Introduction Histological Classification of CNS Tumours Definitions and Explanatory Notes Tumours of Neuroepithelial Tissue Astrocytic Tumours Ependymal Tumours Mixed Gliomas Choroid Plexus Tumours Neuroepithelial Tumours of Uncertain Origin Neuronal and Mixed Neuronal-Glial Tumours Pineal Parenchymal Tumours Embryonal Tumours Tumours of Cranial and Spinal Nerves Schwannoma Neurofibroma Malignant Peripheral Nerve Sheath Tumor Tumours of the Meninges Tumours of Menigothelial Cells Mesenchymal, Non-menigothelial Tumours Primary Melanocytic Lesions Lymphomas and Haemopoietic Neoplasms Germ Cell Tumours Cysts and Tumour-like Lesions Tumours of the Sellar Region Local Extensions from Regional Tumours Metastatic Tumours Unclassified Tumours Illustrations Subject Index Metat Cy Tu

Histological Typing of tumours of the Central Nervous System

Gliomatosis cerebri (GC) is a rare disease, defined as a diffuse neoplastic glial cell infiltration of the brain. Diagnosis and management of GC are difficult. This study analyzed 296 individual cases (90 patients followed through the ANOCEF network, and 206 cases from the literature), aged 1 month to 85 years (median 42), sex ratio = 1.31. Median survival was 14.5 months. It was higher for patients younger than 42 years (17 months vs. 13 months), with performance status ≥80 (27 months vs. 9 months), low grade gliomatosis (grade 2 = 20 months, grade 3 = 11.5 months, grade 4 = 8.5 months), oligodendroglial subtype (36 months compared to 14 months for mixed GC and 11 months for astrocytic GC). Male population was younger (median 39 years vs. 45), had a higher incidence of oligodendroglial GC (22% vs. 13%), which may explain their better prognosis (median survival 17 months vs. 11.5 months) than female population. Despite a high rate of stabilization, the impact on survival of whole brain radiotherapy, which carries the risk of severe toxicity, is still unclear. Up-front chemotherapy benefit to some patients and may be prefered to whole brain radiotherapy. However, the many bias of such retrospective heterogeneous data claim for multicentric clinical trials in this rare disease.

Gliomatosis Cerebri: A Review of 296 Cases from the ANOCEF Database and the Literature

Objective To characterize the diagnosis and treatment of gliomatosis cerebri (GC). Methods Clinical studies of 13 patients with GC (1982-1997) were reviewed retrospectively. Results The main clinical presentation of the 13 cases included headache (11/13), seizure (4/13), and sign of evaluated intracranial pressure later. The tumor involved at least 2 lobes of brain in all patients, in which diffused infiltration in 6 cases and multiple lesions in 7 cases. Nine cases underwent partially surgical removal of the tumor lesions and 3 cases underwent stereotactic biopsy. The Pathologic diagnosis were low-grade astrocytoma (grade Ⅰ～Ⅱ) in 11, grade I oligodendrocytoma in 1, and both mixture in 1. The mean lifetime was 5.75 months. Conclusions The tumorigenesis, clinical presentation, characters of CT and MRI images, pathological classification, therapy and prognosis of GC were discussed.

Gliomatosis cerebri

BACKGROUND AND PURPOSE: The diagnosis of gliomatosis cerebri with MR imaging is known to be difficult. We report on the value of MR spectroscopy in the diagnosis, grading, and biopsy planing in eight patients with histopathologically proved gliomatosis cerebri.

METHODS: Patients underwent MR imaging and MR spectroscopy (single-voxel point-resolved spectroscopy [PRESS] at 1500/135, and chemical-shift imaging [CSI] PRESS at 1500/135) before open (n = 4) or stereotactic (n = 4) biopsy. In six patients who underwent CSI, biopsy samples were taken from regions of maximally elevated levels of choline/ N -acetylaspartate (Cho/NAA).

RESULTS: All patients showed elevated Cho/creatine (Cr) and Cho/NAA levels as well as varying degrees of decreased NAA/Cr ratios, which were most pronounced in the anaplastic lesions. In low-grade lesions, there was a maximum Cho/NAA ratio of 1.3, whereas in anaplastic tumors, the maximum Cho/NAA level was at least 2.5. Spectra in two patients with grade III lesions revealed a lactate peak; lactate and lipid signals were seen in two patients with grade IV lesions. Biopsy specimens from regions with maximally elevated levels of Cho/NAA showed dense infiltration of tumor cells.

CONCLUSION: MR spectroscopy might be used to classify gliomatosis cerebri as a stable or a progressive disease indicating its potential therapeutic relevance.

http://www.ajnr.org/content/ajnr/21/2/375.full.pdf

MR spectroscopy in gliomatosis cerebri.

Gliomatosis Cerebri (GC) is a rare tumor of the central nervous system. It is defined as a diffuse glial tumor that extensively infiltrates the brain, involving more than two lobes. And it is listed as a subtype of astrocytic tumors according to the newest 2007 (4th edition) WHO classification of tumors of the Central Nervous System. GC can be subdivided into Type I and Type II. Clinical findings for patients with GC are usually subtle and nonspecific. The lesions of GC generally show hypo, or isodensity on CT; a poorly defined diffuse hypo-or isointense signal on T1-weighted images, and a scattered diffuse hyperintense signal on T2-weighted images. Histological examination of GC reveals widespread infiltration of neoplastic glial cells with minimal destruction of pre-existing structures.

Gliomatosis Cerebri

A 46-year-old woman presented with a 2-month history of right hemiparesis and disorientation. CT showed only slightly abnormal findings, while the T2-weighted MRI findings disclosed high intensity in both hemispheres. Two weeks before her death, a biopsy specimen showed monotonous increase of gemistocyte-like cells, mimicking reactive gliosis. However, autopsy revealed some different cytological features; i.e. nuclear atypia and higher cellularity, and the diagnosis of gliomatosis cerebri was made. It is suggested that establishing an antemortem diagnosis of gliomatosis cerebri remains difficult in some cases, even with MRI and biopsy. Careful examination of autopsy material may be necessary for a definitive diagnosis.

Difficulties in the antemortem diagnosis of gliomatosis cerebri: report of a case with diffuse increase of gemistocyte-like cells, mimicking reactive gliosis.

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Difficulties in the antemortem diagnosis of gliomatosis cerebri: report of a case with diffuse increase of gemistocyte-like cells, mimicking reactive gliosis.