Gliomatosis cerebri reporte de un caso y revisión de la literatura / Gliomatosis Cerebri case report and literatura review

La Gliomatosis Cerebri (GC) se define como un compromiso infiltrativo difuso de un tumor glial generalmente astrocítico que compromete al menos 3 lóbulos cerebrales de manera bilateral y usualmente la afección se extiende al tallo cerebral. Su incidencia representa aproximadamente el 1% de la totalidad de los tumores encefálicos y se presenta con mayor frecuencia en adultos jóvenes. El diagnóstico se establece por medio de la clínica, la imagenología y confrimación histológica. Las posibilidades terapéuticas son limitadas y la sobrevida cercana a los 2 años mostrando una alta mortalidad. Reportamos el caso de un paciente masculino de 11 años de edad quien fue atendido en el Hospital Militar Central de Bogotá Colombia donde se realizó el diagnóstico y se instauró el tratamiento

Gliomatosis Cerebri (GC) is defined as a diffuse infiltration of a glial tumor generally astrocytic that involves at least 3 cerebral lobes bilaterally and usually the condition extends to the brainstem. Its incidence represents approximately 1% of all brain tumors and occurs more frequently in young adults. The diagnosis is established for the clinic environment, imaging and histological confirmation. The therapeutic possibilities are limited and the survival close to 2 years showing a high mortality. We report the case of an 11-year-old male patient who was treated at the Central Military Hospital of Bogotá Colombia where the diagnosis was made and treatment was instituted

Hipertensão intracraniana relacionada a um raro padrão de neoplasia: gliomatose cerebral / Intracranial hypertension related to a rare pattern of neoplasm: gliomatosis cerebri

Gliomatose cerebral (GC) é um raro padrão de crescimento dos gliomas cerebrais, que infiltra difusamente os hemisférios cerebrais e estruturas adjacentes. As características radiológicas e histopatológicas são fundamentais para estabelecer ante mortem o diagnóstico. Em geral, a GC corresponde a uma neoplasia com diferenciação astrocítica e de grau histológico III / alto grau, a qual apresenta um curso clínico variável. No presente relato, os autores apresentam um caso de GC determinando hipertensão intracraniana, descrevem os principais achados histopatológicos e o diagnóstico diferencial desta neoplasia pouco frequente (AU)

Gliomatosis cerebri (GC) is a rare growth pattern of brain gliomas, which diffusely infiltrates the cerebral hemispheres and adjacent structures. Radiological and histopathological characteristics are key to establish ante-mortem diagnosis. In general, GC corresponds to a neoplasm with astrocytic differentiation and histological grade III/high degree, which presents a variable clinical course. In this report, the authors present a case of GC determining intracranial hypertension and describe the main histopathological findings and the differential diagnosis of this uncommon neoplasm (AU)

Procarbazine, CCNU, and Vincristine Chemotherapy in Gliomatosis Cerebri

A 49-year-old female patient was admitted due to memory disturbances. Magnetic resonance (MR) imaging suggested gliomatosis cerebri (GC), which had spread to both insular lobes, both frontal and basal ganglia and the brain stem. A stereotactic biopsy was performed at the high signal intensity area of the T2-weighted MR image, and the revealed a diffuse astrocytoma. Radiation therapy was judged not to be an appropriate treatment for the patient because of her cognitive impairment. A combinatorial chemotherapy regiment consisting of Procarbazine, CCNU, and Vincristine (PCV) was agreed upon after discussion. The patient underwent six cycles of PCV chemotherapy (a full dose was applied until the 3rd cycle, and dose then was reduced to 75% for the remaining cycles). Although the patient exhibited side effects such as bone marrow suppression and gastrointestinal symptoms, these were managed by medication. Over the 28 months following initiation of treatment, the high signal area in the right frontal and temporal lobes in the T2-weighted MR image decreased, and the patient's cognitive function [global deterioration scale (GDS) 4 points, mini-mental state examination (MMSE) 25 point] also improved (GDS 1 points, MMSE 29 points). PCV chemotherapy can therefore be an alternative therapeutic option for patients with GC who cannot be treated with radiation therapy or other chemotherapies.

Gliomatosis Cerebri Type 1 Mimicking an Ischemic Stroke and Progressing to a Type 2: a Case Study and Literature Review.

Aims: To report a rare clinical case of gliomatosis cerebri, which presented with non-specific clinical, laboratory, and radiological findings. We provide images and stress the importance of differential diagnosis based on imaging, especially magnetic resonance (MR) spectroscopy. Case Presentation: A 73-year-old woman developed a right hemiplegia suggestive of ischemic stroke. Cerebral magnetic resonance imaging (MRI) highlighted a diffuse tumor-related infiltration involving several lobes without contrast enhancement, corresponding to the specific description and definition of gliomatosis cerebri type 1. With the aid of MR spectroscopy, we correctly diagnosed the disease preoperatively, which was finally confirmed pathologically by stereotactic biopsy. During radiological follow-up, a contrast enhancement occurred on cerebral MRI, suggestive of progression to a gliomatosis cerebri type 2. Given a poor performance status, this elderly patient received palliative treatment. Discussion: Gliomatosis cerebri is a relatively rare but well-known entity, which affects mostly middle aged patients. It often presents with confounding clinical and imaging features, thus additional examinations such as MR spectroscopy are almost always necessary before reaching the correct diagnosis before biopsy. Conclusion: Contrast enhancement on cerebral MRI, which is usually absent, is found in case of transformation from type 1 gliomatosis cerebri to type 2. Some features on MR spectroscopy are helpful for gliomatosis cerebri diagnosis: N-acetylaspartate levels are diminished, levels of myoinositol are significantly elevated, but Cho/Cr ratio may be normal.

Gliomatosis cerebri.

A 6-year-old boy who presented with worsening hemiplegia, behaviour problems and seizures after an episode of encephalitis-like illness is reported. MRI revealed diffuse signal change and swelling of the left cerebral hemisphere. The diagnosis of gliomatosis cerebri was confirmed by brain biopsy. Parents refused radiotherapy and the child worsened and died 6 months after diagnosis.

Gliomatose cerebral na infância com evolução favorável: relato de caso / Gliomatosis cerebri with favorable outcome in a child: a case report

OBJETIVO: Relatar caso clínico raro de gliomatose cerebral difusa em criança, que, contrariando o observado na literatura, evoluiu de maneira clinicamente satisfatória. DESCRIÇÃO: Relatamos caso de criança que desenvolveu gliomatose cerebral com manifestações clínicas graves e progressivas. Exames iniciais foram inespecíficos. Melhora clínica inicial após cirurgia neuroendoscópica, porém piorou progressivamente, manifestando hidrocefalia obstrutiva, paraparesia e dificuldade de deambulação. Foram evidenciadas lesões disseminadas no espaço subaracnóideo, sendo realizadas nova intervenção cirúrgica, quimioterapia e radioterapia. Atualmente, após 6 anos de evolução, lesões no encéfalo e canal raquidiano estão inalteradas e há importante seringomielia. Entretanto, a criança apresenta-se clinicamente estável, com desenvolvimento adequado para sua idade, evidenciando resposta satisfatória ao tratamento. COMENTÁRIOS: A apresentação clínica e a propedêutica do caso levaram ao diagnóstico de gliomatose cerebral difusa do sistema nervoso central. Há poucos relatos na literatura desse tipo de tumor em crianças, e não se encontrou nenhum relato com evolução favorável como no caso apresentado.

OBJECTIVE: To report a rare clinical case of gliomatosis cerebri with favorable outcome in a 3-year old child. DESCRIPTION: A 3-year old child developed severe and progressive symptoms of gliomatosis cerebri. The initial tests were unspecific. After clinical improvement following neuroendoscopic surgery, there was a progressive decline in clinical status with development of obstructive hydrocephalus, paraparesis and difficulty in walking. The child was again submitted to surgery after disseminated injuries in the subarachnoid space were identified. She also received chemotherapy and radiotherapy. Currently, 6 years later, spinal canal and brain injuries remain unaltered, with marked syringomyelia. However, the child is clinically stable, with adequate development for her age, indicating a satisfactory response to treatment. COMMENTS: The child's clinical presentation and the combination of symptoms led to the diagnosis of gliomatosis cerebri. There are few descriptions of this kind of tumor in children in the literature, and none reports a favorable outcome as in the present case.

A Case of Gliomatosis Cerebri with Serial MRI Findings

No abstract available.

A Case of Gliomatosis Cerebri with Serial MRI Findings

No abstract available.

Diagnosis and differential diagnosis of gliomatosis cerebri with magnetic resonance imaging / 中国康复理论与实践

@# ObjectiveTo characterize gliomatosis cerebri on magnetic resonance imaging (MRI) and analyze differential diagnosis.MethodsMRI studies of 12 patients with gliomatosis cerebri were reviewed.ResultsTumors involved at least two lobes of the brain in all patients. Widespread invasion with hyperintensity was noted on T2-weighted MR images. No contrast enhancement occurred.Tumors were confirmed with surgery or biopsy.ConclusionGliomatosis cerebri is best detected with MRI.

Surgical treatment of gliomatosis cerebri / 医学研究生学报

Objective:To discuss the diagnosis, treatment and outcome of patients with gliomatosis cerebri (GC). Methods:Retrospectively reviewed the clinical manifestations and radiological appearances of 6 patients with GC, which were diagnosed in our hospital between 1993 and 2003. We employed surgical treatment in three patients, stereotactic biopsy in two, and the other one received both biopsy and surgery. Results: The lesions of GC infiltrated more than two lobes in brain. CT studies showed diffuse hypodensity changes and enhancement was absent in four patients. MRI examinations revealed isointense or hypointense regions on T1WI, and uniformly high signal on T2WI. MRI also disclosed structural enlargement. All patients received radiotherapy after surgery and three patients underwent chemotherapy additionally. Five patients died during follow up with average course of 16.4 months. Conclusion:MRI examination is valuable in the diagnosis and the prognosis of GC is poor.

Gliomatosis Cerebri in the Brain Stem and Unilateral Cerebellar Hemisphere: Case Report

Gliomatosis cerebri is an uncommon primary brain tumor characterized by diffuse neoplastic proliferation of glial cells, with the preservation of anatomical architecture and the sparing of the neurons. In this report, we present a gliomatosis cerebri in a 16-year-old girl which occurred in brain stem and unilateral cerebellar hemisphere.

A Case of Gliomatosis Cerebri; MRI and MR Spectroscopy Findings

No abstract available.

Gliomatosis CerebriClinical Features and Prognostic Factors of Long-term Survival

INTRODUCTION: The authors present a retrospective analysis of the clinical features and prognostic factors of long-term survival in gliomatosis cerebri. METHODS: The authors reviewed the clinical features of 27 cases of gliomatosis cerebri treated between August 1988 and January 2001. Age at diagnosis ranged from 19 to 62(median 41) years and the male to female ratio was 18:9. Most cases presented as a headache or seizure and the mean duration of symptoms was 9.6 months. An ill defined, diffuse high signal intensity lesion extending two lobes or more, without a central necrotic center in T2-weighted magnetic resonance(MR) imaging was characteristic. All patients underwent histological confirmation by craniotomy(12 cases), stereotactic biopsy(14 cases) and stereotactic biopsy followed by craniotomy(1 case). External beam radiation therapy was administered in every case except two, one of which expired within a month of surgery and the other refused treatment. RESULTS: Mean survival time after diagnosis was 41.1 months. By univariate analysis, the symptom duration(lower than 12 months) and the Karnofsky performance scale at discharge(lower than 70), focal enhancement on preoperative MR imaging, postoperative increased intracranial pressure sign, pathologic grade(high grade) and p53(>5%) were correlated with the length of survival(p-values were 0.07, 0.00, 0.007, 0.001, 0.04, and 0.02 respectively). CONCLUSION: We suggest that:1) gliomatosis cerebri can be diagnosed by a combination of MR imaging and histopathological examination:2) confusion with extensive glioma is possible due to vague diagnostic criteria, and so survival might be better than expected had discrete diagnosis been made. 3) Initial active management for increased ICP and further radiation therapy might be an important therapy.

Gliomatosis Cerebri: Clinical Features and Prognosis

OBJECTIVES: Gliomatosis cerebri is an uncommon primary brain tumor characterized by diffuse neoplastic proliferation of glial cells, with the preservation of the underlying cytoarchitecture. The aim of this study is to evaluate clinical features, outcome of surgical treatment and adjuvant therapy of gliomatosis cerebri. METHODS: Between Jan. 1990 and Dec. 2000, 12 patients were diagnosed with gliomatosis cerebri based on characteristic radiological and histological findings. The patients' age ranged from 18 to 77(mean 44) years and the male to female ratio was 7: 5. Nine patients underwent decompressive surgery and three, biopsy only. Postoperative radiation therapy was given in all cases except three. In addition to radiation therapy, four patients received chemotherapy. The mean duration of follow-up period was 18.8 months. RESULTS: The most common presenting symptom were seizure and motor weakness. The mean duration of symptom was 5.9 months. There was 5 bilateral lesions and tumor involved corpus callosum in 5, basal ganglia-thalamus in 4, and brain stem in 2. There was no operative mortality but four patients died during the follow-up. The mean survival period for 11 patients was 20.5 months from the time of diagnosis. In univariate analysis, the lesion involving corpus callosum, basal ganglia-thalamus and brain stem correlated significantly with the short length of survival(p<0.05). Also, postoperative radiation as a adjuvant therapy prolonged the patient's survival(p<0.05). CONCLUSIONS: In the management of gliomatosis cerebri patients, early detection by MR imaging, active management of increased intracranial pressure, decompressive surgical removal and postoperative adjuvant therapy such as radiation is thought to be a good treatment modality.