Gliomatosis Cerebri: A pediatric case series and review of the literature

Abstract

9056 Background: Gliomatosis Cerebri (GC) is a rare and universally fatal glial neoplasm of the central nervous system. Although over 160 cases have been reported in the medical literature, the largest pediatric case series published to date includes only three patients. We describe the largest cohort to date of children with GC and explore relationships between potential prognostic factors, treatment, and survival. Methods: Imaging, pathologic, and outcome information were reviewed for this case series of thirteen patients diagnosed with GC and treated at the Children’s Hospital of Philadelphia (CHOP) between 1982 and 2005. All patients had GC confirmed by biopsy. Twelve patients were treated with cranial irradiation; eight of these received adjuvant chemotherapy as well. A single patient under one year of age was treated with chemotherapy alone. A review of the literature identified 51 reported cases of GC in the pediatric population. Results: The progression free survival (PFS) in the CHOP cohort was 13% (range: 1.5–22.5 months) and the overall survival rate (OS) was 64% (range: 6.5–27 months) at 2 years. Time to progression was significantly prolonged for those with no evidence of tumor enhancement on initial imaging (p = .03). OS was significantly shorter for patients who presented in the first decade of life (p = .04). When survival data from patients reported in the literature are combined with the CHOP cohort, treatment significantly prolonged OS (p = .003). Conclusions: Outcome in pediatric patients with GC is extremely poor, however treatment prolongs OS. Age less that 10 years and enhancement on MRI at diagnosis may be risk factors for shorter survival. No significant financial relationships to disclose.