Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 17 de 17
Filtrar
Mais filtros










Base de dados
Intervalo de ano de publicação
2.
Neurosciences (Riyadh) ; 19(2): 93-9, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24739404

RESUMO

Brainstem gliomas occur in 10-20% of brain tumors in pediatrics. Over the past 3 decades, the treatment of brainstem gliomas has significantly progressed as a result of the gradual advancements in microsurgical techniques, sophisticated imaging technology and, most importantly, the availability of MRI. In this article, we review the current literature on brainstem gliomas and cover diagnosis, imaging, classification, and management. Surgical approaches and intraoperative modalities to tackle operable cases of brainstem gliomas will be discussed in a follow up article.


Assuntos
Neoplasias do Tronco Encefálico , Pediatria , Neoplasias do Tronco Encefálico/classificação , Neoplasias do Tronco Encefálico/diagnóstico , Neoplasias do Tronco Encefálico/cirurgia , Humanos , Neuroimagem , Neurocirurgia
3.
Nat Genet ; 46(5): 457-461, 2014 May.
Artigo em Inglês | MEDLINE | ID: mdl-24705252

RESUMO

Diffuse intrinsic pontine gliomas (DIPGs) are highly infiltrative malignant glial neoplasms of the ventral pons that, due to their location within the brain, are unsuitable for surgical resection and consequently have a universally dismal clinical outcome. The median survival time is 9-12 months, with neither chemotherapeutic nor targeted agents showing substantial survival benefit in clinical trials in children with these tumors. We report the identification of recurrent activating mutations in the ACVR1 gene, which encodes a type I activin receptor serine/threonine kinase, in 21% of DIPG samples. Strikingly, these somatic mutations (encoding p.Arg206His, p.Arg258Gly, p.Gly328Glu, p.Gly328Val, p.Gly328Trp and p.Gly356Asp substitutions) have not been reported previously in cancer but are identical to mutations found in the germ line of individuals with the congenital childhood developmental disorder fibrodysplasia ossificans progressiva (FOP) and have been shown to constitutively activate the BMP-TGF-ß signaling pathway. These mutations represent new targets for therapeutic intervention in this otherwise incurable disease.


Assuntos
Receptores de Ativinas Tipo I/genética , Neoplasias do Tronco Encefálico/genética , Regulação Neoplásica da Expressão Gênica/genética , Genoma Humano/genética , Glioma/genética , Mutação de Sentido Incorreto/genética , Sequência de Bases , Neoplasias do Tronco Encefálico/classificação , Criança , Estudos de Coortes , Exoma/genética , Glioma/classificação , Humanos , Dados de Sequência Molecular , Miosite Ossificante/genética , Análise de Sequência de DNA , Transdução de Sinais/genética
4.
Nat Genet ; 46(5): 451-6, 2014 May.
Artigo em Inglês | MEDLINE | ID: mdl-24705254

RESUMO

Diffuse intrinsic pontine glioma (DIPG) is a fatal brain cancer that arises in the brainstem of children, with no effective treatment and near 100% fatality. The failure of most therapies can be attributed to the delicate location of these tumors and to the selection of therapies on the basis of assumptions that DIPGs are molecularly similar to adult disease. Recent studies have unraveled the unique genetic makeup of this brain cancer, with nearly 80% found to harbor a p.Lys27Met histone H3.3 or p.Lys27Met histone H3.1 alteration. However, DIPGs are still thought of as one disease, with limited understanding of the genetic drivers of these tumors. To understand what drives DIPGs, we integrated whole-genome sequencing with methylation, expression and copy number profiling, discovering that DIPGs comprise three molecularly distinct subgroups (H3-K27M, silent and MYCN) and uncovering a new recurrent activating mutation affecting the activin receptor gene ACVR1 in 20% of DIPGs. Mutations in ACVR1 were constitutively activating, leading to SMAD phosphorylation and increased expression of the downstream activin signaling targets ID1 and ID2. Our results highlight distinct molecular subgroups and novel therapeutic targets for this incurable pediatric cancer.


Assuntos
Receptores de Ativinas Tipo I/genética , Neoplasias do Tronco Encefálico/genética , Regulação Neoplásica da Expressão Gênica/genética , Genoma Humano/genética , Glioma/genética , Animais , Neoplasias do Tronco Encefálico/classificação , Criança , Variações do Número de Cópias de DNA , Metilação de DNA , Perfilação da Expressão Gênica , Glioma/classificação , Humanos , Proteína 1 Inibidora de Diferenciação/metabolismo , Proteína 2 Inibidora de Diferenciação/metabolismo , Fosforilação , Análise de Sequência de DNA , Proteínas Smad/metabolismo , Peixe-Zebra
5.
Childs Nerv Syst ; 29(8): 1321-31, 2013 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-23666431

RESUMO

PURPOSE: Pediatric brain stem tumors (BsT) are a heterogeneous group of diseases. Our aim was to analyze our experience to find out prognostic factors. METHOD: A retrospective study with BsT patients was performed. Imaging characteristics, extension of surgery, pathology, and adjuvant therapy were analyzed and correlated with overall survival (OS) and progression-free survival (PFS) as outcome measures. RESULT: Since 1980 to 2010, we analyzed 65 BsT patients, 41 of them girls (63%), median age of 8 years (range 13.9 months to 17.6 years). Twenty-two patients (33.8%) had diffuse intrinsic pontine gliomas (DIPG) and 43 (66.2%) presented with focal BsT. Histology was available in 42 patients; the most frequent is low-grade glioma in 24/42 patients (57%). DIPG's histology (obtained usually at necropsy) confirmed five high-grade gliomas. After median follow-up of 49.3 months (0.5-175 months), 20/22 DIPG patients have died (90.9%), while 27/43 with focal tumors were alive (62.8%). Variables related to outcome were histology (better for low-grade glioma (LGG) OS p < 0.001), surgery (better if operated OS p < 0.001), and adjuvant therapy (worse if given, PFS p = 0.001, OS p = 0.024). The outcome for DIPG was dismal, median OS/EFS of 14.2/9.4 months, significantly worse than focal BsT (p = 0.000), while OS/EFS was 122.8/87.2 months for focal intrinsic, 88.2/47.1 months for exophytic, and 124.4/54 months for cervico-medullary tumors: no differences were found among them, except the histology (OS p < 0.001 for low-grade vs high-grade tumors). CONCLUSION: BsT in children comprised two different groups: diffuse (DIPG) and focal gliomas. The DIPGs continue having a dismal prognosis, needing new approaches, while focal tumors including LGG have better prognosis.


Assuntos
Neoplasias do Tronco Encefálico/diagnóstico , Neoplasias do Tronco Encefálico/terapia , Adolescente , Idade de Início , Neoplasias do Tronco Encefálico/classificação , Neoplasias do Tronco Encefálico/complicações , Neoplasias do Tronco Encefálico/patologia , Criança , Pré-Escolar , Transtornos Cognitivos/etiologia , Terapia Combinada , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Hidrocefalia/etiologia , Lactente , Imagem por Ressonância Magnética , Masculino , Complicações Pós-Operatórias , Estudos Retrospectivos
6.
Can J Neurol Sci ; 40(3): 348-54, 2013 May.
Artigo em Inglês | MEDLINE | ID: mdl-23603170

RESUMO

OBJECTIVE: In most studies, treatment decisions of brainstem glioma are based solely on MRI features and do not incorporate a histopathological diagnosis. In the current study, we sought to compare MRI characteristics with histopathological findings of bainstem glioma. METHODS: From April 2003 through April 2012, 150 patients were diagnosed with brainstem gliomas by MRI and microsurgically treated in Tiantan Hospital, Beijing, China. All the MRI and histopathological findings of these patients were respectively reviewed. RESULTS: Of the 150 patients, 65 were female and 85 were male, 120 were adults and 30 were children (age < 18 years), 108 were low-grade glioma (72.0%), 35 were high-grade glioma (23.3%). The accuracy of the MRI diagnosis for brainstem glioma was 95.3%. Data analysis of the MRI findings revealed that a focal lesion was associated with a more favorable histopathological diagnosis in intrinsic (P=0.005) and exophytic (P=0.001) brainstem glioma patients. In the intrinsic diffuse type, tumors without enhancement had more favorable pathological findings (P=0.009). CONCLUSIONS: To our knowledge, this is the largest case series of this nature reported in the literature to date. The results of this study suggest that MRI features of brainstem gliomas could predict some pathological features and guide prognosis, choice of biopsy and treatment modalities. The pathology of tumors with a focal appearance on MRI was associated with a prognosis that was significantly better than their diffuse counterparts. For the intrinsic diffuse gliomas, non-enhancing tumors had pathology suggestive of a favorable prognosis.


Assuntos
Neoplasias do Tronco Encefálico/diagnóstico , Glioma/diagnóstico , Imagem por Ressonância Magnética , Adolescente , Adulto , Biópsia , Neoplasias do Tronco Encefálico/classificação , Neoplasias do Tronco Encefálico/cirurgia , Criança , Feminino , Glioma/classificação , Glioma/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos/métodos , Estudos Retrospectivos , Adulto Jovem
7.
Oncologist ; 17(3): 388-97, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22382458

RESUMO

Brainstem gliomas are uncommon in adults and account for only 1%-2% of intracranial gliomas. They represent a heterogeneous group of tumors that differ from those found in their pediatric counterparts. In adults, a low-grade phenotype predominates, which is a feature that likely explains their better prognosis compared to that in children. Because biopsies are rarely performed, classifications based on the radiological aspect of magnetic resonance imaging results have been proposed to establish treatment strategies and to determine outcomes: (a) diffuse intrinsic low-grade, (b) enhancing malignant glioma, (c) focal tectal gliomas, and (d) exophytic gliomas. Despite significant advances in neuroradiology techniques, a purely radiological classification remains imperfect in the absence of a histological diagnosis. Whereas a biopsy may often be reasonably avoided in the diffuse nonenhancing forms, obtaining histological proof seems necessary in many contrast-enhanced brainstem lesions because of the wide variety of differential diagnoses in adults. Conventional radiotherapy is the standard treatment for diffuse intrinsic low-grade brainstem gliomas in adults (the median survival is 5 years). In malignant brainstem gliomas, radiotherapy is the standard treatment. However, the possible benefit of combined radiotherapy and chemotherapy (temozolomide or other agents) has not been thoroughly evaluated in adults. The role of anti-angiogenic therapies in brainstem gliomas remains to be defined. A better understanding of the biology of these tumors is of primary importance for identifying homogeneous subgroups and for improving therapy options and outcomes.


Assuntos
Neoplasias do Tronco Encefálico/patologia , Neoplasias do Tronco Encefálico/terapia , Glioma/patologia , Glioma/terapia , Adulto , Idade de Início , Neoplasias do Tronco Encefálico/classificação , Neoplasias do Tronco Encefálico/epidemiologia , Seguimentos , Glioma/classificação , Glioma/epidemiologia , Humanos , Imagem por Ressonância Magnética/métodos , Estadiamento de Neoplasias , Prognóstico
9.
Neurol India ; 57(3): 274-81, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19587467

RESUMO

BACKGROUND: Brainstem gliomas are highly heterogeneous tumors both in their clinical manifestation and in their pathology. Despite significant advances in the surgery for brainstem gliomas many aspects of this pathology are still unclear. OBJECTIVE: To evaluate the clinical, radiological and surgical outcome of 40 focal 'intrinsic' brainstem gliomas and propose a surgical strategy-oriented classification. MATERIALS AND METHODS: A total of 40 focal 'intrinsic' ("expanding variety") tumors have been operated over a period of 8.5-years (January 1998-June 2007). Our criteria included patients with (1) well-defined gadolinium enhancing tumor; (2) relatively long duration of symptoms (> six months) and (3) good neurological functional status and independent for all activities of daily living. The cutoff size of 2 cm was not rigidly adhered to. RESULTS: The 'intrinsic' brainstem tumors were classified into three types: Expanding, diffuse infiltrative and pure ventral varieties. Only patients with expanding variety of brainstem gliomas were subjected to surgery, mean age 19.2 years (range 4-55 years) and male to female ration mean: 3:2). The tumor location included pons (n=19), midbrain (n=13) and medulla (n=8). Surgical approaches included midline suboccipital (n=28), retromastoid (n=7), subtemporal (n=3) and supracerebellar-infratentorial (n=2). Thirty-two cases with 'diffuse infiltrative' and 'pure ventral' variety were given radiotherapy only. Histology pathology revealed pilocytic variety (n=10), Grade II (n=17) and Grade III (n=13). There was one death in the surgical series (due to aspiration). Complications included meningitis (n=2), wound infection (n=1), chest infection (n=5) and transient mutism (n=1). Follow-up ranged from 3-68 months. Overall, 36 improved /remained same and three worsened in their clinical status at the time of discharge. CONCLUSION: The surgical management of intrinsic brainstem tumors presents a surgical challenge; radical excision yielded a good outcome in the majority of cases. The authors propose a classification system for 'intrinsic' brainstem tumors for defining surgical strategy.


Assuntos
Neoplasias do Tronco Encefálico/classificação , Neoplasias do Tronco Encefálico/cirurgia , Glioma/classificação , Glioma/cirurgia , Neurocirurgia/métodos , Adolescente , Adulto , Fatores Etários , Neoplasias do Tronco Encefálico/diagnóstico , Neoplasias do Tronco Encefálico/mortalidade , Criança , Pré-Escolar , Feminino , Glioma/diagnóstico , Glioma/mortalidade , Humanos , Imagem por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Análise de Sobrevida , Resultado do Tratamento , Adulto Jovem
10.
J Neurosurg ; 106(2 Suppl): 111-9, 2007 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-17330536

RESUMO

OBJECT: Data analysis was performed in a multicenter study to evaluate magnetic resonance (MR) imaging for classification of brain tumors, prognosis, and prediction of tumor histological diagnosis. METHODS: The clinical, MR imaging, and histological findings obtained in 142 pediatric cases of brainstem disease were assessed in a multicenter study performed as a blinded review. Clinical data were available in 142 cases, histopathological findings in 126, and MR images in 131. The subgroup of cases involving brainstem gliomas (78 cases) was analyzed separately. Images that met criteria for evaluation were reviewed in random order by three experienced observers who were initially blinded to clinical data as well as histopathological diagnoses. Subsequently, the images were randomized again and provided to the observers for review together with the clinical symptoms of the specific patients. The three observers were able to correctly identify lesions as tumors or nontumorous disease on MR images in 99, 96, and 95% of cases, resulting in an overall sensitivity of 0.94, a specificity of 0.43, a positive predictive value of 0.96, and a negative predictive value of 0.45. Awareness of clinical symptoms did not change the results. CONCLUSIONS: Based on 14 imaging criteria together with the patient's clinical history and symptoms, laboratory data (results of cerebrospinal fluid analysis as well as infectious and immunological parameters), and imaging follow up, a diagnosis of brainstem tumor, as opposed to demyelination, encephalitis, or granuloma, could generally be made. Given these findings, there is only rarely a need for biopsy, and in those patients in whom it is considered, the potential costs and benefits must be carefully assessed on a case-by-case basis.


Assuntos
Biópsia , Encefalopatias/diagnóstico , Neoplasias do Tronco Encefálico/diagnóstico , Tronco Encefálico/patologia , Imagem por Ressonância Magnética , Adolescente , Biópsia/efeitos adversos , Neoplasias do Tronco Encefálico/classificação , Criança , Pré-Escolar , Doenças dos Nervos Cranianos/diagnóstico , Doenças Desmielinizantes/diagnóstico , Encefalite/diagnóstico , Feminino , Seguimentos , Glioma/diagnóstico , Granuloma/diagnóstico , Humanos , Lactente , Masculino , Valor Preditivo dos Testes , Prognóstico , Sensibilidade e Especificidade , Método Simples-Cego
11.
Neurol Neurochir Pol ; 39(1): 69-74; discussion 75-6, 2005.
Artigo em Polonês | MEDLINE | ID: mdl-15735993

RESUMO

The article presents an MRI-based classification of brainstem gliomas into focal, cervicomedullary, dorsal exophytic and diffuse ones. This classification provides the basis for specifying indications for surgical treatment and outcome. The article also presents the most frequent approaches to the midbrain, pons and medulla oblongata. These approaches include the pterional, orbito-zygomatic, subtemporal transtentorial and supracerebellar approaches to the midbrain. Suboccipital, trans fourth ventricle, subtonsillar, retrosigmoid and anterior petrosal approaches were used in the case of the pons. Suboccipital, trans fourth ventricle and transcondylar approaches were applied for the removal of tumors of medulla oblongata. This paper elaborates on rare approaches: transcondylar, paramedian-supracerebellar, subtonsillar and anterior petrosal ones effectively applied in our clinic. The resection of brain stem tumors is performed by piecemeal resection and not by removal en bloc. We stress the significance of safe entry zones to the brain stem and places at the fossa rhomboidea whose impairment may cause severe disability. Lesion of trigonum nervi hypoglossi, trigonum nervi vagi, colliculus facialis and fasciculus longitudinalis medialis leads to severe disability or death of the patient.


Assuntos
Neoplasias do Tronco Encefálico/cirurgia , Glioma/cirurgia , Procedimentos Neurocirúrgicos/métodos , Neoplasias do Tronco Encefálico/classificação , Neoplasias do Tronco Encefálico/patologia , Glioma/classificação , Glioma/patologia , Humanos , Imagem por Ressonância Magnética , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Complicações Pós-Operatórias/prevenção & controle
12.
Neurocirugia (Astur) ; 15(1): 56-66, 2004 Feb.
Artigo em Espanhol | MEDLINE | ID: mdl-15039850

RESUMO

Brainstem gliomas have been increasingly understood in the last two decades and they are nowadays regarded as an heterogeneous group of tumors with tendency towards the pediatric age, where they account for 10-20% of brain neoplasms. Besides the well known diffuse tumor, several subtypes, with a different biological behaviour, amenable to surgical resection and better prognosis, have been identified, giving rise to many classifications and terms. In the other way, attention has been recently paid to adult brainstem gliomas in contrast to pediatric tumors. Based on a review of the literature, we describe the different subtypes of brainstem gliomas, with particular interest on therapeutic approaches and differences between pediatric and adult tumors, employing iconography from our series.


Assuntos
Neoplasias do Tronco Encefálico/classificação , Glioma/classificação , Adulto , Criança , Humanos
13.
Childs Nerv Syst ; 20(3): 143-53, 2004 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-14669023

RESUMO

INTRODUCTION: Brainstem gliomas have historically been one of the most difficult pediatric cancers to treat. Tumors arising in the brainstem were once uniformly discounted as surgically unresectable lesions. Early neurosurgeons thought this location to be inoperable and fraught with disaster. The advent of computed tomography (CT), magnetic resonance imaging (MRI), and sophisticated neurophysiological monitoring techniques have significantly advanced the surgical treatment of these precarious lesions. REVIEW: Brainstem gliomas are now recognized as a heterogenous group of tumors. They have been broadly classified into several categories depending upon the classification scheme. All these classification systems provide a framework to predict growth patterns, surgical resectability, and overall prognosis of these tumors. These systems allow the surgeon to obtain a better understanding of the distinction between low-grade tumors and diffuse inoperable tumor types. The authors review the current literature and management of brainstem tumors.


Assuntos
Neoplasias do Tronco Encefálico/cirurgia , Glioma/cirurgia , Tronco Encefálico/patologia , Tronco Encefálico/cirurgia , Neoplasias do Tronco Encefálico/classificação , Neoplasias do Tronco Encefálico/diagnóstico , Neoplasias do Tronco Encefálico/patologia , Criança , Glioma/classificação , Glioma/diagnóstico , Glioma/patologia , Humanos , Imagem por Ressonância Magnética , Monitorização Intraoperatória , Ponte/patologia , Ponte/cirurgia , Prognóstico , Tomografia Computadorizada por Raios X
14.
Folia Neuropathol ; 41(4): 227-30, 2003.
Artigo em Inglês | MEDLINE | ID: mdl-14977252

RESUMO

Over the last 25 years there have been reports from the widely recognised neurosurgical centres on the positive clinical results of partial and even complete removal of brainstem tumours. They confirmed relatively high incidence of benign tumours like low-grade gliomas and haemangioblastomas in this region. Based on imaging studies (MRI) and surgical experiences, brainstem tumours can be divided into focal and diffuse. Focal lesions are amenable to surgery; particularly, those with a prominent exophytic portion. Open surgery remains controversial in pure intrinsic brainstem tumours as the surgical approach itself may lead to serious postoperative complications. Different approaches (including the most frequent one--through the fourth ventricle floor) are used depending on the tumour location. As minimal invasiveness is mandatory during transtegmental route safe approach zones within the rhomboid fossa were defined morphologically and morphometrically--suprafacial and infrafacial (i.e., situated above and below facial colliculus, respectively). Application of the proposed minimal invasive surgical approach through the fourth ventricle floor should reduce postoperative morbidity and mortality in patients with tumours in pons and upper medulla oblongata treated surgically.


Assuntos
Neoplasias do Tronco Encefálico/cirurgia , Quarto Ventrículo/cirurgia , Procedimentos Cirúrgicos Minimamente Invasivos , Procedimentos Neurocirúrgicos , Neoplasias do Tronco Encefálico/classificação , Quarto Ventrículo/anatomia & histologia , Humanos , Imagem por Ressonância Magnética , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Procedimentos Neurocirúrgicos/métodos
15.
Curr Opin Neurol ; 14(6): 711-5, 2001 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-11723378

RESUMO

Brainstem gliomas are now regarded as a heterogeneous group of tumors that can be distinguished by age of onset, clinical and radiological presentation and biological behavior. This paper will focus on each subtype of tumor, in children and in adults, and on recent advances in diagnostic criteria and therapeutic options.


Assuntos
Neoplasias do Tronco Encefálico/diagnóstico , Glioma/diagnóstico , Adolescente , Adulto , Neoplasias do Tronco Encefálico/classificação , Neoplasias do Tronco Encefálico/mortalidade , Neoplasias do Tronco Encefálico/terapia , Criança , Pré-Escolar , Glioma/classificação , Glioma/mortalidade , Glioma/terapia , Humanos , Lactente , Prognóstico , Taxa de Sobrevida
16.
Brain ; 124(Pt 12): 2528-39, 2001 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-11701605

RESUMO

In contrast to childhood brainstem gliomas, adult brainstem gliomas are rare and poorly understood. The charts of 48 adults suffering from brainstem glioma were reviewed in order to determine prognostic factors, evaluate the effect of treatment and propose a classification of these tumours. Mean age at onset was 34 years (range 16-70 years). The main presenting symptoms were gait disturbance (61%), headache (44%), weakness of the limbs (42%) and diplopia (40%). Four patterns were identified on MRI, representing non-enhancing, diffusely infiltrative tumours (50%), contrast-enhancing localized masses (31%), isolated tectal tumours (8%) and other patterns (11%). Treatment consisted of partial resection (8%), radiotherapy (94%) and chemotherapy (56%). Overall median survival was 5.4 years. On univariate analysis, the following favourable prognostic factors were identified (P< 0.01): age of onset <40 years, duration of symptoms before diagnosis >3 months, Karnofski performance status >70, low-grade histology, absence of contrast enhancement and 'necrosis' on MRI. On multivariate analysis, the duration of symptoms, the appearance of 'necrosis' on MRI and the histological grade of the tumour remained significant and independent prognostic factors (P< 0.05). Eighty-five percent of the tumours could be classified into one of the following three groups on the basis of clinical, radiological and histological features. (i) Diffuse intrinsic low-grade gliomas (46%) usually occurred in young adults with a long clinical history before diagnosis and a diffusely enlarged brainstem on MRI that did not show contrast enhancement. These patients were improved by radiotherapy in 62% of cases and had a long survival time (median 7.3 years). Anaplastic transformation (appearance of contrast enhancement, 27%) and relentless growth without other changes (23%) were the main causes of death. (ii) Malignant gliomas (31%) occurred in elderly patients with a short clinical history. Contrast enhancement and necrosis were the rule on MRI. These tumours were highly resistant to treatment and the patients had a median survival time of 11.2 months. (iii) Focal tectal gliomas (8%) occurred in young patients and were often revealed by isolated hydrocephalus. The course was indolent and the projected median survival period exceeded 10 years. In conclusion, adult brainstem gliomas are different from the childhood forms and resemble supratentorial gliomas in adults. Low-grade tumours have a clinicoradiological pattern that is so characteristic that the need for a potentially harmful biopsy is debatable. The optimum timing of treatment for supratentorial low-grade tumours remains unclear. In high-grade gliomas, the prognosis remains extremely poor despite aggressive treatment with radiotherapy and chemotherapy.


Assuntos
Neoplasias do Tronco Encefálico/classificação , Neoplasias do Tronco Encefálico/mortalidade , Glioma/classificação , Glioma/mortalidade , Adulto , Fatores Etários , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Tronco Encefálico/patologia , Neoplasias do Tronco Encefálico/patologia , Neoplasias do Tronco Encefálico/terapia , Progressão da Doença , Feminino , Glioma/patologia , Glioma/terapia , Humanos , Hidrocefalia/etiologia , Hidrocefalia/patologia , Imagem por Ressonância Magnética , Masculino , Análise Multivariada , Prognóstico , Radioterapia , Taxa de Sobrevida , Teto do Mesencéfalo/patologia
17.
Cancer ; 89(7): 1569-76, 2000 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-11013373

RESUMO

BACKGROUND: Brain stem tumors in children have been classified pathologically as low grade or high grade gliomas and descriptively as diffuse gliomas, intrinsic gliomas, midbrain tumors, tectal gliomas, pencil gliomas, dorsal exophytic brain stem tumors, pontine gliomas, focal medullary tumors, cervicomedullary tumors, focal gliomas, or cystic gliomas. METHODS: To search for a simplified and prognostic clinicopathologic scheme for brain stem tumors, the authors reviewed a consecutive cohort of patients younger than age 21 years with tumors diagnosed from 1980 through 1997. Pathology specimens and neuroimaging were classified by masked review. Statistical and survival analysis along with Cox proportional hazards regression was performed. RESULTS: Seventy-six patients were identified, with initial diagnostic magnetic resonance imaging available for 51 and pathology specimens for 48 patients. Twenty cases were classified histologically as pilocytic astrocytoma (PA), 14 as fibrillary astrocytoma (FA), and 14 as other tumors or indeterminate pathology. For all tumors, characteristics significantly associated with a worse survival rate were: symptom duration less than 6 months before diagnosis (P = 0.004); abducens palsy at presentation (P < 0.0001); pontine location (P = 0.0002); and engulfment of the basilar artery (P = 0.006). Pilocytic astrocytoma was associated with location outside the ventral pons (P = 0.001) and dorsal exophytic growth (P = 0.013); Fibrillary astrocytoma was associated with symptoms less than 6 months (P = 0. 006), abducens palsy (P < 0.001), and engulfment of the basilar artery (P = 0.002). Pilocytic astrocytoma showed 5-year overall survival (OS) of 95% (standard error [SE], 5%) compared with FA 1-year OS of 23% (SE, 11%;P < 0.0001). CONCLUSIONS: Brain stem tumors can be succinctly and better biologically classified as diffusely infiltrative brain stem gliomas-generally FA located in the ventral pons that present with abducens palsy, often engulf the basilar artery, and carry a grim prognosis-and focal brain stem gliomas-frequently PA arising outside the ventral pons, often with dorsal exophytic growth, a long clinical prodrome, and outstanding prognosis for survival. Our findings emphasize the individuality of PA as a distinct clinicopathologic entity with an exceptional prognosis.


Assuntos
Astrocitoma/patologia , Neoplasias do Tronco Encefálico/patologia , Adolescente , Adulto , Astrocitoma/classificação , Neoplasias do Tronco Encefálico/classificação , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Imagem por Ressonância Magnética , Masculino , Estadiamento de Neoplasias , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA