vascularizaçäo própria do tumor

[Daily living activities evaluation in Parkinson's disease patients underwent to stereotactic surgery]. / Avaliação das atividades da vida diária dos pacientes com doença de Parkinson submetidos a cirurgia estereotáxica.

OBJECTIVE: To evaluate the impact of stereotactic surgery on daily activities of Parkinson's disease (PD) patients. METHOD: Thirty patients with idiopathic PD were evaluated before surgery and one, three, six and twelve months after surgery. Patients were evaluated with the UPDRS - part II (Unified Parkinson's Disease Rating Scale) and the Schwab & England scales. Nine of the patients had also been evaluated after twenty four months. RESULTS: We performed nine posteroventral pallidotomies (PVP), two on the left hemisphere and seven on the right; 17 ventrolateral thalamotomies (VLT), 12 on the left and five on the right; two VLT on the left associated with PVP on the right at the same surgical procedure. The mean "off" phase scores of Schwab & England scale were: 60.6 before surgery, 74 after the first, 76.6 after the third, 75.6 after the sixth, 72.3 after the twelfth and 71.1 after the twenty fourth months after surgery. The mean "off" phase scores of UPDRS - part II scale were: 21 before surgery, 12.3 after the first, 14.7 after the third, 15.27 after the sixth, 17.1 after the twelfth and 17.5 after the twenty fourth months after surgery. CONCLUSION: VTL and PVP are useful procedures to improve daily living activities of the PD patients and the best results are seen by six months after surgery.

Avaliação das atividades da vida diária dos pacientes com doençaáde Parkinson submetidos a cirurgia estereotáxica / Daily living activities evaluation in Parkinson's disease patients underwent to stereotactic surgery

OBJETIVO: Avaliar o impacto da cirurgia estereotáxica sobre a realização das atividades da vida diária dos paciente com doençaáde Parkinson (DP). MÉTODO: Foram avaliados 30 pacientes com DP forma idiopática submetidos a cirurgia estereotáxica nos períodos pré-operatório, 1 , 3 , 6 e 12 mêsápós-operatório, segundo as escala UPDRS - Item II (escala unificada para DP), Schwab & England e Hoehn & Yahr. Destes, nove pacientes foram também­áavaliados no 24 mêsápós-operatório. RESULTADOS: Foram realizadas nove palidotomias póstero-ventrais (PPV), sendo duas àáesquerda e sete àádireita; duas PPV bilaterais no mesmo tempo cirúrgico; 17 talamotomias ventro-laterais (TVL), sendo 12 àáesquerda e cinco àádireita; duas TVL àáesquerda com PPV àádireita no mesmo tempo cirúrgico. Os escores médios, na fase off, das escalas utilizadas foram: 65,6 no pré­-operatório, 74 no 1º mês, 76,6 no 3º mês, 75,6 no 6º mêsáe 72,3 no 12º mêsápós-operatório (Schwab & England); 21 no pré­-operatório, 12,3 no 1º mês, 14,7 no 3 º mês, 15,27 no 6º mêsáe 17,1 no 12 mêsápós-operatório (UPDRS); 3,1 no pré­-operatório, 2,8 no 1º mês, 2,7 no 3º mês, 2,8 no 6º mêsáe 2,85 no 12º mêsápós-operatório (Hoehn & Yarh). CONCLUSÄO: A TVL e a PPV sãoáprocedimentos capazes de melhorar a independência dos pacientes para realização das atividades cotidianas, sendo constatado benefício maior nos seis primeiros meses de pós-operatório

Medulloblastoma in adults: a series from Brazil.

We retrospectively reviewed 15 adult patients (11 males, median age 34 years; range 23-48) who had been treated and followed in our Institution since 1991 from the time of diagnosis until death or last follow-up in December 2001. Headache was the most frequent symptom (93%). The tumor was hemispheric in 11 patients. Complete resection was achieved in eight. CSF in 12 patients and craniospinal MRI in 6 did not show metastatic disease. Two patients refused adjuvant treatment and died with progressive disease. Thirteen patients received adjuvant craniospinal radiotherapy and 11 systemic chemotherapy. After initial treatment only 2 of the 13 patients relapsed in the posterior fossa. Recurrence was probably related to sub-optimal radiotherapy planning: inadequate low dose in the posterior fossa (37.5 Gy) and long delay in initiating treatment. Two of the 13 patients that received adjuvant treatment died: one from meningitis, and one from recurrent disease. Eleven patients remained alive, and disease-free with Karnofsky performance status ranging 80-100. The median overall survival was not reached after a median follow-up of 5.6 years (range 0.7-10.8 years). Estimated 1-, 5- and 10-year overall survival rates were 86.7%, 72.7%, and 72.7%, respectively. Adult medulloblastoma was predominant in males and the majority of patients had hemispheric tumors. Long-term survival was not uncommon. Although chemotherapy may be useful and well tolerated, radiotherapy remains the mainstay adjuvant treatment as suggested by our two recurrences associated with a delay or inadequate dose.

Tratamento cirúrgico das patologias vasculares cerebrais nos pacientes epilépticos / Surgical treatment of cerebral vascular pathologies in epileptic patients

A cirurgia das malformaçöes arteriovenosas (MAV) e dos cavernomas (angioma cavernoso) na maioria das vezes é indicada após episódios de sangramento. Como o desenvolvimento das técnicas de diagnóstico e cirúrgicas na epilepsia de difícil controle, passou a ser cada vez maior a indicaçäo cirúrgica destas lesöes vasculares. Apresentamos nove pacientes com lesöes vasculares cerebrais e crises muito frequentes, apesar do tratamento clínico adequado. A faixa etária variou de 12 a 42 anos de idade, com média de 25 anos; houve prevalência no sexo masculino (2:1). A cirurgia constitui na exérese da lesäo em todos os casos. Em quatro casos houve também ressecçäo da área irritativa perilesional, que foi demostrada pela eletrocorticografia. O estudo anatomopatológico das lesöes mostrou cinco casos de cavernoma, três de MAV e um angioma venoso. Quanto à localizaçäo, observamos três lesöes no lobo temporal, quatro no frontal e duas na regiäo parietal. O acompanhamento ambulatorial mostrou reduçäo das crises em todos os pacientes, sendo que sete evoluíram sem crises após a cirurgia.

Aneurismas intracranianos traumáticos / Traumatic intracranial aneurysms

Säo apresentados 4 casos de aneurismas intracranianos traumáticos em ramos distais, sendo 3 da artéria cerebral média e 1 da artéria cerebral anterior, todos operados após a ocorrência de hemorragia. em um dos pacientes, a hemoragia apresentou-se 15 anos após o trauma. E enfatizada a necessidade da arteriografia carotídea na avaliaçäo dos pacientes com traumatismo craniencefálico que apresentam suspeita de lesäo vascular

Hematoma extradural: estudo radiológico comparativo entre pacientes em coma e näo comatosos / Extradural hematoma: comparative radiological study between comatous and non-comatous patients

Considerando o estado neurológico imediatamente antes da operaçäo como a variável mais fidedigna para antecipaçäo do prognóstico do hematomas extradural, os autores realizaram estudo radiológico comparativo de 129 pacientes operados por esta patologia. Desta amostra, 78 casos se encontravam em coma (Grupo 1) e 31 eram näo comatosos (Grupo II). No grupo I, os 30 casos investigados pré-operatoriamente com tomografia tiveram 23,3// de mortalidade e 50// de bons resultados; dos 31 casos submetidos a angiografia, 48,3// faleceram e 38,7// tiveram bons resultados; 17 casos foram operados pela radiografia simples de crânio e exame neurológico, com 47// de mortalidade e 35,2// de bons resultados. A presença de fratura de crânio e a densidade tomográfica do hematoma näo interferiram nos resultados. A presença de lesäo associada intracraniana aumentou a mortalidade e diminuiu a quantidade de bons resultados tanto no Grupo I quanto no Grupo II. A localizaçäo frontal do hematoma foi associada a elevada mortalidade (52,6// no grupo comatoso, por estarem todos os pacientes entre 3 a 5 pontos na Escala de Glasgow e seis deles por apresentarem lesöes associadas intracranianas únicas ou múltiplas