title: Prognostic factors in pediatric high-grade astrocytoma: the importance of accurate pathologic diagnosis.
abstract: To characterize a population of pediatric high-grade astrocytoma (HGA) patients by confirming the proportion with a correct diagnosis, and determine prognostic factors for survival in a subset diagnosed with uniform pathologic criteria. Sixty-three children diagnosed with HGA were treated at the Johns Hopkins Hospital between 1977 and 2004. A single neuropathologist (P.C.B.) reviewed all available histologic samples (n = 48). Log-rank analysis was used to compare survival by patient, tumor, and treatment factors. Median follow-up was 16 months for all patients and 155 months (minimum 54 months) for surviving patients. Median survival for all patients (n = 63) was 14 months with 10 long-term survivors (survival >48 months). At initial diagnosis, 27 patients were grade III (43%) and 36 grade IV (57%). Forty-eight patients had pathology slides available for review, including seven of ten long-term surviving patients. Four patients had non-HGA pathology, all of whom were long term survivors. The remaining 44 patients with confirmed HGG had a median survival of 14 months and prognostic analysis was confined to these patients. On multivariate analysis, five factors were associated with inferior survival: performance status (Lansky) <80% (13 vs. 15 months), bilaterality (13 vs. 19 months), parietal lobe location (13 vs. 16 months), resection less than gross total (13 vs. 22 months), and radiotherapy dose <50 Gy (9 vs. 16 months). Among patients with more than one of the five adverse factors (n = 27), median survival and proportion of long-term survivors were 12.9 months and 0%, compared with 41.4 months and 18% for patients with 0-1 adverse factors (n = 17). In an historical cohort of children with HGA, the potential for long term survival was confined to the subset with less than two of the following adverse prognostic factors: low performance status, bilaterality, parietal lobe site, less than gross total resection, and radiotherapy dose <50 Gy. Pathologic misdiagnosis should be suspected in patients who are long term survivors of a pediatric high grade astrocytoma.
authors.affiliation: Department of Radiation Oncology and Molecular Radiation Sciences, The Johns Hopkins University School of Medicine, 401 North Broadway, Suite 1440, Baltimore, MD 21231, USA. rhales1@jhmi.edu
authors.forename: Russell K
authors.initials: RK
authors.lastname: Hales
authors.forename: Ori
authors.initials: O
authors.lastname: Shokek
authors.forename: Peter C
authors.initials: PC
authors.lastname: Burger
authors.forename: Nina P
authors.initials: NP
authors.lastname: Paynter
authors.forename: Kaisorn L
authors.initials: KL
authors.lastname: Chaichana
authors.forename: Alfredo
authors.initials: A
authors.lastname: Quiñones-Hinojosa
authors.forename: George I
authors.initials: GI
authors.lastname: Jallo
authors.forename: Kenneth J
authors.initials: KJ
authors.lastname: Cohen
authors.forename: Danny Y
authors.initials: DY
authors.lastname: Song
authors.forename: Benjamin S
authors.initials: BS
authors.lastname: Carson
authors.forename: Moody D
authors.initials: MD
authors.lastname: Wharam
mesh_terms: D000293:Adolescent; D000367:Age Factors; D001921:Brain / Q000473:pathology*; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D015999:Multivariate Analysis; D018302:Neoplasms, Neuroepithelial / Q000175:diagnosis* / Q000401:mortality; D010372:Pediatrics; D011379:Prognosis; D012189:Retrospective Studies; D055815:Young Adult