Clinical and epidemiological characteristics of pediatric gliosarcomas.
Gliosarcoma (GS) is a glioblastoma with a sarcomatous component that is presumed to be a metaplastic differentiation of glioma cells. We studied the clinical relevance of this histological glioblastoma subentity within the pediatric population. We obtained patient data from the German HIT-GBM database, which contains clinical data for more than 600 pediatric patients with centrally reviewed high-grade gliomas. By applying defined inclusion criteria (diagnosis of GS proven by central neuropathological review; patient age 0 to 21 years), four patients were identified. In addition, after a review of the English medical scientific literature, 19 additional cases were found. The relative frequency of GS in the German HIT-GBM database was only 1.9%. In the whole series of 23 pediatric GS patients, including previously reported cases, the male-to-female-ratio was 1.2:1. GS was found in all pediatric age groups with a median age of 11 years, but there was an unexpectedly high accumulation in infants (6 of 23 <3 years of age, 26%). GS showed a strong predilection of the cerebral hemispheres (22 out of 23 cases). Increased intracranial pressure was the leading symptom of a short clinical history with a median duration of 0.7 month. Interestingly, six patients (26%) were reported with a history of cranial radiotherapy prior to GS diagnosis. In 60% of the GS patients in our series, gross total resection was achieved. Median overall (OS) and event-free survivals (EFS) of the total cohort were 12.1 and 9.8 months, respectively. In conclusion, GS is a very rare tumor entity in children. Literature review suggests a relatively higher incidence in infants and in patients with a previous history of radiotherapy.
Karremann
Michael
M
Department of Pediatrics and Adolescence Medicine, Martin Luther University Halle-Wittenberg, Halle, Germany.
Rausche
Ulrike
U
Fleischhack
Gudrun
G
Nathrath
Michaela
M
Pietsch
Torsten
T
Kramm
Christof M
CM
Wolff
Johannes E A
JE
D000293:Adolescent; D001932:Brain Neoplasms / Q000453:epidemiology* / Q000473:pathology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D018316:Gliosarcoma / Q000453:epidemiology* / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D055815:Young Adult