Outcome analysis of childhood low-grade astrocytomas

Authors

  • Paul G. Fisher MD,

    Corresponding author
    1. Departments of Neurology, Pediatrics, Neurosurgery, and Human Biology, Stanford University, Palo Alto, California
    • Stanford University Cancer Center, Room 2220, 875 Blake Wilbur Drive, Palo Alto, CA 94305-5826.
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  • Tarik Tihan MD,

    1. Department of Pathology, University of California, San Francisco, California
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  • Patricia T. Goldthwaite MS,

    1. Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
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  • Moody D. Wharam MD,

    1. Department of Oncology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    2. Department of Radiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    3. Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    4. Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland
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  • Benjamin S. Carson MD,

    1. Department of Oncology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    2. Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    3. Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland
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  • Jon D. Weingart MD,

    1. Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland
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  • Michael X. Repka MD,

    1. Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    2. Department of Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
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  • Kenneth J. Cohen MD, MBA,

    1. Department of Oncology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    2. Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Maryland
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  • Peter C. Burger MD

    1. Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    2. Department of Oncology, The Johns Hopkins University School of Medicine, Baltimore, Maryland
    3. Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland
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Abstract

Background

We aimed to determine the long-term natural history of low-grade astrocytomas (LGA) in children, with respect to pathology, and to evaluate influence of treatment on survival.

Patients and Methods

A consecutive cohort of patients ≤21 years with surgically confirmed LGA from 1965 to 1996 was assembled. All available pathology specimens were reviewed, masked to original diagnosis, patient data, and neuroimaging.

Results

Two hundred seventy-eight children (160 males; mean age 9.1 years; tumor location: 77 cerebrum, 62 cerebellum, 51 hypothalamic, 30 thalamus, 9 ventricle, 40 brainstem, and 9 spine) were assessed. Among 246 specimens reviewed, diagnoses were 135 pilocytic astrocytoma (PA), 27 diffuse astrocytoma (DA), 75 unclassifiable well-differentiated astrocytoma (NOS), and 9 subependymal giant cell astrocytoma. At 5 and 10 years from initial surgery, for all LGA overall survival (OS) was 87% and 83%, while progression-free survival (PFS) was 55% and 42%, respectively. Original pathology diagnoses did not predict PFS (P = 0.47), but reviewed diagnoses were significantly associated with PFS (P = 0.007). Reviewed diagnoses were highly associated with OS (P < 0.0001), with 5-year OS for PA 96%, DA 48%, and NOS 86%; these differences remained significant when stratified by location or extent of resection. Among patients with residual tumor after surgery, 5-year PFS was 48% with observation alone (n = 114), no different (P = 0.32) from that achieved with immediate irradiation (n = 86).

Conclusion

LGA, particularly PA, have excellent long-term OS. While tumor location and resection extent affect outcome, pathologic diagnosis when carefully interpreted significantly influences long-term survival. Immediate postoperative irradiation does not confer an advantage in delaying first progression in children with residual PA. Pediatr Blood Cancer 2008;51:245–250. © 2008 Wiley-Liss, Inc.

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