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

Abstract

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>or=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 preferred to whole brain radiotherapy. However, the many bias of such retrospective heterogeneous data claim for multicentric clinical trials in this rare disease.

Gliomatosis cerebri: no evidence for a separate brain tumor entity

“…Histologically, GC corresponds to diffusely infiltrating, mostly astrocytic gliomas of World Health Organization (WHO) grades II, III or IV [6]. GC with histological features of oligodendroglial differentiation have also been reported but are less frequent [21]. Although the current WHO classification of brain tumors [6] lists GC as a separate glial entity, this entity is not well defined beyond the criteria mentioned above.…”

“…Although the current WHO classification of brain tumors [6] lists GC as a separate glial entity, this entity is not well defined beyond the criteria mentioned above. The highly variable course of disease, with median survival of about 30 months after diagnosis and broad variation from a few months to >40 months [7,8,21], also challenges the view that GC comprises a distinct glioma entity. Moreover, molecular studies on GC have reported genetic alterations that are also common in diffuse and anaplastic astrocytic gliomas or glioblastomas, such as isocitrate dehydrogenese 1 (IDH1) mutation, tumor protein 53 (TP53) mutation and epidermal growth factor receptor (EGFR) amplification [4,5,7,9,11,13,14,18,21].…”

Clinical Manifestations and Imaging Characteristics of Gliomatosis Cerebri with Pathological Confirmation

“…The onset range of GC, ranging from infants of months to the elderly of 85 years, is large and there is no significant difference in morbidity between males and females (Taillibert et al, 2006;Narasimhaiah et al, 2012;Sun et al, 2012). GC is often misdiagnosed as cerebral hemorrhage disease and encephalitis in that its primary symptoms include headache, vomiting and stroke with less positive signs in initial stage, and (Rooney et al, 2013;Boele et al, 2014;Rudà et al, 2014).…”

Clinicopathological Diagnosis of Gliomatosis Cerebri

“…The deletion of the description on MIB-1 suggests the difficulty of the evaluation (Akimoto, 2004;Nishioka, 1996;Vates, 2003). There are many reports discussing the extremely poor prognosis of GC (Taillibert, 2006;Vates, 2003). However, recent studies have reported some cases with relatively better prognosis due to greater sensitivity to adjuvant therapy (Levin, 2004;Sanson, 2004;Taillibert, 2006).…”

“…Moreover, the 4th ed. states that GC is mainly astrocytic tumor but, in some cases, mainly consists of oligodendroglial tumor cells (Akimoto, 2004;Balko, 1996;Levin, 2004;Sanson, 2004, Taillibert, 2006. Unlike the 3rd ed., which specifies the histological malignancy as Grade III, the 4th ed.…”