Abstract
Our aim was to determine the characteristics, treatments and outcomes of patients with primary spinal glioblastomas (GB) or gliosarcomas (GS) reported in literature until March 2015. PubMed and Web of Science were searched for peer-reviewed articles pertaining to cases of glioblastomas / gliosarcomas with primary spinal origin, using predefined search terms. Furthermore we performed hand searches tracking the references from the selected papers. Eighty-two articles published between 1938 and March 2015 were eligible. They reported on 157 patients. Median age at diagnosis was 22 years. The proportion of patients who received adjuvant chemo- or radiotherapy clearly increased from the time before 1980 until present. Median overall survival from diagnosis was 8.0 ± 0.9 months. On univariate analysis age influenced overall survival, whereas tumor location, gender and the extent of initial resection did not. Outcomes did not differ between children (< 18 years) and adults. However, the patients who were treated after 1980 achieved longer survival times than the patients treated before. On multivariable analysis only age (< 60 years) and the time period of treatment (≥ 1980) were confirmed as positive independent prognostic factors. In conclusion, primary spinal GB / GS mainly affect younger patients and are associated with a dismal prognosis. However, most likely due to the increasing use of adjuvant treatment, modest therapeutic progress has been achieved over recent decades. The characteristics and treatments of primary spinal glioblastomas should be entered into a central registry in order to gain more information about the ideal treatment approach in the future.
Highlights
In adults, glioblastoma (GB) is the most frequent primary intracranial neoplasm accounting for more than 50% of all cerebral tumors [1]
Data on childhood malignancies from the European Union show that only 3% of all children with central nervous system (CNS) tumors are affected by this disease [2]
We focused our analysis on patient characteristics, treatments, prognosis and factors associated with survival
Summary
Glioblastoma (GB) is the most frequent primary intracranial neoplasm accounting for more than 50% of all cerebral tumors [1]. Data on childhood malignancies from the European Union show that only 3% of all children with central nervous system (CNS) tumors are affected by this disease [2] Both -in adults and children- spinal origin of glioblastoma is rarely seen and the literature on the topic is mainly restricted to single case reports or small case series. It is noteworthy that there are crucial differences between adult and pediatric malignant gliomas in terms of tumor biology and clinical course, implying that the results from adult clinical trials may not be extrapolated to children and vice versa [3] Beyond their dismal prognosis, little is known about the clinical characteristics of primary spinal glioblastomas / gliosarcomas and their optimal treatment. By means of a systematic review referring to 157 cases of primary spinal GB / GS which were published in literature until March 2015, this paper updates and summarizes the existing knowledge on this rare disease
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