Prognostic factors in pediatric high-grade astrocytoma: the importance of accurate pathologic diagnosis
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Published on: November 17, 2021
Russell K Hales1, Ori Shokek, Peter C Burger
1Department 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
Insights
Pediatric high-grade astrocytoma (HGA) survival is linked to specific prognostic factors. Patients with fewer adverse factors, including performance status and tumor location, had significantly longer survival, suggesting potential for misdiagnosis in long-term survivors.
Area of Science:
- Pediatric neuro-oncology
- Cancer pathology
- Clinical outcomes research
Background:
- Pediatric high-grade astrocytoma (HGA) is a rare and aggressive brain tumor in children.
- Accurate diagnosis and identification of prognostic factors are crucial for improving patient outcomes.
- Previous studies have varied in diagnostic criteria and prognostic factor analysis.
Purpose of the Study:
- To confirm the diagnostic accuracy in a cohort of pediatric HGA patients.
- To identify prognostic factors influencing survival in children with pathologically confirmed HGA.
- To define a subset of patients with potential for long-term survival.
Main Methods:
- Retrospective review of 63 pediatric HGA cases treated between 1977 and 2004.
- Neuropathologist review of 48 available histologic samples to confirm diagnosis.
- Log-rank analysis to assess survival based on patient, tumor, and treatment variables.
Main Results:
- Four of 48 reviewed cases were misdiagnosed as HGA.
- Confirmed HGA patients (n=44) had a median survival of 14 months.
- Five adverse prognostic factors identified: poor performance status, bilaterality, parietal lobe location, subtotal resection, and radiotherapy dose <50 Gy.
- Patients with 0-1 adverse factors had significantly better survival (41.4 months) than those with multiple factors (12.9 months).
Conclusions:
- Long-term survival in pediatric HGA is limited to patients with fewer than two adverse prognostic factors.
- Pathologic misdiagnosis should be considered in long-term survivors of presumed pediatric HGA.
- Accurate pathologic review and consideration of prognostic factors are essential in managing pediatric HGA.
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.
