Pediatric Astrocytomas with Monomorphous Pilomyxoid Features and a Less Favorable Outcome

Among tumors classified as pilocytic astrocytoma (PA) in the Johns Hopkins Hospital Department of Pathology files, we identified 18 cases with a distinctive monomorphous pilomyxoid histological pattern and a higher recurrence rate than that of PA with classical histological features (classical PA)....

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Bibliographic Details
Published in:Journal of neuropathology and experimental neurology 1999-10, Vol.58 (10), p.1061-1068
Main Authors: Tihan, Tarik, Goldthwaite, Patricia T, Burger, Peter C, Fisher, Paul G, Kepner, James L, Godfraind, Catherine, McCom, Rodney D
Format: Article
Language:English
Subjects:
Analysis
Astrocytoma - diagnosis
Astrocytoma - pathology
Astrocytoma - surgery
Astrocytoma - therapy
Biological and medical sciences
Brain Neoplasms - diagnosis
Brain Neoplasms - pathology
Brain Neoplasms - surgery
Brain Neoplasms - therapy
Brain tumors
Child
Child, Preschool
Combined Modality Therapy
Development and progression
Female
Follow-Up Studies
Gliomas
Humans
Infant
Magnetic Resonance Imaging
Male
Medical sciences
Neurology
Pediatrics
Prognosis
Tumors of the nervous system. Phacomatoses
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Summary:Among tumors classified as pilocytic astrocytoma (PA) in the Johns Hopkins Hospital Department of Pathology files, we identified 18 cases with a distinctive monomorphous pilomyxoid histological pattern and a higher recurrence rate than that of PA with classical histological features (classical PA). The majority of the tumors occurred in infants and young children and involved the hypothalamic/chiasmatic region. The tumors were histologically similar to PA, but they were more monomorphous and more myxoid. Rosenthal fibers were not seen and only 1 of 18 tumors had eosinophilic granular bodies. At the end of the follow-up period, 6 patients were dead and 12 were alive with evidence of disease. Progression free survival (PFS) at 1 year was 38.7%. In comparison, we identified a control group of 13 classical PAs in the same age range and location as the study group. In this group, PFS at 1 year was 69.2%, which was significantly better than that for pilomyxoid tumors (p=0.04). There was no CSF dissemination or death due to tumor progression among patients with classical PA. Eight of these patients are alive with recurrent disease, and 4 have no evidence of disease. While the monomorphous pilomyxoid tumors have some resemblance to classical PA, our results suggest that the former is a more aggressive variant or a separate entity that needs to be recognized for prognostic purposes.
ISSN:0022-3069
1554-6578
DOI:10.1097/00005072-199910000-00004