Abstract
Gliomatosis cerebri (GC) is a rare, extensively infiltrating glioma involving multiple contiguous lobes of the brain. This lethal disease affects all age groups, and the majority of patients have a poor outcome despite aggressive treatment. Despite its initial recognition in 1938, GC remains a controversial entity with little consensus in its definition, histology, or treatment. The majority of GC tumors are astrocytic, although mixed phenotypes have been identified. Treatment of GC is challenging as surgery is generally not an option due to the extensive areas of brain involved, the benefit of radiation therapy is unclear, and no chemotherapy has proven efficacy. Due to the rarity of the disease and its heterogeneity, both at histopathological and molecular levels, it is difficult to conduct clinical trials tailored for this diagnosis. This review summarizes our current knowledge, examines clinical studies focusing on the treatment of GC, highlights ongoing challenges, and discusses the recent molecular insights into adult and pediatric GC. We conclude that, although no longer recognized as a distinct pathological entity, GC represents a unique disease phenotype. Given the histologic and molecular overlap with other diffuse gliomas, the research emphasis should be on investigating its unique invasive biology.
Highlights
Gliomatosis cerebri (GC) is a rare, diffusely infiltrating glial brain tumor
Molecular differences were seen between pediatric and adult GC; the isocitrate dehydrogenase (IDH) subgroup was less common and no oligodendroglioma or RTK II subgroup was observed in children [1, 13, 31]
This study to retrospective historical cohorts who received radiotherapy only [7, 33, 34, 38], the NOA-05 trial results suggest that initial treatment with procarbazine and lomustine may have potential clinical benefit for patients with GC
Summary
Gliomatosis cerebri (GC) is a rare, diffusely infiltrating glial brain tumor. Prognosis is poor, with 26–52% surviving less than a year from symptom onset [1, 2]. The 2007 World Health Organization (WHO) classification of central nervous system (CNS) tumors defined GC as an extensively infiltrative diffuse glioma involving at least three cerebral lobes; the revised 2016 WHO CNS classification no longer identifies GC as a distinct pathological entity [3] Rather, this new classification designates it as a special pattern of growth and categorizes it under the various subtypes of diffuse gliomas. Patients with GC generally have a worse prognosis compared to patients with diffuse glioma of corresponding grades It is unclear whether this is due to the distinct GC biology causing extensive invasiveness or merely because of the fact the due to the large areas of brain involved, treatment options are limited. The question as to whether GC is a distinct disease entity or a distinctive phenotype of diffuse glioma will require additional investigations
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