Incidence and survival of gliomatosis cerebri: a population-based cancer registration study

Abstract

Gliomatosis cerebri (GC) comprises a rare widespread infiltrating growth pattern of diffuse gliomas. We explored the incidence patterns and survival rates of GC in a population-based registration sample from the Surveillance, Epidemiology and End, Results database (1973-2012). GC cases (n = 176) were identified based on their International Classification of Diseases in Oncology (ICD-O-3) morphology code (9381). We calculated age-adjusted incidence rates (AIR) and evaluated temporal trends. Survival was assessed with Kaplan-Meier curves and Cox regression models. The annual AIR of GC was 0.1/million. We noted increasing trends in the preceding registration years (1973-2002; annually, + 7%) and a tendency of clinical/radiological approaches to substitute the gold-standard histological assessment for diagnosis. GC was diagnosed in the entire age spectrum (range 1-98 years), but higher incidence rates (0.43/million) were noted among the elderly (≥ 65 years). A slight male preponderance was identified (male-to-female ratio: 1.4). Median overall survival was 9 months with a 5 year survival rate of 18%. Increasing age, primary tumor location not restricted to the cerebral hemispheres and rural residence at diagnosis were identified as negative prognostic factors, whereas receipt of radiotherapy, surgical treatment, race and method of diagnosis were not associated with outcome. This first comprehensive overview of GC epidemiology exemplifies the rarity of the disease, provides evidence for male preponderance and increased incidence among the elderly and shows lower survival rates compared to the published single center reports. Expansion of registration to histological and molecular characteristics would allow emergence of clinical prognostic factors at the population level.

Gliomatosis Cerebri Growth Pattern: Association of Differential First-Line Treatment with Overall Survival in WHO Grade II and III Gliomas

“…Radiotherapy has been attributed efficacy for GC treatment in case studies as well as retrospective studies containing a variety of radiation protocols [10,[24][25][26]. In contrast, studies with larger patient numbers failed to evidence the beneficial impact of radiotherapy on OS in GC patients [11,27,28]. Furthermore, no association between radiotherapy dose/ volume and OS has been established until now [10,29].…”

Gliomatosis cerebri mimicking diffuse demyelinating disease: Case Report

“…of 1648 patients found that seizures, associated with temporal lobe involvement, were present in roughly half of all patients; headache, associated with increased ICP, in 36% of patients; cognitive decline, associated with wide spread disease, in 32%; focal motor deficits, associated with motor cortex involvement, in 32%; and gait abnormalities, associated with cerebellar involvement, in 15% [4] . Rare, but important, complaints include blurred vision due to tumor involving of the optic nerves and pathways, cranial nerve palsies via involvement of the brainstem, and atypical parkinsonian syndromes via involvement of the basal ganglia [4 , 5] . The clinical exam findings and imaging/anatomical correlates of this case include left-sided weakness corresponding with involvement of the right paracentral lobule and corticospinal tract and global headache that was most likely related to increased ICP as evidence by diffuse effacement of the sulci and fissures.…”

Gliomatosis cerebri

“…Her extensive involvement evidenced by imaging suggests a gliomatosis cerebri growth pattern. Gliomatosis cerebri refers to an extensive and invasive growth pattern that involves at least three contiguous lobes and typically resembles a low-grade lesion radiologically (e.g., non-contrast enhancing), but is associated with a poor prognosis [1]. Until recently, it was classified separately within gliomas, but molecular studies did not warrant separation [2].…”